Materiały źródłowe

• #1 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).

• #1 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.

• #1 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.

• #1 Pheochromocytoma Medication: Alpha Blockers, Antihypertensives, BPH, Alpha Blocker, Vasodilators, Beta Blockers, Nonselective, Beta Blockers, Beta1 Selective, Antihypertensives, Other, Radiopharmaceuticals

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

  Labetalol (Trandate, Normodyne) is a noncardioselective beta-adrenergic blocker and selective alpha-adrenergic blocker that has been shown to be effective in controlling hypertension associated with pheochromocytoma. However, it has also been associated with paradoxic episodes of hypertension thought to be secondary to incomplete alpha blockade. Thus, its use in the preoperative treatment of patients with pheochromocytoma is controversial. […] During surgery, intravenous phentolamine, a rapid-acting alpha-adrenergic antagonist, is used to control blood pressure. Rapid-acting intravenous beta blockers, such as esmolol, are also used to normalize blood pressure. […] Selective alpha1 blocking agents, such as prazosin (Minipress), terazosin (Hytrin), and doxazosin (Cardura), have more favorable adverse effect profiles and are used when long-term therapy is required (metastatic pheochromocytoma). These medications are not used to prepare patients for surgery, because of their incomplete alpha blockade.

• #1 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. […] Initiate a beta blocker only after adequate alpha blockade (usually, 2 days). […] 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.

• #1

  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). This latter should never be started first because blockade of vasodilatory peripheral -adrenergic receptors with unopposed -adrenergic receptor stimulation can lead to a further elevation of BP. […] 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.

• #1 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. Sometimes cortical-sparing surgery is an alternative. […] 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.

• #1 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.

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

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

  Phenoxybenzamine is effective, and metyrosine, which is a drug that blocks catecholamine synthesis, can be added if needed. […] 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.

• #1 Pheochromocytoma: What is a Pheochromocytoma? – Endocrine Surgery | UCLA Health

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

  Pheochromocytomas are treated with surgery to remove the adrenal gland that contains the tumor. […] A medication called an alpha blocker is given for about 2 weeks prior to surgery to stabilize the patients vital signs and get their blood pressure and heart rate under control. This step limits serious complications during surgery, as manipulation of the tumor can cause release of hormones that can raise the blood pressure and heart rate. Having an experienced surgeon and anesthesiologist team is critical for a safe and successful surgery. […] After surgery, patients vital signs are closely monitored and hormone levels are tested every year to make sure the pheochromocytoma does not recur (come back). Most patients are cured with surgery as long as the pheochromocytoma is completely removed without any break of the tumor capsule during surgery.

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

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

  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%. […] Surgical mortality rates are less than 2-3% when the operation is performed by a surgeon and an anesthesiologist who are experienced. […] 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. […] 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.

• #1 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.

• #1 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. […] Treatment options for metastatic pheochromocytoma include surgery and palliative therapy. […] If all identifiable disease is resectable, including a limited number of distant metastases, surgery can provide occasional long-term remission. […] 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. […] Options for patients with local-regional or metastatic disease who are not considered candidates for surgical resection include chemotherapy, targeted therapies, high-dose iodine I 131-metaiodobenzylguanidine radiation therapy, ablation therapies, and radiation therapy.

• #1 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. […] If one healthy adrenal gland remains, it can carry out the functions usually done by two glands. Blood pressure usually returns to a healthy range after surgery. You’ll need regular checkups and blood tests with your healthcare professional for the rest of your life. These appointments help track your health, find other health concerns and check to see if the tumor has come back. […] 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:

• #1 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

  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). […] 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.

• #1 Pheochromocytoma Symptoms, Treatment, Diagnosis & More

  https://pheopara.org/education/pheochromocytoma

  Ablation is a treatment to remove or destroy a body part or tissue or its function. […] Embolization therapy is a treatment to block the artery leading to the adrenal gland. […] TKI is a targeted therapy treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. […] Treatment of metastatic pheo can include surgery, MIBG therapy, PRRT therapy (Lutathera), chemotherapy, and others.

