Materiały źródłowe

• #1 Phaeochromocytoma

  https://dermnetnz.org/topics/phaeochromocytoma

  The management of patients with phaeochromocytoma should be performed by multidisciplinary teams of experienced endocrinologists, anesthesthetists and surgeons, to prevent perioperative complications and reduce morbidity. […] Genetic counselling may be required. […] Alpha-blockers, eg phenoxybenzamine (long-acting), doxazosin (short-acting) […] B-blockers, eg atenolol do not use without concurrent alpha-blockade due to risk of severe refractory hypertension. […] Surgical excision is the only curative treatment modality. Effective management of perioperative hypertension improves outcomes. […] Laparoscopic surgery is generally preferred to open surgery. […] Treatment of metastatic phaeochromocytoma is limited, with no curative treatment options. […] Chemotherapy options for metastatic phaeochromocytoma include: Cyclophosphamide, vincristin, dacarbazine: 5year survival is 3060%. […] Other treatment options for metastatic phaeochromocytoma are: Radiotherapy, Ablation procedures, De-bulking surgery.

• #2 Pheochromocytoma – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pheochromocytoma/symptoms-causes/syc-20355367

  Surgery to remove a pheochromocytoma often returns blood pressure to a healthy range. […] If a pheochromocytoma isn’t treated, serious or life-threatening damage to other body systems can happen. […] A pheochromocytoma can lead to other health problems. The high blood pressure linked with a pheochromocytoma can damage organs, especially tissues of the heart and blood vessel system, brain and kidneys. This damage can cause dangerous conditions, including: Heart disease, Stroke, Kidney failure, Vision loss. […] Rarely, a pheochromocytoma spreads to other parts of the body. This tumor is then considered cancerous, also called malignant, and is referred to as metastatic pheochromocytoma.

• #3 Pheochromocytoma – Wikipedia

  https://en.wikipedia.org/wiki/Pheochromocytoma

  Surgery, chemotherapy, radiation, and pharmacologic agents are the treatment options for pheochromocytoma. […] Surgical resection is the only curative option for pheochromocytoma as of 2019. […] The United States Endocrine Society 2014 Clinical Practice Guideline for pheochromocytoma recommend a laparoscopic adrenalectomy (minimally invasive technique) for most adrenal tumors, unless they are invasive or are larger than 6.0 centimeters. […] A successful excision is a multidisciplinary effort involving the endocrinologist and the patient pre-operatively and the surgical team and anesthesiologist intraoperatively. […] Pre-operative alpha-adrenoceptor blockade is recommended to help circumvent a catecholamine-crisis. […] Beta-adrenoceptor antagonists should not be given alone in a pheochromocytoma patient; this can lead to severe consequences.

• #4 Pheochromocytoma Treatment | SpringerLink

  https://link.springer.com/chapter/10.1007/978-1-59259-757-4_14

  Surgical resection remains the cornerstone of pheochromocytoma therapy. Because most of these tumors are benign and unifocal, resection produces a permanent cure. […] Combined surgical and medical therapy is required for the management of metastatic pheochromocytoma.

• #5 Pheochromocytoma and Paraganglioma Treatment – NCI

  https://www.cancer.gov/types/pheochromocytoma/patient/pheochromocytoma-treatment-pdq

  Pheochromocytoma and paraganglioma are described as localized, regional, or metastatic. […] There are different types of treatment for patients with pheochromocytoma or paraganglioma. […] Patients receive medication to treat the signs and symptoms of pheochromocytoma and paraganglioma. […] The following types of treatment are used: Surgery, Radiation therapy, Chemotherapy, Ablation therapy, Embolization therapy, Targeted therapy. […] Drug therapy begins when pheochromocytoma or paraganglioma is diagnosed. […] Surgery to remove pheochromocytoma is usually an adrenalectomy (removal of one or both adrenal glands). […] Radiation therapy uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. […] Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing.

• #6 Pheochromocytoma: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/23373-pheochromocytoma

  Treatment options for pheochromocytoma include: Surgery. Radiation therapy. Chemotherapy. Ablation therapy. Embolization therapy. Targeted therapy. […] Surgery is the main form of treatment for pheochromocytoma. Approximately 90% of pheochromocytomas are successfully removed by surgery. […] After surgery, your provider will check the catecholamine levels in your blood or urine. Normal catecholamine levels are a sign that all the pheochromocytoma cells were removed. […] Radiation therapy is a cancer treatment that focuses strong beams of energy to destroy cancer cells or keep them from growing while sparing as much surrounding healthy tissue as possible. […] Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells by killing the cells or by preventing them from dividing and multiplying.

• #7 Pheochromocytoma – Wikipedia

  https://en.wikipedia.org/wiki/Pheochromocytoma

  Surgery, chemotherapy, radiation, and pharmacologic agents are the treatment options for pheochromocytoma. […] Surgical resection is the only curative option for pheochromocytoma as of 2019. […] The United States Endocrine Society 2014 Clinical Practice Guideline for pheochromocytoma recommend a laparoscopic adrenalectomy (minimally invasive technique) for most adrenal tumors, unless they are invasive or are larger than 6.0 centimeters. […] A successful excision is a multidisciplinary effort involving the endocrinologist and the patient pre-operatively and the surgical team and anesthesiologist intraoperatively. […] Pre-operative alpha-adrenoceptor blockade is recommended to help circumvent a catecholamine-crisis. […] Beta-adrenoceptor antagonists should not be given alone in a pheochromocytoma patient; this can lead to severe consequences.

• #8 Pheochromocytoma – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pheochromocytoma/diagnosis-treatment/drc-20355372

  The main treatment for a pheochromocytoma is surgery to remove the tumor. Before you have surgery, your healthcare professional likely will prescribe certain blood pressure medicines. These medicines block high-adrenaline hormones to lower the risk of dangerously high blood pressure during surgery. […] You’ll likely take medicines for 7 to 14 days before surgery to help lower blood pressure. These medicines will either replace or be added to other blood pressure medicines you take. You also may be told to eat a high-sodium diet. […] Most often, a surgeon makes a few small cuts called incisions in the stomach area. Wandlike devices equipped with video cameras and small tools are placed through the cuts to do the surgery. This is called laparoscopic surgery. Some surgeons do the procedure with robotic technology. They sit at a nearby console and control robotic arms, which hold a camera and surgery tools. If the tumor is very large, surgery that involves a larger incision and opening the abdominal cavity may be needed.

• #9 Pheochromocytoma – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pheochromocytoma/diagnosis-treatment/drc-20355372

  The main treatment for a pheochromocytoma is surgery to remove the tumor. Before you have surgery, your healthcare professional likely will prescribe certain blood pressure medicines. These medicines block high-adrenaline hormones to lower the risk of dangerously high blood pressure during surgery. […] You’ll likely take medicines for 7 to 14 days before surgery to help lower blood pressure. These medicines will either replace or be added to other blood pressure medicines you take. You also may be told to eat a high-sodium diet. […] Most often, a surgeon makes a few small cuts called incisions in the stomach area. Wandlike devices equipped with video cameras and small tools are placed through the cuts to do the surgery. This is called laparoscopic surgery. Some surgeons do the procedure with robotic technology. They sit at a nearby console and control robotic arms, which hold a camera and surgery tools. If the tumor is very large, surgery that involves a larger incision and opening the abdominal cavity may be needed.

• #10 Pheochromocytoma – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pheochromocytoma/diagnosis-treatment/drc-20355372

  The main treatment for a pheochromocytoma is surgery to remove the tumor. Before you have surgery, your healthcare professional likely will prescribe certain blood pressure medicines. These medicines block high-adrenaline hormones to lower the risk of dangerously high blood pressure during surgery. […] You’ll likely take medicines for 7 to 14 days before surgery to help lower blood pressure. These medicines will either replace or be added to other blood pressure medicines you take. You also may be told to eat a high-sodium diet. […] Most often, a surgeon makes a few small cuts called incisions in the stomach area. Wandlike devices equipped with video cameras and small tools are placed through the cuts to do the surgery. This is called laparoscopic surgery. Some surgeons do the procedure with robotic technology. They sit at a nearby console and control robotic arms, which hold a camera and surgery tools. If the tumor is very large, surgery that involves a larger incision and opening the abdominal cavity may be needed.

• #11 Phaeochromocytoma (Investigations and Treatment)

  https://patient.info/doctor/phaeochromocytoma-pro

  Laparoscopic surgery is being used more often for tumours smaller than 6 cm but for larger tumours, an open operation is probably safer. […] After surgery, a 24-hour urine collection for total catecholamines, metanephrines and VMA is required two weeks after operation. If results are normal, the prognosis is excellent. It is important to ensure that hypertension is controlled or resolved. Lifelong annual biochemical testing is recommended to detect recurrent or metastatic disease. […] In the rare malignant cases, palliative care may be achieved with radiotherapy and chemotherapy. New emerging therapies, such as the tyrosine kinase inhibitor sunitinib, which rectifies the results of genetic abnormalities, may revolutionise the treatment of malignancy in the future.

• #12 Pheochromocytoma: Symptoms, Causes, Diagnosis, and Treatment

  https://www.webmd.com/cancer/what_is_pheochromocytoma

  Youll most likely need surgery to remove the tumor. […] Before the surgery, you may need to take a blood pressure medicine to block adrenal gland hormones from raising blood pressure to dangerously high levels during surgery. These may include an alpha-blocker, beta-blocker, or calcium channel blocker, drugs that help your blood vessels relax and open. You will need to take these medicines one to two weeks before surgery and may be prescribed a low-sodium diet. […] Your doctor might perform your surgery using tiny cuts instead of one large opening. This is called laparoscopic or minimally invasive surgery. You generally recover faster from those procedures than from traditional surgery. […] If you have a tumor in only one adrenal gland, your doctor may remove that whole gland. The other gland will make the hormones your body needs. […] If you have tumors in both glands, your surgeon may remove just the tumors and leave part of the glands. […] If your tumor is cancerous, you may also have radiation, chemotherapy, or targeted therapy (using drugs to attack specific cancer cells) to help keep it from growing.

• #13 Treatments for cancerous pheochromocytoma | Canadian Cancer Society

  https://cancer.ca/en/cancer-information/cancer-types/adrenal-gland/treatment/pheochromocytoma

  The following are treatment options for cancerous pheochromocytomas. Your healthcare team will suggest treatments based on your needs and work with you to develop a treatment plan. […] The treatments depend on whether the cancer can be completely removed with surgery (is resectable) or cant be completely removed with surgery (is unresectable). […] Different types of supportive therapy drugs may be offered as treatment for pheochromocytomas. […] Drugs to control high blood pressure and irregular heart rhythms are also called alpha blockers and beta blockers. These drugs are usually given 1 to 2 weeks before surgery. Blood pressure and heart rhythms must be normal before surgery can be done. […] Drugs to replace very low levels of adrenal gland hormones after surgery are also called hormone replacement therapy (HRT) drugs. They are given until hormone levels return to normal. Hormone levels usually return to normal within 2 weeks after surgery if the cancer was completely removed. If both of the adrenal glands are removed, you will need to take HRT for the rest of your life.

• #14 Pheochromocytoma: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/124059-overview

  Surgical resection of the tumor is the treatment of choice and usually cures the hypertension. Careful preoperative treatment with alpha and beta blockers is required to control blood pressure and prevent intraoperative hypertensive crises. […] Preoperative medical stabilization is provided as follows: Start alpha blockade with phenoxybenzamine 7-10 days preoperatively; Provide volume expansion with isotonic sodium chloride solution; Encourage liberal salt intake; Initiate a beta blocker only after adequate alpha blockade, to avoid precipitating a hypertensive crisis from unopposed alpha stimulation; Administer the last doses of oral alpha and beta blockers on the morning of surgery.

