Materiały źródłowe

• #1 Perioperative Management of Pheochromocytoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK589634/

  Pheochromocytoma (PCC) is a rare catecholamine-secreting tumor of adrenal origin. […] This activity entails pre-operative optimization of patients for resection of PCC. […] Post-operative management of patients has been discussed. […] Pre-op preparation and optimization are essential to decrease peri-operative morbidity and mortality in pheochromocytoma. […] It is vital to optimize intravascular volume to decrease peri-operative hemodynamic fluctuation. […] A detailed preoperative assessment by an experienced anesthesiologist is required to evaluate cardiovascular status, adequacy of alpha and beta blockade, volume resuscitation, and any other comorbidities. […] Minimally-invasive adrenalectomy via retroperitoneal or transperitoneal approach is the gold standard for pheochromocytoma resection. […] An interprofessional team consisting of an experienced surgeon, anesthesiologist, and endocrinologist is required. […] Pre-operative optimization with alpha-blocking agents and close hemodynamic monitoring in the peri-operative period is essential for the best outcome.

• #1 PERIOPERATIVE CARE OF PHAEOCHROMOCYTOMA – Anaesthesia News

  https://anaesthesianews.wordpress.com/2023/03/17/perioperative-care-of-phaeochromocytoma/

  1. What is a pheochromocytoma? A pheochromocytoma is a vascular tumor of chromaffin tissue (most commonly the adrenal medulla) that produces and secretes norepinephrine and epinephrine. The diagnosis and management of pheochromocytoma are based on the effects of abnormally high circulating levels of these endogenous adrenergic agonists. (Morgan) […] Phaeochromocytomas may present with a classic symptom triad of headache, palpitations, and sweating. Hypertension is present in around 90% of cases, although it is paroxysmal in 35-50% of these. Other non-specific presentations include anxiety, lethargy, nausea, weight loss, hyperglycaemia, and tremor. […] The objectives of preoperative care include: arterial pressure control, reversal of chronic circulating volume depletion, heart rate and arrhythmia control, assessment and optimization of myocardial function, reversal of glucose and electrolyte disturbances.

• #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. 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.

• #1 Pheochromocytoma Symptoms, Treatment, Diagnosis & More

  https://pheopara.org/education/pheochromocytoma

  It is extremely important that practitioners involved in the care of the patient have experience with pheo para surgery and that patients be blocked for the best possible outcome. Alpha and beta-blockers are prescribed to normalize blood pressure and heart rate, which protect the patient from the effects of high levels of hormones (catecholamines) released during surgery. First, an alpha blocking medication is prescribed for at least 2 weeks before the surgery. Phenoxybenzamine(Dibenzyline) doxazosin, prazosin and terazosin are used. Once blockage has been obtained, a beta-blocker is prescribed, sometimes in combination with calcium channel blockers. A high salt diet may be recommended, as well.

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

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

  In the preoperative period, the goal is to optimize the BP and normalize the intravascular volume. […] Patients are encouraged to be well hydrated with a high sodium diet prior to surgery. […] Selective or non-selective alpha blockade is always initiated first in preoperative preparation of patients with PPGLs. […] The communication between the anesthesiologist and the surgeon is paramount to a safe outcome. […] The surgical approaches for patients with pheochromocytoma include open and minimally invasive techniques. Minimally invasive techniques can be approached either laparoscopically or robotically. […] 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. Patients who have ongoing hemodynamic instability may require a monitored bed with telemetry or the intensive care unit. […] The extent of adrenalectomy can be dictated by genetic and familial predisposition.

• #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 PERIOPERATIVE CARE OF PHAEOCHROMOCYTOMA – Anaesthesia News

  https://anaesthesianews.wordpress.com/2023/03/17/perioperative-care-of-phaeochromocytoma/

  A nonselective -blocker should never be administered before -blockade, because blockade of vasodilatory 2-receptors results in unopposed -agonism, leading to vasoconstriction and hypertensive crises. […] If -receptors are blocked first, norepinephrine and epinephrine will produce unopposed stimulation. […] Across most centers, the laparoscopic approach is preferred for most pheochromocytoma resections. […] Surgical resection of pheochromocytoma can be divided into two phases based on ligation of the blood supply to the tumor. […] The chosen anaesthetic technique should: Avoid drug-induced catecholamine release, Avoid catecholamine release induced by anaesthetic or surgical manoeuvres, Minimize haemodynamic responses to tumour handling, Treat episodes of hypotension, particularly after tumour devascularization.

• #1 Post-Surgery Care for Pheochromocytoma Patients

  https://www.rupahealth.com/post/post-surgery-care-for-pheochromocytoma-patients

  Pheochromocytoma is a rare adrenal gland tumor that triggers an overproduction of hormones, causing issues like high blood pressure, headaches, and tachycardia (rapid heart rate). To treat it, surgeons perform an adrenalectomy, removing the affected adrenal gland. However, the journey doesn’t end there—post-surgery care is just as important as the surgery itself. […] Post-operative management of blood pressure fluctuations, monitoring hormone levels, and preventing complications are all essential for recovery and good outcomes. This article provides the essential steps of post-operative care that help to ensure a smooth and safe recovery after adrenalectomy for pheochromocytoma. […] After surgery for pheochromocytoma, patients are typically monitored in an intensive care unit (ICU) or other high-dependency unit for one to two days. This permits continuous observation of vital signs, particularly blood pressure, which can fluctuate due to hormonal changes after the tumor removal.