• #1 Pheochromocytoma – Diagnosis and treatment – Mayo Clinic

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

  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.

• #1 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

  On a case-by-case decision, prolonged treatment with CVD chemotherapy with 20 cycles of CVD is suggested, especially in the case of patients with SDHB mutations. […] Alternatively, temozolomide monotherapy (150 mg/m2 on days 1–5, at 28 day intervals) or a metronomic scheme with temozolomide (75 mg/m2 per day for three weeks followed by one week off treatment) may be considered, either as initial therapy or following stabilization with CVD. […] 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.

• #1 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.

• #1 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.

• #1 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.

• #1 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.

• #1 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

  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.

• #1 Pheochromocytoma | Stanford Health Care

  https://stanfordhealthcare.org/medical-conditions/blood-heart-circulation/pheochromocytoma.html

  Research has found that removing pheochromocytoma tumors, at any stage, can increase life expectancy. Stanfords surgeons have exceptional expertise in specialized surgery to remove all types of adrenal tumors. In nearly all cases, your surgeon will remove the pheochromocytoma and the adrenal gland. […] Radiation therapy targets tumors with strong radioactive beams to destroy cancer cells. Stanford participated in developing an advanced treatment, peptide receptor radionuclide therapy (PRRT). PRRT takes advantage of a receptor on the tumors surface to concentrate cancer-killing radiation on the tumor and destroy tumor cells. This targeted treatment helps reduce the side effects of treatment. […] Medical cancer therapy delivers treatments by mouth, through a vein by IV (intravenous) infusion, or by injection. These systemic (whole body) treatments attack pheochromocytoma cells to control the tumors growth. […] Genetic counseling plays a crucial role in managing pheochromocytomas. These tumors are genetic in 30% to 40% of cases, which means there is a significant chance that your relatives may share the condition, or you may pass a higher risk to your children.

• #1 Pheochromocytoma | Stanford Health Care

  https://stanfordhealthcare.org/medical-conditions/blood-heart-circulation/pheochromocytoma.html

  Our doctors are studying new mutations (changes) that cause pheochromocytoma and paraganglioma syndromes. Their work has helped establish that providing precision treatment care based on your genetics and advanced imaging leads to better outcomes for you. […] Genetic evaluation equips your doctors to understand the metabolic basis of a particular disease. The diseases genetic makeup influences how a tumor might behave. It also offers clues about what other tumors, in what other areas of the body, to watch for. […] People with the mutation that causes pheochromocytoma have a higher likelihood of developing recurrent pheochromocytoma tumors or related tumors, such as gastrointestinal stromal tumors (GIST). Our doctors tune your care specifically to look for those other tumor types. Our goal is to catch other tumors as early as possible, so that you receive appropriate care.

• #1

  https://more.navigatingcare.com/library/pheochromocytoma/learn/treatment-option-overview

  Systemic chemotherapy is used to treat pheochromocytomas and paragangliomas. […] Ablation therapies used to help kill cancer cells include: […] Embolization therapy is a treatment to block the artery leading to the adrenal gland. […] Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells. […] New types of treatment are being tested in clinical trials. […] Treatment for pheochromocytoma and paraganglioma may cause side effects. […] Patients may want to think about taking part in a clinical trial. […] Patients can enter clinical trials before, during, or after starting their cancer treatment. […] Follow-up tests will be needed. […] For patients with pheochromocytoma or paraganglioma that causes symptoms, catecholamine levels in the blood and urine will be checked on a regular basis. […] For patients with inherited pheochromocytoma, catecholamine levels in the blood and urine will be checked on a regular basis. […] Patients with pheochromocytoma or paraganglioma need lifelong follow-up.

• #1 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.

• #1 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.

• #2 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.

• #2 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. […] Initiate a beta blocker only after adequate alpha blockade (usually, 2 days). […] 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.

• #2 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.

• #2 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.