• #15 Phaeochromocytoma

  https://www.nhs.uk/conditions/phaeochromocytoma/

  It’s usually possible to successfully remove a phaeochromocytoma using surgery. […] Most people with a phaeochromocytoma will need surgery to have it removed. […] You’ll usually be given medicine called alpha blockers (and in some cases beta blockers) for several weeks before the operation. They block the effects of the excess hormones on your body and stabilise your heart rate and blood pressure. […] The operation is carried out under general anaesthetic (when you’re unconscious). It may be done as: „keyhole” (laparoscopic) surgery a number of small cuts (incisions) are made and fine instruments are passed through these to remove the tumour; this is the most common type of surgery for phaeochromocytomas […] open surgery a single larger incision is made in the skin to access and remove the tumour. […] If your phaeochromocytoma is cancerous, you may need chemotherapy or radiotherapy in addition to surgery. […] If your tumour cannot be removed, you’ll need medicine to manage your condition. This will usually be a combination of medicines to control the effects of the excessive hormones.

• #16 Pheochromocytoma treatment – USZ

  https://www.usz.ch/en/department/endocrinology/service/pheochromocytoma-treatment/

  Pheochromocytoma is treated surgically. We remove the adrenal gland together with the tumor. The surgical procedure is called an „adrenalectomy”. […] Whenever possible, we perform minimally invasive keyhole surgery. This laparoscopy involves just a few small incisions and is considered to be gentle. If this is not possible (e.g. in the case of very large tumors), open surgery via abdominal incision is used. […] Seven to 14 days before the operation, you must take so-called alpha blockers as special blood pressure medication. Phenoxybenzamine is a frequently used active ingredient. The drug neutralizes the effect of adrenaline and noradrenaline on the blood vessels. This helps to prevent high blood pressure incidents and cardiac arrhythmias during anesthesia and surgery. […] In around five to ten percent of cases, the pheochromocytoma is malignant. The tumor has either invaded neighboring tissue or has led to cancer metastases. The operation is then followed by further treatments.

• #17 Pheochromocytoma – Endocrine and Metabolic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/endocrine-and-metabolic-disorders/adrenal-disorders/pheochromocytoma

  Surgical removal is the treatment of choice. The operation is usually delayed until hypertension is controlled by a combination of alpha-blockers and beta-blockers (usually phenoxybenzamine 20 to 40 mg orally 3 times a day and propranolol 20 to 40 mg orally 3 times a day). […] The blood pressure target is 130/80 mmHg; some suggest the target should also include achieving a certain postural drop in blood pressure, but this is not essential. Re-equilibration of the cardiovascular system requires approximately 10 to 14 days, after which blockade is presumed to be effective. Beta-blockers should not be used until adequate alpha-blockade has been achieved. Some alpha-blockers, such as doxazosin, may be equally effective but better tolerated. […] Nitroprusside can be infused for hypertensive crises preoperatively or intraoperatively.

• #18 Phaeochromocytoma | Endocrine Conditions

  https://www.yourhormones.info/endocrine-conditions/phaeochromocytoma/

  Patients will normally be placed on medications to block the effects of excess hormones. These are called alpha-blockers (phenoxybenzamine, doxazosin, prazosin, terazosin) and beta-blockers (propranolol, labetalol). […] Surgical removal of the tumour is the best curative treatment. A patient may need to take tablets for several days prior to surgery to ensure the blood pressure is well controlled. Surgery should be carried out by a surgeon and anaesthetist experienced in operating on this type of tumour and in centres where there is a multidisciplinary endocrine team that deals with these tumours. […] If the tumour has spread or surgery cannot be undertaken or any residual tumor after surgery, then other treatments include chemotherapy and/or a radioactive form of treatment called radiolabelled MIBG.

• #19 Pheochromocytoma Treatment & Management: Approach Considerations, Laparoscopic Adrenalectomy, Pheochromocytoma in Pregnancy

  https://emedicine.medscape.com/article/124059-treatment

  Surgical resection of the tumor is the treatment of choice for pheochromocytoma and usually results in cure of the hypertension. Careful preoperative management is required to control blood pressure, correct fluid volume, and prevent intraoperative hypertensive crises. […] Although there is no consensus regarding the preferred drugs for preoperative blood pressure control, alpha blockers, beta blockers, calcium channel blockers, and angiotensin receptor blockers have all been used. […] Start alpha blockade with phenoxybenzamine 10-14 days preoperatively to allow for expansion of blood volume. The patient should undergo volume expansion with isotonic sodium chloride solution. Encourage liberal salt intake. […] Initiate a beta blocker only after adequate alpha blockade (usually, 2 days). If beta blockade is started prematurely, unopposed alpha stimulation could precipitate a hypertensive crisis. Administer the last doses of oral alpha and beta blockers on the morning of surgery.

• #20 Pheochromocytoma – Knowledge @ AMBOSS

  https://www.amboss.com/us/knowledge/pheochromocytoma/

  A no-touch technique should be used because contact with the tumor can lead to massive catecholamine release. […] Preoperative management of blood pressure and heart rate is required. Treatment target: BP < 90 mmHg systolic. Heart rate 60-80/min while seated. [...] Pharmacotherapy: Initiate 7-14 days prior to surgery. Indicated for all patients with hormonally active pheochromocytoma. Start with alpha-adrenergic blockade. [...] Starting beta blockers before alpha blockers is contraindicated because doing so can cause life-threatening hypertensive crisis.

• #21

  https://link.springer.com/article/10.1007/s12020-013-0007-y

  Pheochromocytoma (PH) and paraganglioma (PG) are neuroendocrine neoplasms arising from chromaffin cells of the adrenal medulla and the sympathetic ganglia, respectively. […] HT treatment may be difficult when PH or PG occurs in pregnancy or in the elderly subjects and in these cases a multidisciplinary team is required. When surgical excision is mandatory the perioperative management requires the administration of selective 1-adrenergic blocking agents (i.e., doxazosin, prazosin or terazosin) followed by a -adrenergic blockade (i.e., propranolol, atenolol). […] Although labetalol is traditionally considered the ideal agent due to its – and -adrenergic antagonism, experimental studies do not support its use in this clinical setting. As second regimen, the administration of vasodilators as calcium channel blockers (i.e., nicardipine, nifedipine) may be required to control BP. […] Oral and sublingual short-acting nifedipine are potentially dangerous in patients with hypertensive emergencies and are not recommend. The latest evidences into the diagnosis and treatment of hypertensive crisis due to PH and PG are reviewed here.

• #22 Pheochromocytoma: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/124059-overview

  Surgical resection of the tumor is the treatment of choice and usually cures the hypertension. Careful preoperative treatment with alpha and beta blockers is required to control blood pressure and prevent intraoperative hypertensive crises. […] Preoperative medical stabilization is provided as follows: Start alpha blockade with phenoxybenzamine 7-10 days preoperatively; Provide volume expansion with isotonic sodium chloride solution; Encourage liberal salt intake; Initiate a beta blocker only after adequate alpha blockade, to avoid precipitating a hypertensive crisis from unopposed alpha stimulation; Administer the last doses of oral alpha and beta blockers on the morning of surgery.

• #23 Pheochromocytoma Treatment & Management: Approach Considerations, Laparoscopic Adrenalectomy, Pheochromocytoma in Pregnancy

  https://emedicine.medscape.com/article/124059-treatment

  Surgical resection of the tumor is the treatment of choice for pheochromocytoma and usually results in cure of the hypertension. Careful preoperative management is required to control blood pressure, correct fluid volume, and prevent intraoperative hypertensive crises. […] Although there is no consensus regarding the preferred drugs for preoperative blood pressure control, alpha blockers, beta blockers, calcium channel blockers, and angiotensin receptor blockers have all been used. […] Start alpha blockade with phenoxybenzamine 10-14 days preoperatively to allow for expansion of blood volume. The patient should undergo volume expansion with isotonic sodium chloride solution. Encourage liberal salt intake. […] Initiate a beta blocker only after adequate alpha blockade (usually, 2 days). If beta blockade is started prematurely, unopposed alpha stimulation could precipitate a hypertensive crisis. Administer the last doses of oral alpha and beta blockers on the morning of surgery.

• #24 Pheochromocytoma – Endocrine and Metabolic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/endocrine-and-metabolic-disorders/adrenal-disorders/pheochromocytoma

  When bilateral tumors are documented or suspected (as in a patient with multiple endocrine neoplasia [MEN]), sufficient hydrocortisone (100 mg IV twice a day) given before and during surgery avoids acute glucocorticoid insufficiency due to bilateral adrenalectomy. […] Most pheochromocytomas can be removed laparoscopically. Blood pressure must be continuously monitored via an intra-arterial catheter, and volume status is closely monitored. […] Malignant metastatic pheochromocytoma should be treated with alpha-blockers and beta-blockers. The tumor may be indolent and survival long-lasting. However, even with rapid tumor growth, blood pressure can be controlled. I-131 MIBG or more recently lutetium-177 dotatate can help relieve symptoms in patients with residual disease. Metyrosine, a tyrosine hydroxylase inhibitor, may be used to decrease catecholamine production in patients whose blood pressure is difficult to control. Radiation therapy may reduce bone pain due to metastases. Chemotherapy is rarely effective, but the most common regimen tried is the combination of cyclophosphamide, vincristine, and dacarbazine. The chemotherapy agent temozolomide and targeted therapy with sunitinib or possibly cabozantinib may also be effective. Belzutifan, a hypoxia-inducible factor inhibitor used in the treatment of associated cancers in patients with von Hippel-Lindau disease, is currently undergoing clinical trials in patients with malignant metastatic pheochromocytoma.

• #25 Pheochromocytoma FAQs: Treatment & Diagnosis – Endocrine Surgery | UCLA Health

  https://www.uclahealth.org/medical-services/surgery/endocrine-surgery/conditions-treated/adrenal/adrenal-tumors/pheochromocytoma/pheochromocytoma-treatment-diagnosis

  We deliver effective, minimally invasive treatments in a caring environment. […] Surgery can only be performed safely after the careful administration of alpha-blockers (medications such as phenxoybenzamine, which render the body less sensitive to catecholamine surges) for at least two to three weeks prior to surgery. […] The importance of meticulous pre-operative conditioning with alpha-blockers cannot be overemphasized. […] In select cases, beta-blockers (medications that slow the heart rate) may be added after adequate alpha-blockade has been established. […] At expert centers, most pheochromocytomas are removed laparoscopically. […] The key to successful surgery is effective teamwork between the surgeon and anesthesiologist. […] After surgery, patients frequently require close monitoring in the intensive care unit.

• #26 Pheochromocytoma – Endocrine and Metabolic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/endocrine-and-metabolic-disorders/adrenal-disorders/pheochromocytoma

  When bilateral tumors are documented or suspected (as in a patient with multiple endocrine neoplasia [MEN]), sufficient hydrocortisone (100 mg IV twice a day) given before and during surgery avoids acute glucocorticoid insufficiency due to bilateral adrenalectomy. […] Most pheochromocytomas can be removed laparoscopically. Blood pressure must be continuously monitored via an intra-arterial catheter, and volume status is closely monitored. […] Malignant metastatic pheochromocytoma should be treated with alpha-blockers and beta-blockers. The tumor may be indolent and survival long-lasting. However, even with rapid tumor growth, blood pressure can be controlled. I-131 MIBG or more recently lutetium-177 dotatate can help relieve symptoms in patients with residual disease. Metyrosine, a tyrosine hydroxylase inhibitor, may be used to decrease catecholamine production in patients whose blood pressure is difficult to control. Radiation therapy may reduce bone pain due to metastases. Chemotherapy is rarely effective, but the most common regimen tried is the combination of cyclophosphamide, vincristine, and dacarbazine. The chemotherapy agent temozolomide and targeted therapy with sunitinib or possibly cabozantinib may also be effective. Belzutifan, a hypoxia-inducible factor inhibitor used in the treatment of associated cancers in patients with von Hippel-Lindau disease, is currently undergoing clinical trials in patients with malignant metastatic pheochromocytoma.

• #27 Pheochromocytoma FAQs: Treatment & Diagnosis – Endocrine Surgery | UCLA Health

  https://www.uclahealth.org/medical-services/surgery/endocrine-surgery/conditions-treated/adrenal/adrenal-tumors/pheochromocytoma/pheochromocytoma-treatment-diagnosis

  We deliver effective, minimally invasive treatments in a caring environment. […] Surgery can only be performed safely after the careful administration of alpha-blockers (medications such as phenxoybenzamine, which render the body less sensitive to catecholamine surges) for at least two to three weeks prior to surgery. […] The importance of meticulous pre-operative conditioning with alpha-blockers cannot be overemphasized. […] In select cases, beta-blockers (medications that slow the heart rate) may be added after adequate alpha-blockade has been established. […] At expert centers, most pheochromocytomas are removed laparoscopically. […] The key to successful surgery is effective teamwork between the surgeon and anesthesiologist. […] After surgery, patients frequently require close monitoring in the intensive care unit.

• #28 Pheochromocytoma FAQs: Treatment & Diagnosis – Endocrine Surgery | UCLA Health

  https://www.uclahealth.org/medical-services/surgery/endocrine-surgery/conditions-treated/adrenal/adrenal-tumors/pheochromocytoma/pheochromocytoma-treatment-diagnosis

  Most patients who undergo laparoscopic surgery stay one to two days in the hospital, after which they return to normal activities within one to two weeks. […] After aggressive surgery has been carried out, adjuvant treatment options include: Combination chemotherapy, External beam radiation therapy, High-dose 131I-meta-iodobenzylguanidine (MIBG) radionuclide therapy. […] Of course, ongoing hormone excess must be treated with long-term alpha-blocker therapy in all cases where catecholamine levels remain demonstrably high after surgery.

• #29 Pheochromocytoma: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/23373-pheochromocytoma

  Treatment options for pheochromocytoma include: Surgery. Radiation therapy. Chemotherapy. Ablation therapy. Embolization therapy. Targeted therapy. […] Surgery is the main form of treatment for pheochromocytoma. Approximately 90% of pheochromocytomas are successfully removed by surgery. […] After surgery, your provider will check the catecholamine levels in your blood or urine. Normal catecholamine levels are a sign that all the pheochromocytoma cells were removed. […] Radiation therapy is a cancer treatment that focuses strong beams of energy to destroy cancer cells or keep them from growing while sparing as much surrounding healthy tissue as possible. […] Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells by killing the cells or by preventing them from dividing and multiplying.

• #30 Pheochromocytoma – Endocrinology Advisor

  https://www.endocrinologyadvisor.com/ddi/pheochromocytoma/

  Lifelong monitoring after surgery will be required to ensure that the pheochromocytoma treatment was successful, identify complications, and monitor tumor recurrence. Repeating biochemical tests should be done no sooner than 14 days after surgery. If metanephrine levels are elevated for three months after surgical resection, further imaging is indicated to look for another tumor.

• #31 Pheochromocytoma – Endocrinology Advisor

  https://www.endocrinologyadvisor.com/ddi/pheochromocytoma/

  Lifelong monitoring after surgery will be required to ensure that the pheochromocytoma treatment was successful, identify complications, and monitor tumor recurrence. Repeating biochemical tests should be done no sooner than 14 days after surgery. If metanephrine levels are elevated for three months after surgical resection, further imaging is indicated to look for another tumor.

• #32 What are the treatments for pheochromocytoma? | NICHD – Eunice Kennedy Shriver National Institute of Child Health and Human Development

  http://www.nichd.nih.gov/health/topics/pheochrom/conditioninfo/treatments

  Standard treatments for pheochromocytoma include: Surgical removal of the tumor […] Medications (chemotherapy) designed to kill tumor cells […] Radiotherapy: using radio waves to destroy the tumors […] Medications to control the signs and symptoms of the disease. Ninety percent of patients are cured by surgery to remove benign pheochromocytoma tumors. Surgery for tumor removal is typically done by laparoscopy, during which a small incision is made in the abdomen. During surgery to remove the tumor, the physician will usually examine nearby organs to determine whether the pheochromocytoma has spread to other parts of the body. Medications are prescribed to treat the clinical signs and symptoms of pheochromocytoma. Commonly prescribed medications include: Alpha-adrenergic blockers to lower blood pressure […] Beta blockers for controlling rapid, irregular pulse. For patients whose tumors are successfully removed, blood pressure and hormone levels usually return to expected levels over the weeks immediately following surgery.

• #33 Pheochromocytoma Treatment & Management: Approach Considerations, Laparoscopic Adrenalectomy, Pheochromocytoma in Pregnancy

  https://emedicine.medscape.com/article/124059-treatment

  Test plasma free metanephrines 2 weeks postoperatively. If results are within the reference range, resection is deemed complete; in such cases, patient survival approaches age-matched controls. In addition, ensure resolution of the hypertension and any associated complications. […] For surgical follow-up, obtain plasma metanephrine levels yearly for 10 years. Ensure that blood pressure is under control. In patients with an underlying genetic mutation, lifelong follow-up is mandatory. […] A literature review by Holscher et al indicated that following curative adrenalectomy, the pooled recurrence rate for sporadic pheochromocytomas is 3%. The weighted mean time to recurrence in the study was 49.4 months, with the weighted mean follow-up period being 77.3 months. […] Surgical mortality rates are less than 2-3% when the operation is performed by a surgeon and an anesthesiologist who are experienced.

• #34 Pheochromocytoma – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pheochromocytoma/diagnosis-treatment/drc-20355372

  If you have metastatic pheochromocytoma, which means the tumor has spread to other organs, surgery may not be able to remove all of the cancer tissue. Removing as much of the tumor as possible along with medical therapy might ease pheochromocytoma symptoms. It also makes blood pressure easier to control. […] Very few pheochromocytomas spread through the body as cancer, called metastatic pheochromocytomas. Because they are rare, research about the best treatments is limited. Treatments for metastatic pheochromocytoma may include: […] Targeted therapies. These use a medicine combined with a radioactive substance that seeks out cancer cells and kills them. […] Chemotherapy. This treatment uses powerful drugs that kill fast-growing cancer cells. It may help ease symptoms in people with pheochromocytomas whose cancer has spread. […] Radiation therapy. This treatment uses beams of intense energy to kill cancer cells. It may relieve symptoms of tumors that have spread to the bone and cause pain. […] Ablation. This treatment can destroy cancer tumors with freezing temperatures, high-energy radio waves or ethanol alcohol.

• #35 Pheochromocytoma – Wikipedia

  https://en.wikipedia.org/wiki/Pheochromocytoma

  Metastatic pheochromocytoma is best managed with a multidisciplinary team of oncologists, surgeons, radiologists, nuclear medicine physicians, and endocrinologists. […] Normally, the goal of surgery is complete cytoreductive surgery; however, with widespread metastatic disease, this is not always feasible. […] Radiation techniques are primarily used for pain control, specifically with regards to bone metastases, local control of the disease, and to limit spinal cord compression. […] The most common chemotherapy regimen for metastatic pheochromocytoma is cyclophosphamide, vincristine, and dacarbazine, collectively known as CVD. […] Iodine-131 meta-iodobenzylguanidine (MIBG) is not only useful in locating the presence of metastatic disease, but also as an available treatment modality. […] Peptide Receptor Radionuclide Therapy (PRRT) utilizes the 68-Ga DOTA analogues mentioned above in the functional imaging section.

• #36 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  Surgery is always the therapy of choice of non-metastatic PCC/PGL, whenever possible. […] However, surgery of non-functioning HN PGLs has to be carefully balanced against surgery-related morbidities, especially for the cranial nerves for vagal and jugular PGLs. […] In cases of a high risk related to surgery, radiotherapy/radiosurgery (gamma-knife/cyberknife) might be a less invasive option with non-curative but controlling outcomes. […] In patients with hereditary PCCs, cortical sparing surgery should always be considered since there is frequently a high risk of bilateral PCCs in hereditary disease, and cortical-sparing surgery was not associated with decreased survival despite PCC recurrence in 13% of cases in a very recently published study. […] With metastatic disease, primary tumor resection should be recommended if feasible in order to alleviate cardiovascular and other symptoms from catecholamine excess or from tumor invasion, and to minimize the target for radiopharmaceutical therapies.

• #37 Pheochromocytoma – Wikipedia

  https://en.wikipedia.org/wiki/Pheochromocytoma

  Metastatic pheochromocytoma is best managed with a multidisciplinary team of oncologists, surgeons, radiologists, nuclear medicine physicians, and endocrinologists. […] Normally, the goal of surgery is complete cytoreductive surgery; however, with widespread metastatic disease, this is not always feasible. […] Radiation techniques are primarily used for pain control, specifically with regards to bone metastases, local control of the disease, and to limit spinal cord compression. […] The most common chemotherapy regimen for metastatic pheochromocytoma is cyclophosphamide, vincristine, and dacarbazine, collectively known as CVD. […] Iodine-131 meta-iodobenzylguanidine (MIBG) is not only useful in locating the presence of metastatic disease, but also as an available treatment modality. […] Peptide Receptor Radionuclide Therapy (PRRT) utilizes the 68-Ga DOTA analogues mentioned above in the functional imaging section.

• #38 Pheochromocytoma – Wikipedia

  https://en.wikipedia.org/wiki/Pheochromocytoma

  Metastatic pheochromocytoma is best managed with a multidisciplinary team of oncologists, surgeons, radiologists, nuclear medicine physicians, and endocrinologists. […] Normally, the goal of surgery is complete cytoreductive surgery; however, with widespread metastatic disease, this is not always feasible. […] Radiation techniques are primarily used for pain control, specifically with regards to bone metastases, local control of the disease, and to limit spinal cord compression. […] The most common chemotherapy regimen for metastatic pheochromocytoma is cyclophosphamide, vincristine, and dacarbazine, collectively known as CVD. […] Iodine-131 meta-iodobenzylguanidine (MIBG) is not only useful in locating the presence of metastatic disease, but also as an available treatment modality. […] Peptide Receptor Radionuclide Therapy (PRRT) utilizes the 68-Ga DOTA analogues mentioned above in the functional imaging section.

• #39 Pheochromocytoma: Staging and Treatment | OncoLink

  https://www.oncolink.org/cancers/pheochromocytoma/pheochromocytoma-staging-and-treatment

  Meta-iodo-benzylguanidine (MIBG), can be used to treat pheochromocytoma. MIBG has small amounts of radioactive material in it, which collect in the tumor, causing it to light up on a scan. […] Peptide receptor radionuclide therapy (PRRT) combines a medication (Lutetium 177 (Lu-177)) that targets cancer cells with a radioactive substance. This delivers radiation directly to the cancer cells. […] Targeted therapies stop or slow the growth of a tumor by stopping a specific molecule needed for cancer growth. Molecular-targeted therapies, such as sunitinib, can be used after surgery if there is a high risk of cancer coming back (called recurrence). […] Chemotherapy are medications that are often given intravenously (IV, into a vein) or in pill form. Chemotherapy travels through the bloodstream and throughout the body to kill cancer cells. For pheochromocytoma, chemotherapy is not used as a primary treatment, but as an option for malignant pheochromocytoma that causes symptoms and that has not responded to MIBG therapy. […] You may be offered a clinical trial as part of your treatment plan.