• #2 Pheochromocytoma Medication: Alpha Blockers, Antihypertensives, BPH, Alpha Blocker, Vasodilators, Beta Blockers, Nonselective, Beta Blockers, Beta1 Selective, Antihypertensives, Other, Radiopharmaceuticals

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

  Iobenguane I 131 was approved by the US Food and Drug Administration (FDA) in July 2018 for iobenguane scanpositive, unresectable, locally advanced or metastatic pheochromocytoma or paraganglioma in patients aged 12 years or older in whom systemic anticancer therapy is needed. The drugs efficacy was demonstrated in a single-arm, open-label, phase-2 clinical trial performed under a special protocol assessment with the FDA. Seventeen (25%) of the studys 68 evaluable patients were able to reduce all antihypertensive medication by 50% or more for at least 6 months; 15 patients (22%) obtained an overall tumor response. […] Metyrosine inhibits tyrosine hydroxylase, the rate-limiting step in catecholamine synthesis. In patients with pheochromocytoma, administration of metyrosine reduces catecholamine biosynthesis by 35-80%, as measured by urinary catecholamine levels. Metyrosine is indicated in patients with pheochomocytoma who are awaiting surgery, for long-term management of patients with malignant pheochromocytoma, or in cases of pheochromocytoma in which surgery is contraindicated. It can be useful in patients whose condition is refractory to phenoxybenzamine therapy, or it can be administered as an adjunct to that therapy. […] Iobenguane I 131 is the first drug approved by the FDA for pheochromocytomas or paragangliomas that cannot be removed by surgery.

• #2 Pheochromocytoma – OpenAnesthesia

  https://www.openanesthesia.org/keywords/pheochromocytoma/

  Preparation for pheochromocytoma surgery takes 10-14 days to control hypertension, tachycardia, and restoration of intravascular volume. […] Alpha-blockers are the mainstay of preoperative treatment. They counter excess systemic catecholamine and improve intraoperative hemodynamic stability. Current guidelines recommend this practice despite no proven benefits in randomized trials. Nonselective alpha antagonists and selective alpha-2 antagonists have been used in this capacity. […] A high sodium diet and fluid intake are used to facilitate volume expansion and limit orthostatic symptoms. […] Selective and nonselective beta blockade is frequently used in patients with persistent arrhythmias or tachycardia, which is common in tumors that secrete large amounts of epinephrine and can be exacerbated with alpha blockade.

• #2 Pheochromocytoma | Columbia Surgery

  https://columbiasurgery.org/conditions-and-treatments/pheochromocytoma

  The best treatment for pheochromocytoma is to remove it surgically. One of the few exceptions to this rule are patients who have other medical problems that are so dangerous that they would not survive an operation. In general, medical therapy is only effective temporarily and patients can „breakthrough” the medications. […] Before an operation to remove a pheochromocytoma, the patient must be properly prepared with certain medications to block the effects of adrenaline that will be released during the operation. This preparation must be done by a doctor (ex. endocrinologist, endocrine surgeon, or hypertension specialist) with experience managing patients with pheochromocytoma. […] In order to reverse and block this vasoconstriction, a medication called an alpha-blocker will be given for at least 10 to 14 days before the operation. The alpha-blocker causes the blood vessels to relax and prevents them from clamping down again when more adrenaline is released.

• #2 Pheochromocytoma Symptoms, Treatment, Diagnosis & More

  https://pheopara.org/education/pheochromocytoma

  Pheochromocytoma, if detected early, can be successfully treated and managed in the vast majority of cases. The treatment of choice is to surgically remove the tumor(s), but there are other treatment options. Surgical treatment usually returns blood pressure to normal and alleviates symptoms. […] Chemotherapy, radiation or radiopharmaceutical therapy are also treatment options in some cases. Alternatively, an experienced doctor may suggest only regular monitoring of the tumor(s) if they are not secreting catecholamines, there are no symptoms and the tumor(s) are stable (not growing). […] Surgery to remove a pheochromocytoma is the preferred treatment option. It may be done laproscopically or robotically, which is less invasive or by making a larger incision, if necessary. […] Before undergoing surgery, the patient must be adequately blocked with medication. Going under anesthesia without being blocked is very dangerous.