• #40 Treatments for cancerous pheochromocytoma | Canadian Cancer Society

  https://cancer.ca/en/cancer-information/cancer-types/adrenal-gland/treatment/pheochromocytoma

  Radiation therapy may be offered to treat metastatic and recurrent cancerous pheochromocytomas. […] Chemotherapy may be offered to treat metastatic and recurrent cancerous pheochromocytomas. The most common combination of chemotherapy drugs used is CVD, which is cyclophosphamide, vincristine and dacarbazine. […] Targeted therapy may also be called molecular targeted therapy. […] Sunitinib may be offered to treat metastatic pheochromocytoma. […] Other treatments may be offered for metastatic and recurrent pheochromocytomas, including radiofrequency ablation (RFA) and transarterial chemoembolization (TACE). […] Watchful waiting means that your healthcare team watches the cancer closely rather than giving treatment right away. […] You may want to consider a type of care to make you feel better without treating the cancer itself.

• #41 Pheochromocytoma – Wikipedia

  https://en.wikipedia.org/wiki/Pheochromocytoma

  Metastatic pheochromocytoma is best managed with a multidisciplinary team of oncologists, surgeons, radiologists, nuclear medicine physicians, and endocrinologists. […] Normally, the goal of surgery is complete cytoreductive surgery; however, with widespread metastatic disease, this is not always feasible. […] Radiation techniques are primarily used for pain control, specifically with regards to bone metastases, local control of the disease, and to limit spinal cord compression. […] The most common chemotherapy regimen for metastatic pheochromocytoma is cyclophosphamide, vincristine, and dacarbazine, collectively known as CVD. […] Iodine-131 meta-iodobenzylguanidine (MIBG) is not only useful in locating the presence of metastatic disease, but also as an available treatment modality. […] Peptide Receptor Radionuclide Therapy (PRRT) utilizes the 68-Ga DOTA analogues mentioned above in the functional imaging section.

• #42 Treatments for cancerous pheochromocytoma | Canadian Cancer Society

  https://cancer.ca/en/cancer-information/cancer-types/adrenal-gland/treatment/pheochromocytoma

  Radiation therapy may be offered to treat metastatic and recurrent cancerous pheochromocytomas. […] Chemotherapy may be offered to treat metastatic and recurrent cancerous pheochromocytomas. The most common combination of chemotherapy drugs used is CVD, which is cyclophosphamide, vincristine and dacarbazine. […] Targeted therapy may also be called molecular targeted therapy. […] Sunitinib may be offered to treat metastatic pheochromocytoma. […] Other treatments may be offered for metastatic and recurrent pheochromocytomas, including radiofrequency ablation (RFA) and transarterial chemoembolization (TACE). […] Watchful waiting means that your healthcare team watches the cancer closely rather than giving treatment right away. […] You may want to consider a type of care to make you feel better without treating the cancer itself.

• #43 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  In the case of progression after chemotherapy or radionuclide therapy, or if chemotherapy or radionuclide therapy are not possible/tolerated by the patient, the TKIs sunitinib (37.5 mg or 50 mg daily) or cabozantinib (60 mg daily, or titrated down to a tolerable dose) may be considered, especially for RET and SDHx-mutation carriers. […] Several targeted therapies (PI3K inhibitors in combination with mTORC1 inhibitors, HIF-2α inhibitors, PARP inhibitors, SSTR2 analogs, HDAC inhibitors, DNA demethylating agents) and immunotherapy are currently under investigation and may have strong potential for future personalized therapy approaches.

• #44 Pheochromocytoma Treatment – San Diego – Scripps Health

  https://www.scripps.org/services/cancer-care/pheochromocytoma-treatment

  Cryoablation uses liquid carbon dioxide to freeze and destroy abnormal cells. […] During this minimally invasive procedure, a radiologist uses a catheter to place a substance or medication into the artery that leads to the adrenal gland, blocking the flow of blood to cancer cells to kill them. […] Chemotherapy drugs may be used to fight pheochromocytoma that has spread to other parts of the body. […] External beam radiation therapy can be provided for palliative care of pheochromocytoma. […] Tyrosine kinase inhibitors, such as sunitinib, can block signals that tumors need to grow. These drugs are being studied as new treatment options for pheochromocytoma.

• #45 Pheochromocytoma: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/23373-pheochromocytoma

  Ablation therapy is a minimally invasive treatment option that uses very high or very low temperatures to destroy tumors. […] Embolization therapy is a pheochromocytoma treatment that blocks the artery leading to your adrenal gland. Blocking the blood flow to your adrenal glands helps kill the cancer cells that are growing there. […] Targeted therapy is a treatment option that uses medications or other substances to attack specific cancer cells without harming healthy cells. Healthcare providers use targeted therapies to treat metastatic and recurrent pheochromocytoma.

• #46 Pheochromocytoma: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/23373-pheochromocytoma

  Ablation therapy is a minimally invasive treatment option that uses very high or very low temperatures to destroy tumors. […] Embolization therapy is a pheochromocytoma treatment that blocks the artery leading to your adrenal gland. Blocking the blood flow to your adrenal glands helps kill the cancer cells that are growing there. […] Targeted therapy is a treatment option that uses medications or other substances to attack specific cancer cells without harming healthy cells. Healthcare providers use targeted therapies to treat metastatic and recurrent pheochromocytoma.

• #47 Pheochromocytoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK589700/

  However, it is well-recognized that initiating medical therapy with – and -blockade in the preoperative period can significantly reduce the risk of uncontrolled hypertension, hypertensive crises, tachycardia, and volume expansion during the perioperative period. […] According to the Endocrine Society Clinical Practice Guidelines, the first-line drugs for preoperative preparation are -adrenergic receptor blockers. […] Nonsurgical management of pheochromocytomas may involve radiotherapy, chemotherapy, and palliative care. […] Embolization involves blocking the artery supplying blood to a tumor. […] The palliative treatment approach for advanced metastatic pheochromocytoma focuses on relieving symptoms and improving quality of life. This may involve surgical intervention to remove as much of the tumor as possible or radiation therapy to target areas affected by the cancer. Additionally, medications such as – and -blockers can be used to manage disease-related symptoms effectively.

• #48 Pheochromocytoma Treatment & Management: Approach Considerations, Laparoscopic Adrenalectomy, Pheochromocytoma in Pregnancy

  https://emedicine.medscape.com/article/124059-treatment

  Use an arterial line, cardiac monitor, and Swan-Ganz catheter. Administer stress-dose steroids if bilateral resection is planned. […] If pheochromocytoma is found during pregnancy, initiate alpha-adrenergic blockade (with phenoxybenzamine) as soon as the diagnosis is confirmed. Remove the tumor by laparoscopic adrenalectomy as soon as possible during the first 2 trimesters, after proper preparation. […] In a retrospective study detailing the efficacy of various therapeutic options for metastatic pheochromocytoma/paraganglioma, Fischer et al reported the disease-control rates (DCRs) at 3 months and periods of survival until detected progress (SDP) for these treatments. […] In second-line therapy with temozolomide, the DCR was 60.0% (n = 5), with SDP reaching 10 months.

• #49 Pheochromocytoma and Paraganglioma Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/pheochromocytoma/hp/pheochromocytoma-treatment-pdq

  Surgical management of pheochromocytoma in patients with the hereditary syndromes multiple endocrine neoplasia type 2 (MEN2) or von Hippel-Lindau (VHL) disease has been controversial. […] If all identifiable disease is resectable, including a limited number of distant metastases, surgery can provide occasional long-term remission. […] Chemotherapy has not been shown to improve survival in patients with metastatic pheochromocytoma; however, chemotherapy may be useful for symptom palliation. […] Other palliative treatment modalities include external-beam radiation therapy and embolization, radiofrequency ablation, or cryoablation. […] Treatment for recurrent disease involves appropriate medical management (i.e., alpha-adrenergic blockade) followed by complete surgical resection, when possible. […] Phenoxybenzamine use is safe in pregnancy, but beta-adrenergic blockers should be initiated only if needed because their use has been associated with intrauterine growth restriction.

• #50 Pheochromocytoma Treatment & Management: Approach Considerations, Laparoscopic Adrenalectomy, Pheochromocytoma in Pregnancy

  https://emedicine.medscape.com/article/124059-treatment

  Use an arterial line, cardiac monitor, and Swan-Ganz catheter. Administer stress-dose steroids if bilateral resection is planned. […] If pheochromocytoma is found during pregnancy, initiate alpha-adrenergic blockade (with phenoxybenzamine) as soon as the diagnosis is confirmed. Remove the tumor by laparoscopic adrenalectomy as soon as possible during the first 2 trimesters, after proper preparation. […] In a retrospective study detailing the efficacy of various therapeutic options for metastatic pheochromocytoma/paraganglioma, Fischer et al reported the disease-control rates (DCRs) at 3 months and periods of survival until detected progress (SDP) for these treatments. […] In second-line therapy with temozolomide, the DCR was 60.0% (n = 5), with SDP reaching 10 months.

• #51 Pheochromocytoma and Paraganglioma Treatment (PDQ®): Treatment – Health Professional Information [NCI] – Statesboro Ear, Nose, & Throat Clinic

  https://drcrews.com/patient-education/healthwise?DOCHWID=ncicdr0000681246

  If all identifiable disease is resectable, including a limited number of distant metastases, surgery can provide occasional long-term remission. […] Chemotherapy has not been shown to improve survival in patients with metastatic pheochromocytoma; however, chemotherapy may be useful for symptom palliation. […] Other palliative treatment modalities include external-beam radiation therapy and embolization, radiofrequency ablation, or cryoablation. […] Treatment options for recurrent pheochromocytoma include: Surgery, Palliative therapy. […] After resection of a localized pheochromocytoma presumed to represent a benign tumor and documented normal postoperative biochemical testing, disease recurrence occurs in 6.5% to 16.5% of patients. […] Phenoxybenzamine use is safe in pregnancy, but beta-adrenergic blockers should be initiated only if needed because their use has been associated with intrauterine growth restriction. […] Resection of the tumor can often be performed safely during the second trimester, or tumor resection can be combined with cesarean delivery for patients diagnosed later in pregnancy.

• #52 A Functional Medicine Approach to Pheochromocytoma

  https://www.rupahealth.com/post/a-functional-medicine-approach-to-pheochromocytoma

  A patient-centered approach to pheochromocytoma takes a personalized look at factors that impact an individual’s mind, body, and spiritual health to optimize quality of life. […] While each person’s treatment plan will depend on several personalized factors and complete resolution of pheochromocytoma symptoms usually requires intervention to shrink or remove the tumor, such as surgery, additional strategies may be helpful for managing common symptoms of pheochromocytoma, such as high blood pressure, headaches, and sweating. […] Long-term management of adrenal tumors is important since recurrence can occur years after the initial tumor. Regular monitoring and follow-up in managing pheochromocytoma are needed to detect potential recurrence or metastasis. […] Functional medicine provides a holistic approach to health management post-treatment of pheochromocytoma to optimize quality of life and health in all areas.

• #53 Phaeochromocytoma Treatment – Endocrinesurgery.net.au

  http://www.endocrinesurgery.net.au/phaeochromocytoma-treatment/

  The adrenalectomy can generally be achieved by laparoscopic (keyhole) surgery, except in the case of very large tumours or those suspected to be malignant, when open operation is safer. […] To avoid the release of any catecholamines into the systemic circulation, the priority of the surgeon is to isolate the adrenal vein with as little manipulation of the gland as possible, to prevent release of a massive dose of catecholamines into the bloodstream and precipitation of a hypertensive crisis. […] As a result of surgery 75% can expect a normalisation of blood pressure, with the remission of hypertensive paroxysms. […] In the case of malignant tumours the 5-year survival rate is 44%. […] Although surgery has a high success rate, all patients should attend annual follow up appointments to have urinary catecholamine levels checked so that any further tumours may be detected.

• #54 Pheochromocytoma Treatment & Management: Approach Considerations, Laparoscopic Adrenalectomy, Pheochromocytoma in Pregnancy

  https://emedicine.medscape.com/article/124059-treatment

  Test plasma free metanephrines 2 weeks postoperatively. If results are within the reference range, resection is deemed complete; in such cases, patient survival approaches age-matched controls. In addition, ensure resolution of the hypertension and any associated complications. […] For surgical follow-up, obtain plasma metanephrine levels yearly for 10 years. Ensure that blood pressure is under control. In patients with an underlying genetic mutation, lifelong follow-up is mandatory. […] A literature review by Holscher et al indicated that following curative adrenalectomy, the pooled recurrence rate for sporadic pheochromocytomas is 3%. The weighted mean time to recurrence in the study was 49.4 months, with the weighted mean follow-up period being 77.3 months. […] Surgical mortality rates are less than 2-3% when the operation is performed by a surgeon and an anesthesiologist who are experienced.

• #55 Pheochromocytoma: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/23373-pheochromocytoma

  The best treatment option is surgery, when feasible. […] Treatment options for pheochromocytoma depend on several factors, including: The size of the tumor. If the tumor is benign (not cancer) or malignant (cancer). If you have symptoms of catecholamines that are higher than normal. If the tumor is in one area only or has spread to other places in your body (metastasized). If the tumor has been diagnosed for the first time or has come back (recurred). […] If you have pheochromocytoma that causes symptoms due to excess adrenal hormones, your healthcare provider will likely recommend medication to manage the symptoms. Medications may include: Medication that keeps your blood pressure normal, such as alpha-blockers. Medication that keeps your heart rate normal, such as beta-blockers. Medication that blocks the effect of the excess hormones released by your adrenal gland(s).

• #56 Pheochromocytoma – Knowledge @ AMBOSS

  https://www.amboss.com/us/knowledge/pheochromocytoma/

  Surgical resection is the treatment of choice. Preoperative alpha-adrenergic blockade (e.g., phenoxybenzamine) is indicated to prevent intraoperative complications such as hypertensive crises. […] Multidisciplinary care at a specialized center is recommended. Surgical tumor resection is the only curative treatment. Preoperative blood pressure management and postoperative monitoring for adrenal crisis are essential. […] Treat hypertensive crisis due to pheochromocytoma with IV antihypertensives that target catecholamine excess (e.g., phentolamine, clevidipine, or nicardipine) and avoid beta blockers. […] Adrenalectomy: treatment of choice for pheochromocytoma. Subtotal (organ sparing) adrenalectomy: removal of the part of the gland containing the tumor. Total (radical) adrenalectomy: removal of the entire adrenal gland.

• #57 Pheochromocytoma – Endocrinology Advisor

  https://www.endocrinologyadvisor.com/ddi/pheochromocytoma/

  The primary pheochromocytoma treatment is surgical resection. The catecholamine effect must be blocked before surgery in all patients to prevent hypertensive crises and arrhythmias. Any alpha-adrenoceptor antagonist, calcium-channel blocker, or angiotensin-receptor blocker can be used, with no preference for one option over another. […] Beta-adrenergic blockers should never be started before adequate alpha-adrenergic blockade has been achieved, due to the risk for further elevations in blood pressure. The surgical technique will vary based on the specific circumstances of the case, although a laparoscopic procedure is preferred. Surgery is usually curative for non-metastatic tumors, and the risk of operative mortality is extremely low if performed by an experienced surgical team. […] Surgical resection is the malignant pheochromocytoma treatment of choice, with targeted therapies, radiation therapy, and chemotherapy being alternative options for metastasized tumors if surgery is contraindicated or unsuccessful.

• #58 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  Minimally-invasive procedures such as radiofrequency ablation, cryoablation, and ethanol injection may be considered in the treatment of metastatic PCC/PGL, especially in oligo-metastatic disease. […] Adequate blood pressure control with alpha adrenoceptor blockade at least 10–14 days prior to surgery is essential in functioning PCCs/PGLs to prevent severe cardiovascular events during surgery. […] In palliative scenarios, alpha adrenoceptor blockade should also be considered—balanced against side effects—to alleviate hormonal symptoms and prevent complications from catecholamine excess. […] It has been conventional to use phenoxybenzamine at starting doses approximating 10 mg 2–3x per day, although other similar drugs such as doxazosin and prazosin have been used. […] There is no clear evidence for the superiority of one alpha-blocker for the pre-operative blockade of PCC/PGL patients.

• #59 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  Nevertheless, perioperative hypertension seems to be slightly better controlled with phenoxybenzamine (especially in those patients with high catecholamine or metanephrine levels), although with more pronounced postoperative hypotension. […] Indeed, there were fewer side effects in the doxazosin group. […] Moreover, in functioning metastatic PCCs/PGLs, pre-treatment alpha blockade is recommended prior to initiation of therapy to prevent symptomatic catecholamine release in response to locoregional or systemic treatment. […] For metastatic PCCs/PGLs, there are few established treatment options but radiotherapy ([131I]MIBG therapy, recently PRRT) as well as classic chemotherapy (Averbuch scheme, and temozolomide), and different targeted therapy options, have been extensively used outside of controlled clinical trials.

• #60 Pheochromocytoma – Endocrine and Metabolic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/endocrine-and-metabolic-disorders/adrenal-disorders/pheochromocytoma

  Surgical removal is the treatment of choice. The operation is usually delayed until hypertension is controlled by a combination of alpha-blockers and beta-blockers (usually phenoxybenzamine 20 to 40 mg orally 3 times a day and propranolol 20 to 40 mg orally 3 times a day). […] The blood pressure target is 130/80 mmHg; some suggest the target should also include achieving a certain postural drop in blood pressure, but this is not essential. Re-equilibration of the cardiovascular system requires approximately 10 to 14 days, after which blockade is presumed to be effective. Beta-blockers should not be used until adequate alpha-blockade has been achieved. Some alpha-blockers, such as doxazosin, may be equally effective but better tolerated. […] Nitroprusside can be infused for hypertensive crises preoperatively or intraoperatively.

• #61 Pheochromocytoma – Endocrine and Metabolic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/endocrine-and-metabolic-disorders/adrenal-disorders/pheochromocytoma

  Surgical removal is the treatment of choice. The operation is usually delayed until hypertension is controlled by a combination of alpha-blockers and beta-blockers (usually phenoxybenzamine 20 to 40 mg orally 3 times a day and propranolol 20 to 40 mg orally 3 times a day). […] The blood pressure target is 130/80 mmHg; some suggest the target should also include achieving a certain postural drop in blood pressure, but this is not essential. Re-equilibration of the cardiovascular system requires approximately 10 to 14 days, after which blockade is presumed to be effective. Beta-blockers should not be used until adequate alpha-blockade has been achieved. Some alpha-blockers, such as doxazosin, may be equally effective but better tolerated. […] Nitroprusside can be infused for hypertensive crises preoperatively or intraoperatively.

• #62 Phaeochromocytoma | Endocrine Conditions

  https://www.yourhormones.info/endocrine-conditions/phaeochromocytoma/

  Patients will normally be placed on medications to block the effects of excess hormones. These are called alpha-blockers (phenoxybenzamine, doxazosin, prazosin, terazosin) and beta-blockers (propranolol, labetalol). […] Surgical removal of the tumour is the best curative treatment. A patient may need to take tablets for several days prior to surgery to ensure the blood pressure is well controlled. Surgery should be carried out by a surgeon and anaesthetist experienced in operating on this type of tumour and in centres where there is a multidisciplinary endocrine team that deals with these tumours. […] If the tumour has spread or surgery cannot be undertaken or any residual tumor after surgery, then other treatments include chemotherapy and/or a radioactive form of treatment called radiolabelled MIBG.

• #63

  https://link.springer.com/article/10.1007/s12020-013-0007-y

  Pheochromocytoma (PH) and paraganglioma (PG) are neuroendocrine neoplasms arising from chromaffin cells of the adrenal medulla and the sympathetic ganglia, respectively. […] HT treatment may be difficult when PH or PG occurs in pregnancy or in the elderly subjects and in these cases a multidisciplinary team is required. When surgical excision is mandatory the perioperative management requires the administration of selective 1-adrenergic blocking agents (i.e., doxazosin, prazosin or terazosin) followed by a -adrenergic blockade (i.e., propranolol, atenolol). […] Although labetalol is traditionally considered the ideal agent due to its – and -adrenergic antagonism, experimental studies do not support its use in this clinical setting. As second regimen, the administration of vasodilators as calcium channel blockers (i.e., nicardipine, nifedipine) may be required to control BP. […] Oral and sublingual short-acting nifedipine are potentially dangerous in patients with hypertensive emergencies and are not recommend. The latest evidences into the diagnosis and treatment of hypertensive crisis due to PH and PG are reviewed here.

• #64 Pheochromocytoma – Endocrine and Metabolic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/endocrine-and-metabolic-disorders/adrenal-disorders/pheochromocytoma

  Surgical removal is the treatment of choice. The operation is usually delayed until hypertension is controlled by a combination of alpha-blockers and beta-blockers (usually phenoxybenzamine 20 to 40 mg orally 3 times a day and propranolol 20 to 40 mg orally 3 times a day). […] The blood pressure target is 130/80 mmHg; some suggest the target should also include achieving a certain postural drop in blood pressure, but this is not essential. Re-equilibration of the cardiovascular system requires approximately 10 to 14 days, after which blockade is presumed to be effective. Beta-blockers should not be used until adequate alpha-blockade has been achieved. Some alpha-blockers, such as doxazosin, may be equally effective but better tolerated. […] Nitroprusside can be infused for hypertensive crises preoperatively or intraoperatively.

• #65 Pheochromocytoma – Endocrine and Metabolic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/endocrine-and-metabolic-disorders/adrenal-disorders/pheochromocytoma

  Surgical removal is the treatment of choice. The operation is usually delayed until hypertension is controlled by a combination of alpha-blockers and beta-blockers (usually phenoxybenzamine 20 to 40 mg orally 3 times a day and propranolol 20 to 40 mg orally 3 times a day). […] The blood pressure target is 130/80 mmHg; some suggest the target should also include achieving a certain postural drop in blood pressure, but this is not essential. Re-equilibration of the cardiovascular system requires approximately 10 to 14 days, after which blockade is presumed to be effective. Beta-blockers should not be used until adequate alpha-blockade has been achieved. Some alpha-blockers, such as doxazosin, may be equally effective but better tolerated. […] Nitroprusside can be infused for hypertensive crises preoperatively or intraoperatively.

• #66 Pheochromocytoma treatment – USZ

  https://www.usz.ch/en/department/endocrinology/service/pheochromocytoma-treatment/

  For tumors that cannot be operated on and cause symptoms, long-term therapy with alpha blockers is a possible adjuvant therapy. […] A nuclear medicine procedure in which we use radioactive iodine or somatostatin analogs as a radiation source. […] To date, there is little data on the efficacy of cytotoxic agents (cytostatics, chemotherapeutics) in pheochromocytomas. […] Researchers are also working on the development of new drugs, such as multi-tyrosine kinase inhibitors. However, the drugs are still being tested in trials. […] All patients with a pheochromocytoma should have an annual follow-up examination by an endocrinologist in order to detect and treat a recurring tumor at an early stage.

• #67 Pheochromocytoma treatment – USZ

  https://www.usz.ch/en/department/endocrinology/service/pheochromocytoma-treatment/

  For tumors that cannot be operated on and cause symptoms, long-term therapy with alpha blockers is a possible adjuvant therapy. […] A nuclear medicine procedure in which we use radioactive iodine or somatostatin analogs as a radiation source. […] To date, there is little data on the efficacy of cytotoxic agents (cytostatics, chemotherapeutics) in pheochromocytomas. […] Researchers are also working on the development of new drugs, such as multi-tyrosine kinase inhibitors. However, the drugs are still being tested in trials. […] All patients with a pheochromocytoma should have an annual follow-up examination by an endocrinologist in order to detect and treat a recurring tumor at an early stage.