• #2 Surgical approach to patients with pheochromocytoma – Patel – Gland Surgery

  https://gs.amegroups.org/article/view/34955/html

  Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors that secrete excess catecholamines leading to secondary hypertension and cardiovascular morbidity. Once biochemical testing with either 24-hour urinary fractioned metanephrines or plasma free metanephrines confirms the diagnosis, patients are optimized with adequate hydration to maintain their intravascular volume and the appropriate antihypertensive medications are initiated for optimal blood pressure control. […] The surgical approach and extent of adrenalectomy is personalized based on multiple factors including germline genetic test results, the size of the tumor, body mass index, surgeon experience, and the likelihood of malignancy. […] Surgical approaches include transabdominal or retroperitoneal minimally invasive approaches, and transabdominal open approaches.

• #2 Surgical approach to patients with pheochromocytoma – Patel – Gland Surgery

  https://gs.amegroups.org/article/view/34955/html

  Minimally invasive techniques can be approached either laparoscopically or robotically. […] The surgical approaches for patients with pheochromocytoma include open and minimally invasive techniques. […] Minimally invasive (laparoscopic or robotic) adrenalectomy is the preferred approach for patients with pheochromocytomas. […] The open approach is preferred when there is a clinical suspicion or concern for an invasive malignant pheochromocytoma. […] A minimally invasive or open surgical approach can be utilized in cortical sparing (partial) adrenalectomies for patients with pheochromocytomas related to syndromes. […] Postoperatively, most patients who have had an uneventful intraoperative course with minimal hemodynamic instability are admitted to the floors. […] Outcomes after surgery show improved blood pressure control in most patients and normalization of blood pressure in about a third of patients. Long-term follow-up is required for all patients to assess for recurrence.

• #2 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.

• #2 Pheochromocytoma – OpenAnesthesia

  https://www.openanesthesia.org/keywords/pheochromocytoma/

  Alternative medication choices include calcium channel blockers as well as inhibitors of catecholamine synthesis like metyrosine. […] The anesthetic goals can be summarized as providing stable hemodynamics during excessive catecholamine surges and depletion during critical periods of surgery. […] Management of hypertension: Short-acting vasodilators and beta-blockers should be readily available. Hypertension is treated by increasing the depth of anesthesia and administering direct-acting arteriolar vasodilators such as sodium nitroprusside, nicardipine, or cilnidipine. […] Management of Hypotension: Short-acting vasopressors are the mainstay of treatment. Phenylephrine or norepinephrine, in combination with vasopressin or epinephrine, is commonly used.

• #2 Pheochromocytoma and Paraganglioma Treatment (PDQ®): Treatment – Patient Information [NCI]

  https://www.cham.org/HealthwiseArticle.aspx?id=ncicdr0000270724

  Treatment of metastatic pheochromocytoma or paraganglioma may include the following: Surgery to completely remove the cancer, including tumors that have spread to distant parts of the body. […] Treatment of recurrent pheochromocytoma or paraganglioma may include the following: Surgery to completely remove the cancer. […] Treatment of pheochromocytoma during pregnancy may include the following: Surgery to completely remove the cancer during the second trimester (the fourth through the sixth month of pregnancy).

• #2 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.

• #2 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.

• #2 Pheochromocytoma: Symptoms, Treatment, and More

  https://www.verywellhealth.com/pheochromocytoma-overview-and-more-5196422

  Laparoscopic surgery is the preferred method for removal. This minimally invasive procedure is done using a thin, camera-equipped surgical tool that is inserted through a small incision in the abdomen. […] If tumors are present in both adrenal glands, management must be individualized. Many physicians try to save part of an adrenal gland as it is very uncommon for these tumors to be cancerous. […] If both adrenal glands are removed, lifelong therapy is required to replace the hormones lost. Even with replacement, people may still develop acute adrenal crisis (Addisonian crisis), a condition in which the body doesn’t have enough adrenal hormones to regulate body functions. […] A radiopharmaceutical is often recommended first for people with metastatic tumors. In this therapy, a radioactive substance that accumulates in pheochromocytoma tumor cells is injected into a vein. It then travels to the tumor, and when taken up by the tumor cells, it gives off radiation that causes the cells to die.