• #68 Pheochromocytoma – Endocrine and Metabolic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/endocrine-and-metabolic-disorders/adrenal-disorders/pheochromocytoma

  When bilateral tumors are documented or suspected (as in a patient with multiple endocrine neoplasia [MEN]), sufficient hydrocortisone (100 mg IV twice a day) given before and during surgery avoids acute glucocorticoid insufficiency due to bilateral adrenalectomy. […] Most pheochromocytomas can be removed laparoscopically. Blood pressure must be continuously monitored via an intra-arterial catheter, and volume status is closely monitored. […] Malignant metastatic pheochromocytoma should be treated with alpha-blockers and beta-blockers. The tumor may be indolent and survival long-lasting. However, even with rapid tumor growth, blood pressure can be controlled. I-131 MIBG or more recently lutetium-177 dotatate can help relieve symptoms in patients with residual disease. Metyrosine, a tyrosine hydroxylase inhibitor, may be used to decrease catecholamine production in patients whose blood pressure is difficult to control. Radiation therapy may reduce bone pain due to metastases. Chemotherapy is rarely effective, but the most common regimen tried is the combination of cyclophosphamide, vincristine, and dacarbazine. The chemotherapy agent temozolomide and targeted therapy with sunitinib or possibly cabozantinib may also be effective. Belzutifan, a hypoxia-inducible factor inhibitor used in the treatment of associated cancers in patients with von Hippel-Lindau disease, is currently undergoing clinical trials in patients with malignant metastatic pheochromocytoma.

• #69 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  However, currently, a fixed empiric dose is applied in analogy to the NETTER-1 trial in most centers and dosimetry is not a standard procedure. […] Interestingly, there are few or no data on the use of unlabeled long-acting somatostatin analogs, such as octreotide LAR or lanreotide autogel, in therapy. […] These are administered once a month, and in patients with other forms of neuroendocrine tumors (NETs), especially pancreatic and midgut NETs, they cannot only inhibit hormonal secretion but, according to two large-scale trials, attenuate tumor progression. […] As such agents are usually well-tolerated with generally mild adverse effects, they may be trialed in patients with the aim of lowering catecholamine secretion and possibly stabilizing tumor growth, especially when 68Ga[Ga]-DOTATATE PET/CT is positive.

• #70 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  In the case of progression after chemotherapy or radionuclide therapy, or if chemotherapy or radionuclide therapy are not possible/tolerated by the patient, the TKIs sunitinib (37.5 mg or 50 mg daily) or cabozantinib (60 mg daily, or titrated down to a tolerable dose) may be considered, especially for RET and SDHx-mutation carriers. […] Several targeted therapies (PI3K inhibitors in combination with mTORC1 inhibitors, HIF-2α inhibitors, PARP inhibitors, SSTR2 analogs, HDAC inhibitors, DNA demethylating agents) and immunotherapy are currently under investigation and may have strong potential for future personalized therapy approaches.

• #71 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  In the case of progression after chemotherapy or radionuclide therapy, or if chemotherapy or radionuclide therapy are not possible/tolerated by the patient, the TKIs sunitinib (37.5 mg or 50 mg daily) or cabozantinib (60 mg daily, or titrated down to a tolerable dose) may be considered, especially for RET and SDHx-mutation carriers. […] Several targeted therapies (PI3K inhibitors in combination with mTORC1 inhibitors, HIF-2α inhibitors, PARP inhibitors, SSTR2 analogs, HDAC inhibitors, DNA demethylating agents) and immunotherapy are currently under investigation and may have strong potential for future personalized therapy approaches.

• #72 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  In the case of progression after chemotherapy or radionuclide therapy, or if chemotherapy or radionuclide therapy are not possible/tolerated by the patient, the TKIs sunitinib (37.5 mg or 50 mg daily) or cabozantinib (60 mg daily, or titrated down to a tolerable dose) may be considered, especially for RET and SDHx-mutation carriers. […] Several targeted therapies (PI3K inhibitors in combination with mTORC1 inhibitors, HIF-2α inhibitors, PARP inhibitors, SSTR2 analogs, HDAC inhibitors, DNA demethylating agents) and immunotherapy are currently under investigation and may have strong potential for future personalized therapy approaches.

• #73 Management of Pheochromocytoma | Endocrine Society

  https://www.endocrine.org/journals/endocrine-reviews/management-of-pheochromocytoma

  Pheochromocytomas/paragangliomas are characterized by a unique molecular landscape that allows their assignment to clusters based on underlying genetic alterations. […] In this review, we explore and explain why cluster-specific (personalized) management of pheochromocytoma/paraganglioma is essential to ascertain clinical behavior and prognosis, guide individual diagnostic procedures (biochemical interpretation, choice of the most sensitive imaging modalities), and provide personalized management and follow-up. […] Although cluster-specific therapy of inoperable/metastatic disease has not yet entered routine clinical practice, we suggest that informed personalized genetic-driven treatment should be implemented as a logical next step. This review amalgamates published guidelines and expert views within each cluster for a coherent individualized patient management plan.

• #74 Advances in the management of pheochromocytoma – a short review | Miciak | Nowotwory. Journal of Oncology

  https://journals.viamedica.pl/nowotwory_journal_of_oncology/article/view/96815

  Surgical treatment seems to be associated with a relatively low 5-year survival rate (45%). […] The preferred method is laparoscopy due to the lower invasiveness of the procedure, but the presence and nature of metastatic lesions most often require modification of the procedure to laparotomy. […] Treatment with somatostatin analogs like Yttrium-90-DOTATOC (90Y-DOTATOC) and lutetium-177-DOTA0-Tyr3-octreotate (177Lu-DOTATATE) has become one of the radiotherapeutic approaches. […] Another treatment method is the use of chemotherapeutics, especially in patients refractory to radiotherapy. […] The known molecular pathway responsible for the pathogenesis of pheochromocytoma has allowed the use of inhibitors of its individual substrates. […] The latest treatment direction for pheochromocytoma was published by Tabebi et al. The target point of the therapy is suggested to be striking the nuclear and mitochondrial genetic material of the neoplasm cells. […] The management of patients with pheochromocytoma is reviewed in figure 1.

• #75 Advances in the management of pheochromocytoma – a short review | Miciak | Nowotwory. Journal of Oncology

  https://journals.viamedica.pl/nowotwory_journal_of_oncology/article/view/96815

  Surgical treatment seems to be associated with a relatively low 5-year survival rate (45%). […] The preferred method is laparoscopy due to the lower invasiveness of the procedure, but the presence and nature of metastatic lesions most often require modification of the procedure to laparotomy. […] Treatment with somatostatin analogs like Yttrium-90-DOTATOC (90Y-DOTATOC) and lutetium-177-DOTA0-Tyr3-octreotate (177Lu-DOTATATE) has become one of the radiotherapeutic approaches. […] Another treatment method is the use of chemotherapeutics, especially in patients refractory to radiotherapy. […] The known molecular pathway responsible for the pathogenesis of pheochromocytoma has allowed the use of inhibitors of its individual substrates. […] The latest treatment direction for pheochromocytoma was published by Tabebi et al. The target point of the therapy is suggested to be striking the nuclear and mitochondrial genetic material of the neoplasm cells. […] The management of patients with pheochromocytoma is reviewed in figure 1.

• #76 Pheochromocytoma – Endocrine and Metabolic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/endocrine-and-metabolic-disorders/adrenal-disorders/pheochromocytoma

  When bilateral tumors are documented or suspected (as in a patient with multiple endocrine neoplasia [MEN]), sufficient hydrocortisone (100 mg IV twice a day) given before and during surgery avoids acute glucocorticoid insufficiency due to bilateral adrenalectomy. […] Most pheochromocytomas can be removed laparoscopically. Blood pressure must be continuously monitored via an intra-arterial catheter, and volume status is closely monitored. […] Malignant metastatic pheochromocytoma should be treated with alpha-blockers and beta-blockers. The tumor may be indolent and survival long-lasting. However, even with rapid tumor growth, blood pressure can be controlled. I-131 MIBG or more recently lutetium-177 dotatate can help relieve symptoms in patients with residual disease. Metyrosine, a tyrosine hydroxylase inhibitor, may be used to decrease catecholamine production in patients whose blood pressure is difficult to control. Radiation therapy may reduce bone pain due to metastases. Chemotherapy is rarely effective, but the most common regimen tried is the combination of cyclophosphamide, vincristine, and dacarbazine. The chemotherapy agent temozolomide and targeted therapy with sunitinib or possibly cabozantinib may also be effective. Belzutifan, a hypoxia-inducible factor inhibitor used in the treatment of associated cancers in patients with von Hippel-Lindau disease, is currently undergoing clinical trials in patients with malignant metastatic pheochromocytoma.

• #77 Pheochromocytoma: Staging and Treatment | OncoLink

  https://www.oncolink.org/cancers/pheochromocytoma/pheochromocytoma-staging-and-treatment

  There are different ways to treat your pheochromocytoma depending on if it is cancerous, has metastasized, and your overall health. There is not much research about treatments, but many options are available. Ask your provider what options are best for you. Your treatment options may be some of these: […] Surgery is often used whether the tumor is benign (non-cancerous) or malignant (cancerous). Surgery for pheochromocytoma may mean taking out the whole tumor and adrenal gland or the tumor and only part of the adrenal gland. This is called an adrenalectomy. If the cancer has spread to other parts of the body, those organs and tissues may also be taken out. […] Radiation therapy uses high-energy x-rays to kill cancer cells. External beam radiotherapy can be used for patients who have malignant pheochromocytoma that has spread to the bone. The goal is to ease symptoms such as pain.

• #78 Pheochromocytoma: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/23373-pheochromocytoma

  Treatment options for pheochromocytoma include: Surgery. Radiation therapy. Chemotherapy. Ablation therapy. Embolization therapy. Targeted therapy. […] Surgery is the main form of treatment for pheochromocytoma. Approximately 90% of pheochromocytomas are successfully removed by surgery. […] After surgery, your provider will check the catecholamine levels in your blood or urine. Normal catecholamine levels are a sign that all the pheochromocytoma cells were removed. […] Radiation therapy is a cancer treatment that focuses strong beams of energy to destroy cancer cells or keep them from growing while sparing as much surrounding healthy tissue as possible. […] Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells by killing the cells or by preventing them from dividing and multiplying.

• #79 Pheochromocytoma and Paraganglioma Treatment – NCI

  https://www.cancer.gov/types/pheochromocytoma/patient/pheochromocytoma-treatment-pdq

  Pheochromocytoma and paraganglioma are described as localized, regional, or metastatic. […] There are different types of treatment for patients with pheochromocytoma or paraganglioma. […] Patients receive medication to treat the signs and symptoms of pheochromocytoma and paraganglioma. […] The following types of treatment are used: Surgery, Radiation therapy, Chemotherapy, Ablation therapy, Embolization therapy, Targeted therapy. […] Drug therapy begins when pheochromocytoma or paraganglioma is diagnosed. […] Surgery to remove pheochromocytoma is usually an adrenalectomy (removal of one or both adrenal glands). […] Radiation therapy uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. […] Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing.

• #80 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  Moreover, several studies have shown that surgical resection of the primary tumor is associated with improved survival even with metastatic disease. […] In addition, complete metastatic surgery may be considered in oligo-metastatic PCC/PGL on a case-by-case decision, although there is only little evidence for such an approach from single case reports. […] Watchful waiting with frequent follow-up may be the optimal initial approach in patients with non-functioning HN PGL, especially without evidence of significant tumor growth and/or compression of surrounding structures. […] Conventional external beam radiation therapy (cEBRT) or radiosurgery (gamma-knife/cyberknife) are well-established methods in the case of bone metastases and also may play a significant palliative role in oligo-metastatic scenarios.

• #81 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  Surgery is always the therapy of choice of non-metastatic PCC/PGL, whenever possible. […] However, surgery of non-functioning HN PGLs has to be carefully balanced against surgery-related morbidities, especially for the cranial nerves for vagal and jugular PGLs. […] In cases of a high risk related to surgery, radiotherapy/radiosurgery (gamma-knife/cyberknife) might be a less invasive option with non-curative but controlling outcomes. […] In patients with hereditary PCCs, cortical sparing surgery should always be considered since there is frequently a high risk of bilateral PCCs in hereditary disease, and cortical-sparing surgery was not associated with decreased survival despite PCC recurrence in 13% of cases in a very recently published study. […] With metastatic disease, primary tumor resection should be recommended if feasible in order to alleviate cardiovascular and other symptoms from catecholamine excess or from tumor invasion, and to minimize the target for radiopharmaceutical therapies.

• #82 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  The only officially (FDA)-approved treatment option in the US is high-specific activity (HSA) [131I]MIBG therapy. […] The best investigated and well established therapy for metastatic PGL/PCC is [131I]MIBG therapy, which is preferentially recommended for slow-growing [123/131I]MIBG positive metastatic PCCs/PGLs. […] In a meta-analysis of 17 studies with 243 PCC/PGL patients on [131I]MIBG therapy, there was a complete response in 3% of patients, a partial response in 27% of patients and stable disease in 52% of patients: 40% of patients showed a partial hormonal response. […] The most common side effects are anorexia, nausea, vomiting, and hematologic toxicity with grade 3–4 neutropenia in 87% of patients and grade 3–4 thrombocytopenia in 83%. […] However, in long-term survivors there is a risk of myelodysplastic disorders.

• #83 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  The only officially (FDA)-approved treatment option in the US is high-specific activity (HSA) [131I]MIBG therapy. […] The best investigated and well established therapy for metastatic PGL/PCC is [131I]MIBG therapy, which is preferentially recommended for slow-growing [123/131I]MIBG positive metastatic PCCs/PGLs. […] In a meta-analysis of 17 studies with 243 PCC/PGL patients on [131I]MIBG therapy, there was a complete response in 3% of patients, a partial response in 27% of patients and stable disease in 52% of patients: 40% of patients showed a partial hormonal response. […] The most common side effects are anorexia, nausea, vomiting, and hematologic toxicity with grade 3–4 neutropenia in 87% of patients and grade 3–4 thrombocytopenia in 83%. […] However, in long-term survivors there is a risk of myelodysplastic disorders.

• #84 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  The only officially (FDA)-approved treatment option in the US is high-specific activity (HSA) [131I]MIBG therapy. […] The best investigated and well established therapy for metastatic PGL/PCC is [131I]MIBG therapy, which is preferentially recommended for slow-growing [123/131I]MIBG positive metastatic PCCs/PGLs. […] In a meta-analysis of 17 studies with 243 PCC/PGL patients on [131I]MIBG therapy, there was a complete response in 3% of patients, a partial response in 27% of patients and stable disease in 52% of patients: 40% of patients showed a partial hormonal response. […] The most common side effects are anorexia, nausea, vomiting, and hematologic toxicity with grade 3–4 neutropenia in 87% of patients and grade 3–4 thrombocytopenia in 83%. […] However, in long-term survivors there is a risk of myelodysplastic disorders.

• #85 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  The only officially (FDA)-approved treatment option in the US is high-specific activity (HSA) [131I]MIBG therapy. […] The best investigated and well established therapy for metastatic PGL/PCC is [131I]MIBG therapy, which is preferentially recommended for slow-growing [123/131I]MIBG positive metastatic PCCs/PGLs. […] In a meta-analysis of 17 studies with 243 PCC/PGL patients on [131I]MIBG therapy, there was a complete response in 3% of patients, a partial response in 27% of patients and stable disease in 52% of patients: 40% of patients showed a partial hormonal response. […] The most common side effects are anorexia, nausea, vomiting, and hematologic toxicity with grade 3–4 neutropenia in 87% of patients and grade 3–4 thrombocytopenia in 83%. […] However, in long-term survivors there is a risk of myelodysplastic disorders.

• #86 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  One problem with conventional [131I]MIBG therapy is the relatively low specific activity of the radiopharmaceutical (most of the MIBG molecules are not 131I-labeled) potentially leading to less uptake into the tumor and life-threatening side-effects. […] Very recently, high-specific activity (HSA) [131I]MIBG that consists almost entirely of 131I-labeled molecules has been developed. […] A recent multicentric phase II study led to the FDA-approval of HSA [131I]MIBG (Ultratrace, Azedra) in the United States. […] The most common treatment-related side effects were nausea, myelosuppression, and fatigue, with a higher rate of hematologic toxicity compared to conventional [131I]MIBG-therapy. […] In order to maximize radionuclide uptake in the tumor with minimal risk to organs at risk (especially the kidney), an internal patient-specific dosimetry prior to PRRT is a potential future approach (personalized PRRT).

• #87 Pheochromocytoma survivor achieves remission through targeted therapy clinical trial | MD Anderson Cancer Center

  https://www.mdanderson.org/cancerwise/pheochromocytoma-survivor-achieves-remission-through-targeted-therapy.h00-159464001.html

  Today, Susan is almost completely back to normal. Surgery plus a clinical trial she joined at MD Anderson put her in remission within a year, resolving virtually all of her symptoms. […] To treat Susans cancer, Jimenez recommended surgery to remove the primary tumor, along with a part of her kidney and spleen. Then, he suggested a clinical trial he was leading that involved a radioactive form of iodine called ultratrace iobenguane (Azedra). The medication would be absorbed by any remaining cancer and concentrated inside of its cells, ultimately leading to their destruction. […] Susan had the surgery under the late urologic oncologist Christopher Wood, M.D., on July 21, 2015. She received her first infusion of Azedra in October 2015, and her second dose in January 2016. […] Jimenez notes that only 60% to 70% of patients with pheochromocytoma have the MIBG receptor, so there is still work to be done. But in 2018, ultratrace iobenguane became the only drug approved by the Food and Drug Administration (FDA) for the treatment of pheochromocytoma. […] Before this, we didnt have any effective treatment options, he says. And Susan is still doing well after getting this medicine only twice, six years ago. That’s a very impressive result.

• #88 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  One problem with conventional [131I]MIBG therapy is the relatively low specific activity of the radiopharmaceutical (most of the MIBG molecules are not 131I-labeled) potentially leading to less uptake into the tumor and life-threatening side-effects. […] Very recently, high-specific activity (HSA) [131I]MIBG that consists almost entirely of 131I-labeled molecules has been developed. […] A recent multicentric phase II study led to the FDA-approval of HSA [131I]MIBG (Ultratrace, Azedra) in the United States. […] The most common treatment-related side effects were nausea, myelosuppression, and fatigue, with a higher rate of hematologic toxicity compared to conventional [131I]MIBG-therapy. […] In order to maximize radionuclide uptake in the tumor with minimal risk to organs at risk (especially the kidney), an internal patient-specific dosimetry prior to PRRT is a potential future approach (personalized PRRT).

• #89 Pheochromocytoma: Staging and Treatment | OncoLink

  https://www.oncolink.org/cancers/pheochromocytoma/pheochromocytoma-staging-and-treatment

  Meta-iodo-benzylguanidine (MIBG), can be used to treat pheochromocytoma. MIBG has small amounts of radioactive material in it, which collect in the tumor, causing it to light up on a scan. […] Peptide receptor radionuclide therapy (PRRT) combines a medication (Lutetium 177 (Lu-177)) that targets cancer cells with a radioactive substance. This delivers radiation directly to the cancer cells. […] Targeted therapies stop or slow the growth of a tumor by stopping a specific molecule needed for cancer growth. Molecular-targeted therapies, such as sunitinib, can be used after surgery if there is a high risk of cancer coming back (called recurrence). […] Chemotherapy are medications that are often given intravenously (IV, into a vein) or in pill form. Chemotherapy travels through the bloodstream and throughout the body to kill cancer cells. For pheochromocytoma, chemotherapy is not used as a primary treatment, but as an option for malignant pheochromocytoma that causes symptoms and that has not responded to MIBG therapy. […] You may be offered a clinical trial as part of your treatment plan.

• #90 Pheochromocytoma – Wikipedia

  https://en.wikipedia.org/wiki/Pheochromocytoma

  Metastatic pheochromocytoma is best managed with a multidisciplinary team of oncologists, surgeons, radiologists, nuclear medicine physicians, and endocrinologists. […] Normally, the goal of surgery is complete cytoreductive surgery; however, with widespread metastatic disease, this is not always feasible. […] Radiation techniques are primarily used for pain control, specifically with regards to bone metastases, local control of the disease, and to limit spinal cord compression. […] The most common chemotherapy regimen for metastatic pheochromocytoma is cyclophosphamide, vincristine, and dacarbazine, collectively known as CVD. […] Iodine-131 meta-iodobenzylguanidine (MIBG) is not only useful in locating the presence of metastatic disease, but also as an available treatment modality. […] Peptide Receptor Radionuclide Therapy (PRRT) utilizes the 68-Ga DOTA analogues mentioned above in the functional imaging section.

• #91 Treatment for phaeochromocytomas | Adrenal gland cancer | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/adrenal-gland-cancer/phaeochromocytoma/treatment

  The main treatment for phaeochromocytoma is surgery. Other treatments include radioisotope therapy, external radiotherapy and chemotherapy. […] You usually have an operation to remove the adrenal gland. This is called an adrenalectomy. It will cure most people with phaeochromocytoma. […] If you can’t have surgery for phaeochromocytoma, you might have radioisotope therapy instead. […] You might be able to have surgery if the phaeochromocytoma has come back in the nearby area. […] Sometimes doctors recommend surgery to remove phaeochromocytoma that has spread to other parts of the body. […] Radioisotope therapy uses radioactive medicines to treat some types of cancer. […] For this treatment, the chemical MIBG is attached to a radioactive form of iodine called iodine-131 (I-131). […] DOTATATE is another chemical that is taken up by many phaeochromocytomas.

• #92 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  One problem with conventional [131I]MIBG therapy is the relatively low specific activity of the radiopharmaceutical (most of the MIBG molecules are not 131I-labeled) potentially leading to less uptake into the tumor and life-threatening side-effects. […] Very recently, high-specific activity (HSA) [131I]MIBG that consists almost entirely of 131I-labeled molecules has been developed. […] A recent multicentric phase II study led to the FDA-approval of HSA [131I]MIBG (Ultratrace, Azedra) in the United States. […] The most common treatment-related side effects were nausea, myelosuppression, and fatigue, with a higher rate of hematologic toxicity compared to conventional [131I]MIBG-therapy. […] In order to maximize radionuclide uptake in the tumor with minimal risk to organs at risk (especially the kidney), an internal patient-specific dosimetry prior to PRRT is a potential future approach (personalized PRRT).

• #93 Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

  https://www.mdpi.com/2072-6694/11/10/1505

  However, currently, a fixed empiric dose is applied in analogy to the NETTER-1 trial in most centers and dosimetry is not a standard procedure. […] Interestingly, there are few or no data on the use of unlabeled long-acting somatostatin analogs, such as octreotide LAR or lanreotide autogel, in therapy. […] These are administered once a month, and in patients with other forms of neuroendocrine tumors (NETs), especially pancreatic and midgut NETs, they cannot only inhibit hormonal secretion but, according to two large-scale trials, attenuate tumor progression. […] As such agents are usually well-tolerated with generally mild adverse effects, they may be trialed in patients with the aim of lowering catecholamine secretion and possibly stabilizing tumor growth, especially when 68Ga[Ga]-DOTATATE PET/CT is positive.