• #2 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. […] Treatment options for metastatic pheochromocytoma include surgery and palliative therapy. […] If all identifiable disease is resectable, including a limited number of distant metastases, surgery can provide occasional long-term remission. […] 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. […] Options for patients with local-regional or metastatic disease who are not considered candidates for surgical resection include chemotherapy, targeted therapies, high-dose iodine I 131-metaiodobenzylguanidine radiation therapy, ablation therapies, and radiation therapy.

• #2 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. And the clinical trial she joined paved the way for the Food and Drug Administrations (FDA) approval of the very first targeted therapy drug for the treatment of pheochromocytoma. […] 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. The surgery, combined with the drug Susan took, have kept her in remission since 2016.

• #2 Pheochromocytoma Symptoms, Treatment, Diagnosis & More

  https://pheopara.org/education/pheochromocytoma

  Drugs may be given before, during, and after surgery to keep blood pressure and heart rate normal. […] Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. […] Pheochromocytoma can be treated with MIBG, which is a radiopharmaceutical that is injected into the patient’s bloodstream. […] PRRT (Peptide Receptor Radionuclide Therapy) is a radiopharmaceutical that when injected into the patient’s bloodstream travels to and binds to the tumor delivering a targeted high dose of radiation directly to the cancer cells. […] Somatostatin is a naturally occurring hormone that acts by binding to somatostatin receptor (SSTR), a receptor that is overexpressed in pheo para. […] 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.

• #2 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

  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. […] CVD chemotherapy (cyclophosphamide 750 mg/m2, vincristine 1.4 mg/m2, and dacarbazine 600 mg/m2 on day 1 and dacarbazine 600 mg/m2 on day 2, at 21 day intervals) is recommended for rapidly progressive (<6 months) metastatic PCC/PGL and especially effective in SDHB-related disease.

• #2 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

  On a case-by-case decision, prolonged treatment with CVD chemotherapy with 20 cycles of CVD is suggested, especially in the case of patients with SDHB mutations. […] Alternatively, temozolomide monotherapy (150 mg/m2 on days 1–5, at 28 day intervals) or a metronomic scheme with temozolomide (75 mg/m2 per day for three weeks followed by one week off treatment) may be considered, either as initial therapy or following stabilization with CVD. […] 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.

• #2 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.

• #2 Medical Treatment of Malignant Pheochromocytoma | SpringerLink

  https://link.springer.com/chapter/10.1007/978-3-031-62301-1_17

  Systemic treatments are indicated for progressing or symptomatic metastatic pheochromocytoma or paraganglioma (mPPGL). […] Chemotherapy, targeted therapy and immunotherapy are the treatment options available so far. […] CVD chemotherapy is mainly recommended for tumors characterized by rapid progression. […] TKIs may be used for patients with rapid disease progression, and the toxicity of TKIs is expected to be lower than that of CVD chemotherapy. […] Clinical trials are extremely important to evaluate novel therapies targeting pathways involved in the development of mPPGL. […] A phase II clinical trial with pembrolizumab for patients with mPPGL revealed modest responses to pembrolizumab, with an overall response rate of 9%.

• #2 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.

• #2 Pheochromocytoma: What is a Pheochromocytoma? – Endocrine Surgery | UCLA Health

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

  In the rare cases of malignant pheochromocytoma, treatment options include surgery, radiation, chemotherapy, and Peptide Receptor Radionuclide Therapy (PRRT). […] Patients with hypertension prior to surgery often have rapid and significant improvement immediately after surgery. […] The other adrenal gland produces all of the hormones that the body needs, and hormone replacement is not needed after adrenalectomy for pheochromocytoma. […] Those cases require multi-disciplinary treatment at a specialized center for medical management of blood pressure and symptoms, possible reoperation, and other treatment options previously mentioned.