• #94 Pheochromocytoma and Paraganglioma Symptoms and Treatment | Saint John’s Cancer Institute

  https://www.saintjohnscancer.org/endocrine/conditions/pheochromocytoma-and-paraganglioma/

  […] […] Pheochromocytomas and paragangliomas can come back either in the same area, the other adrenal gland, or in other areas in the body. All patients need lifelong follow-up with an endocrinologist and possibly your surgeon to monitor for recurrence. […] This will be done with blood tests to measure hormone levels. Additionally, all patients should be referred for genetic testing.

• #95 Pheochromocytoma Treatment & Management: Approach Considerations, Laparoscopic Adrenalectomy, Pheochromocytoma in Pregnancy

  https://emedicine.medscape.com/article/124059-treatment

  Test plasma free metanephrines 2 weeks postoperatively. If results are within the reference range, resection is deemed complete; in such cases, patient survival approaches age-matched controls. In addition, ensure resolution of the hypertension and any associated complications. […] For surgical follow-up, obtain plasma metanephrine levels yearly for 10 years. Ensure that blood pressure is under control. In patients with an underlying genetic mutation, lifelong follow-up is mandatory. […] A literature review by Holscher et al indicated that following curative adrenalectomy, the pooled recurrence rate for sporadic pheochromocytomas is 3%. The weighted mean time to recurrence in the study was 49.4 months, with the weighted mean follow-up period being 77.3 months. […] Surgical mortality rates are less than 2-3% when the operation is performed by a surgeon and an anesthesiologist who are experienced.

• #96 Pheochromocytoma Treatment & Management: Approach Considerations, Laparoscopic Adrenalectomy, Pheochromocytoma in Pregnancy

  https://emedicine.medscape.com/article/124059-treatment

  Test plasma free metanephrines 2 weeks postoperatively. If results are within the reference range, resection is deemed complete; in such cases, patient survival approaches age-matched controls. In addition, ensure resolution of the hypertension and any associated complications. […] For surgical follow-up, obtain plasma metanephrine levels yearly for 10 years. Ensure that blood pressure is under control. In patients with an underlying genetic mutation, lifelong follow-up is mandatory. […] A literature review by Holscher et al indicated that following curative adrenalectomy, the pooled recurrence rate for sporadic pheochromocytomas is 3%. The weighted mean time to recurrence in the study was 49.4 months, with the weighted mean follow-up period being 77.3 months. […] Surgical mortality rates are less than 2-3% when the operation is performed by a surgeon and an anesthesiologist who are experienced.

• #97 Pheochromocytoma – Endocrinology Advisor

  https://www.endocrinologyadvisor.com/ddi/pheochromocytoma/

  Lifelong monitoring after surgery will be required to ensure that the pheochromocytoma treatment was successful, identify complications, and monitor tumor recurrence. Repeating biochemical tests should be done no sooner than 14 days after surgery. If metanephrine levels are elevated for three months after surgical resection, further imaging is indicated to look for another tumor.

• #98 Pheochromocytoma – Endocrinology Advisor

  https://www.endocrinologyadvisor.com/ddi/pheochromocytoma/

  Lifelong monitoring after surgery will be required to ensure that the pheochromocytoma treatment was successful, identify complications, and monitor tumor recurrence. Repeating biochemical tests should be done no sooner than 14 days after surgery. If metanephrine levels are elevated for three months after surgical resection, further imaging is indicated to look for another tumor.

• #99 Pheochromocytoma and Paraganglioma Treatment (PDQ®): Treatment – Health Professional Information [NCI] – Health Information Library | PeaceHealth

  https://www.peacehealth.org/medical-topics/id/ncicdr0000681246

  Surgical management of pheochromocytoma in patients with the hereditary syndromes multiple endocrine neoplasia type 2 (MEN2) or von Hippel-Lindau (VHL) disease has been controversial. […] If all identifiable disease is resectable, including a limited number of distant metastases, surgery can provide occasional long-term remission. […] Chemotherapy has not been shown to improve survival in patients with metastatic pheochromocytoma; however, chemotherapy may be useful for symptom palliation. […] Iodine I 131 (131I)-MIBG radiation therapy has been used for the treatment of patients with MIBG-avid metastases. […] After resection of a localized pheochromocytoma presumed to represent a benign tumor and documented normal postoperative biochemical testing, disease recurrence occurs in 6.5% to 16.5% of patients, and 50% of patients with disease recurrence develop metastatic disease. […] Palliation of symptoms, including those related to catecholamine excess and local mass effect, is the primary focus of treatment for disease that is not resectable.

• #100 Pheochromocytoma Treatment & Management: Approach Considerations, Laparoscopic Adrenalectomy, Pheochromocytoma in Pregnancy

  https://emedicine.medscape.com/article/124059-treatment

  Test plasma free metanephrines 2 weeks postoperatively. If results are within the reference range, resection is deemed complete; in such cases, patient survival approaches age-matched controls. In addition, ensure resolution of the hypertension and any associated complications. […] For surgical follow-up, obtain plasma metanephrine levels yearly for 10 years. Ensure that blood pressure is under control. In patients with an underlying genetic mutation, lifelong follow-up is mandatory. […] A literature review by Holscher et al indicated that following curative adrenalectomy, the pooled recurrence rate for sporadic pheochromocytomas is 3%. The weighted mean time to recurrence in the study was 49.4 months, with the weighted mean follow-up period being 77.3 months. […] Surgical mortality rates are less than 2-3% when the operation is performed by a surgeon and an anesthesiologist who are experienced.

• #101 Pheochromocytoma and Paraganglioma Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/pheochromocytoma/hp/pheochromocytoma-treatment-pdq

  Surgical management of pheochromocytoma in patients with the hereditary syndromes multiple endocrine neoplasia type 2 (MEN2) or von Hippel-Lindau (VHL) disease has been controversial. […] If all identifiable disease is resectable, including a limited number of distant metastases, surgery can provide occasional long-term remission. […] Chemotherapy has not been shown to improve survival in patients with metastatic pheochromocytoma; however, chemotherapy may be useful for symptom palliation. […] Other palliative treatment modalities include external-beam radiation therapy and embolization, radiofrequency ablation, or cryoablation. […] Treatment for recurrent disease involves appropriate medical management (i.e., alpha-adrenergic blockade) followed by complete surgical resection, when possible. […] Phenoxybenzamine use is safe in pregnancy, but beta-adrenergic blockers should be initiated only if needed because their use has been associated with intrauterine growth restriction.

• #102 Pheochromocytoma and Paraganglioma Treatment (PDQ®): Treatment – Health Professional Information [NCI] | Kaiser Permanente

  https://healthy.kaiserpermanente.org/health-wellness/health-encyclopedia/he.pheochromocytoma-and-paraganglioma-treatment-pdq%C2%AE-treatment-health-professional-information-nci.ncicdr0000681246

  Palliative therapy for recurrent pheochromocytoma includes chemotherapy, targeted therapies, high-dose iodine I 131-metaiodobenzylguanidine radiation therapy, and ablation therapies. […] Phenoxybenzamine is effective for managing symptoms of catecholamine excess, and metyrosine can be added if needed. […] Surgical resection is the definitive treatment for pheochromocytoma or extra-adrenal paraganglioma that is regionally advanced. […] If all identifiable disease is resectable, including a limited number of distant metastases, surgery can provide occasional long-term remission. […] Iodine I 131 (131I)-MIBG radiation therapy has been used for the treatment of patients with MIBG-avid metastases.

• #103 Pheochromocytoma: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/23373-pheochromocytoma

  The best treatment option is surgery, when feasible. […] Treatment options for pheochromocytoma depend on several factors, including: The size of the tumor. If the tumor is benign (not cancer) or malignant (cancer). If you have symptoms of catecholamines that are higher than normal. If the tumor is in one area only or has spread to other places in your body (metastasized). If the tumor has been diagnosed for the first time or has come back (recurred). […] If you have pheochromocytoma that causes symptoms due to excess adrenal hormones, your healthcare provider will likely recommend medication to manage the symptoms. Medications may include: Medication that keeps your blood pressure normal, such as alpha-blockers. Medication that keeps your heart rate normal, such as beta-blockers. Medication that blocks the effect of the excess hormones released by your adrenal gland(s).

• #104 Phaeochromocytoma Treatment – Endocrinesurgery.net.au

  http://www.endocrinesurgery.net.au/phaeochromocytoma-treatment/

  Surgical excision of phaeochromocytoma is the treatment of choice, and for non-malignant tumours the 5-year survival rate is 95%. […] There is no effective medical treatment, but adrenalectomy should not be undertaken without adequate preparation of the patient. […] Blood pressure should be controlled for 10-14 days prior to surgery, usually with phenoxybenzamine, starting at a dose of 20-60mg per day in divided doses. […] If tachycardia becomes a problem, then beta-blockade can be introduced, but only after adequate alpha-blockade has been achieved. […] However, even the use of alpha blockade at all prior to surgery is now controversial, with several centres now no longer insisting on it before minimally invasive adrenalectomy is undertaken. […] Excellent and highly-skilled anaesthetic care is required, with maximal haemodynamic monitoring and resuscitation available.

• #105 Phaeochromocytoma Treatment – Endocrinesurgery.net.au

  http://www.endocrinesurgery.net.au/phaeochromocytoma-treatment/

  The adrenalectomy can generally be achieved by laparoscopic (keyhole) surgery, except in the case of very large tumours or those suspected to be malignant, when open operation is safer. […] To avoid the release of any catecholamines into the systemic circulation, the priority of the surgeon is to isolate the adrenal vein with as little manipulation of the gland as possible, to prevent release of a massive dose of catecholamines into the bloodstream and precipitation of a hypertensive crisis. […] As a result of surgery 75% can expect a normalisation of blood pressure, with the remission of hypertensive paroxysms. […] In the case of malignant tumours the 5-year survival rate is 44%. […] Although surgery has a high success rate, all patients should attend annual follow up appointments to have urinary catecholamine levels checked so that any further tumours may be detected.

• #106 Pheochromocytoma – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pheochromocytoma/symptoms-causes/syc-20355367

  Surgery to remove a pheochromocytoma often returns blood pressure to a healthy range. […] If a pheochromocytoma isn’t treated, serious or life-threatening damage to other body systems can happen. […] A pheochromocytoma can lead to other health problems. The high blood pressure linked with a pheochromocytoma can damage organs, especially tissues of the heart and blood vessel system, brain and kidneys. This damage can cause dangerous conditions, including: Heart disease, Stroke, Kidney failure, Vision loss. […] Rarely, a pheochromocytoma spreads to other parts of the body. This tumor is then considered cancerous, also called malignant, and is referred to as metastatic pheochromocytoma.

• #107 Pheochromocytoma > Fact Sheets > Yale Medicine

  https://www.yalemedicine.org/conditions/pheochromocytoma

  Treatment includes surgery, medications, chemotherapy, radiation therapy […] Surgical removal of the tumor is the main treatment for pheochromocytoma. Usually, several weeks before the surgery, patients begin taking medications to lower and stabilize their blood pressure and heart rate. […] Surgery for pheochromocytoma involves a total adrenalectomy, a procedure in which one or both adrenal glands are completely removed. […] Cancerous pheochromocytomas are rare and usually require multimodal therapy with surgery, radiation therapy, and chemotherapy. […] With treatment, the outlook is better. In many cases, surgical removal of the tumor can cure people of the disease. […] At Yale, we provide a robust multidisciplinary approach to the treatment of pheochromocytomas, with involvement of experienced clinicians in the fields of medical endocrinology, endocrine surgery, radiology, and anesthesiology.

Zobacz więcej