• #2 Pheochromocytoma and Paraganglioma Treatment (PDQ®): Treatment – Health Professional Information [NCI] – Metro Family Physicians Medical Group

  https://metrofamilyphysicians.com/patient-information/health-library?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.

• #2 Systemic Radiopharmaceutical Therapy of Pheochromocytoma and Paraganglioma | Journal of Nuclear Medicine

  https://jnm.snmjournals.org/content/62/9/1192

  Whereas benign pheochromocytomas and paragangliomas are often successfully cured by surgical resection, treatment of metastatic disease can be challenging in terms of both disease control and symptom control. Fortunately, several options are available, including chemotherapy, radiation therapy, and surgical debulking. […] The goals of treatment are palliative, including prevention of progression and improvement of symptoms related to tumor burden or catecholamine release. For systemic treatment, the most common chemotherapy regimen is cisplatin, vinblastine, and dacarbazine, which may be preferred for rapidly progressing disease. […] Currently, systemic radiopharmaceutical therapies (RPT) for pheochromocytoma and paraganglioma include 131I-metaiodobenzylguanidine (iobenguane, 131I-MIBG) of low specific activity (LSA) or high specific activity (HSA) and peptide receptor radionuclide therapy (PRRT) with 90Y- or 177Lu-DOTA-somatostatin analogs.

• #2 Pheochromocytoma | Stanford Health Care

  https://stanfordhealthcare.org/medical-conditions/blood-heart-circulation/pheochromocytoma.html

  Our doctors are studying new mutations (changes) that cause pheochromocytoma and paraganglioma syndromes. Their work has helped establish that providing precision treatment care based on your genetics and advanced imaging leads to better outcomes for you. […] Genetic evaluation equips your doctors to understand the metabolic basis of a particular disease. The diseases genetic makeup influences how a tumor might behave. It also offers clues about what other tumors, in what other areas of the body, to watch for. […] People with the mutation that causes pheochromocytoma have a higher likelihood of developing recurrent pheochromocytoma tumors or related tumors, such as gastrointestinal stromal tumors (GIST). Our doctors tune your care specifically to look for those other tumor types. Our goal is to catch other tumors as early as possible, so that you receive appropriate care.

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

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

  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%. […] Surgical mortality rates are less than 2-3% when the operation is performed by a surgeon and an anesthesiologist who are experienced. […] 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. […] 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.

• #2 Pheochromocytoma – National Adrenal Diseases Foundation

  https://www.nadf.us/pheochromocytoma.html

  The first step any patient should take is establishing care at a specialized center with physicians who have treated at least a few hundred patients with these syndromes. […] After comprehensive evaluation and confirming the diagnosis, this is followed by advanced imaging studies to localize and stage the disease to establish a treatment plan and the prognosis. Medical therapy by expert endocrinologists is the initial step in management, and this starts with special medications that control blood pressure and heart rate and block the active hormones from causing damage on the body. […] When lesions are confined to the adrenal gland, or resectable extra-adrenal lesions are found, surgery follows. This occurs after very careful monitoring and medication optimization by the endocrinologist.

• #3 Management of Patients with Treatment of Pheochromocytoma: A Critical Appraisal

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9405588/

  Preoperative medical preparation with an alpha blockade has been implemented early 1960s to prevent intraoperative hemodynamic instability and consequently decrease morbi-mortality in patients at a high risk of complications. […] A preoperative blockade using antihypertensive medications to prevent intraoperative hemodynamic instability and cardiocirculatory events is recommended by current clinical guidelines. […] Preoperative medical preparation should be questioned because it does not significantly improve intraoperative events or the risk of postoperative complications in current clinical practice. […] Current clinical recommendations should be revised and upgraded to current clinical practices. […] Surgical resection is the only curative treatment for patients with pheochromocytoma.

• #3 Phaeochromocytoma (Investigations and Treatment)

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

  Phaeochromocytoma treatment and management […] Surgical resection of the tumour is the treatment of choice and usually results in cure of the hypertension. Pre-operative treatment with alpha-blockers and beta-blockers is required to control blood pressure and prevent intraoperative hypertensive crises. […] Alpha blockade with phenoxybenzamine is started at least 7 to 10 days before operation to allow for expansion of blood volume. […] Only once this is achieved is beta blockade considered. If beta blockade is started too soon, unopposed alpha stimulation can precipitate a hypertensive crisis. […] Calcium-channel blockers are also useful. […] Complete resection of the tumour is usually possible and surgical mortality rates are less than 2% or 3% with an experienced anaesthetist and surgeon.

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

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

  Definitive treatment for localized and regional pheochromocytoma, including localized disease recurrence, consists of alpha- and beta-adrenergic blockade followed by surgery. […] For patients with unresectable or metastatic pheochromocytoma, treatment may include a combination of catecholamine blockade, surgery, chemotherapy, radiofrequency ablation, cryoablation, and radiation therapy. […] Surgery is the mainstay of treatment for most patients; however, preoperative medical preparation is critical. Alpha-adrenergic blockade should be initiated at the time of diagnosis and maximized preoperatively to prevent potentially life-threatening cardiovascular complications, which can occur as a result of excess catecholamine secretion during surgery. […] Surgical resection (i.e., adrenalectomy) is the definitive treatment for patients with localized pheochromocytoma.

• #3 Surgical approach to patients with pheochromocytoma – Patel – Gland Surgery

  https://gs.amegroups.org/article/view/34955/html

  Minimally invasive techniques can be approached either laparoscopically or robotically. […] The surgical approaches for patients with pheochromocytoma include open and minimally invasive techniques. […] Minimally invasive (laparoscopic or robotic) adrenalectomy is the preferred approach for patients with pheochromocytomas. […] The open approach is preferred when there is a clinical suspicion or concern for an invasive malignant pheochromocytoma. […] A minimally invasive or open surgical approach can be utilized in cortical sparing (partial) adrenalectomies for patients with pheochromocytomas related to syndromes. […] Postoperatively, most patients who have had an uneventful intraoperative course with minimal hemodynamic instability are admitted to the floors. […] Outcomes after surgery show improved blood pressure control in most patients and normalization of blood pressure in about a third of patients. Long-term follow-up is required for all patients to assess for recurrence.

• #3 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.

• #3 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%. […] 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.

• #3 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.

• #3 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

  On a case-by-case decision, prolonged treatment with CVD chemotherapy with 20 cycles of CVD is suggested, especially in the case of patients with SDHB mutations. […] Alternatively, temozolomide monotherapy (150 mg/m2 on days 1–5, at 28 day intervals) or a metronomic scheme with temozolomide (75 mg/m2 per day for three weeks followed by one week off treatment) may be considered, either as initial therapy or following stabilization with CVD. […] 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.

• #3 Pheochromocytoma and Paraganglioma Treatment (PDQ®): Treatment – Patient Information [NCI]

  https://www.cham.org/HealthwiseArticle.aspx?id=ncicdr0000270724

  Genetic counseling is part of the treatment plan for patients with pheochromocytoma or paraganglioma. […] All patients who are diagnosed with pheochromocytoma or paraganglioma should have genetic counseling to find out their risk for having an inherited syndrome and other related cancers. […] Drug therapy begins when pheochromocytoma or paraganglioma is diagnosed. This may include: Drugs that keep the blood pressure normal. For example, one type of drug called alpha-blockers stops noradrenaline from making small blood vessels more narrow. Keeping the blood vessels open and relaxed improves blood flow and lowers blood pressure. […] Surgery to remove pheochromocytoma is usually an adrenalectomy (removal of one or both adrenal glands). During this surgery, the tissues and lymph nodes inside the abdomen will be checked and if the tumor has spread, these tissues may also be removed.

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