• #1 Post-Surgery Care for Pheochromocytoma Patients

  https://www.rupahealth.com/post/post-surgery-care-for-pheochromocytoma-patients

  Medical teams will monitor for adrenal insufficiency, as removing one or both adrenal glands can reduce hormone production. […] Medications to control blood pressure and manage hormonal levels will be adjusted during this period. […] Once discharged, patients will be given specific instructions for home care. […] Wound care is essential to prevent infection, so keeping the surgical site clean and dry and changing bandages as directed is important. […] In the immediate postoperative period, home health nursing services may be needed for patients living alone or with limited self-care ability. […] Patients must adhere to their medication schedule, especially if prescribed hormone replacements. […] Physical activity will likely be restricted for several weeks, with limitations on heavy lifting or strenuous exercise. Based on the doctor’s advice, a gradual reintroduction of normal activities is recommended.

• #1 Pheochromocytoma – Diagnosis and treatment – Mayo Clinic

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

  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. […] If both adrenal glands are removed, you’ll need to take steroid medicines for the rest of your life. These medicines replace certain hormones that the adrenal glands make.

• #1 Post-Surgery Care for Pheochromocytoma Patients

  https://www.rupahealth.com/post/post-surgery-care-for-pheochromocytoma-patients

  Some patients may experience post-operative hypertension, where their blood pressure remains elevated due to residual hormonal effects. This involves monitoring blood pressure and continuing medications like beta-blockers or calcium channel blockers. Over time, as the body adjusts, blood pressure often stabilizes. […] After surgery, managing diet becomes important for controlling blood pressure and ensuring a smooth recovery. […] A low-sodium diet is often recommended to help keep blood pressure in check, as excess salt can lead to higher blood pressure. […] Patients should aim to eat fresh, whole foods like fruits, vegetables, lean proteins, and whole grains while avoiding processed and packaged foods, which tend to be high in sodium. […] Staying hydrated is also key for recovery. Drinking plenty of water supports blood circulation, helps flush out toxins, and maintains proper bodily functions.

• #1 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. […] No distinction is found in hypertensive episodes during surgery for pheochromocytoma associated with MEN 2 and non-MENassociated pheochromocytoma. Therefore, pretreatment using alpha and beta-adrenergic blockers remains a standard of care in both groups of patients.

• #1 Management of phaeochromocytoma and paraganglioma in patients with germline SDHB pathogenic variants: an international expert Consensus statement | Nature Reviews Endocrinology

  https://www.nature.com/articles/s41574-023-00926-0

  R10. We recommend that a personalized and interdisciplinary cardiovascular management plan should be in place to prevent complications before, during and after surgical resection (Grade 1, low). […] R16. We recommend that for biochemically positive SDHB PPGL, a patient’s plasma or urinary levels of metanephrines (and, if available, also plasma levels of 3-methoxytyramine) should be measured by 8 weeks postoperatively, and thereafter at least once a year (Grade 1, very low). […] R17. We recommend that for all patients after surgery, re-evaluation with the preferred CT or MRI should be performed within 6 months. SSTR PET-CT should also be performed within 6 months after surgery, especially if not performed preoperatively (Grade 1, low). If there is no evidence of disease within 6 months, including repeated negative biochemistry, we suggest performing MRI from skull base-to-pelvis at least every 12 years to detect new PPGLs, recurrences or metastases. If there is evidence of disease persistence either at the first postoperative scan or positive postoperative biochemistry, more frequent imaging (CT or MRI, with or without SSTR PET-CT) and possible therapeutic interventions might need to be considered. We suggest lifelong follow-up with increasing intervals after long-term tumour stability (Grade 2, low).

• #1 Pheochromocytoma and Paraganglioma Treatment – NCI

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

  Pheochromocytoma and paraganglioma are rare tumors that come from the same type of tissue. […] Pheochromocytoma is a rare tumor that forms in the adrenal medulla (the center of the adrenal gland). […] Signs and symptoms of pheochromocytoma and paraganglioma include high blood pressure and headache. […] Genetic counseling is part of the treatment plan for patients with pheochromocytoma or paraganglioma. […] Patients receive medication to treat the signs and symptoms of pheochromocytoma and paraganglioma. […] Drug therapy begins when pheochromocytoma or paraganglioma is diagnosed. […] Surgery to remove pheochromocytoma is usually an adrenalectomy (removal of one or both adrenal glands). […] Treatment of pheochromocytoma during pregnancy may include surgery. […] Pregnant women with pheochromocytoma need special care.

• #1 Phaeochromocytoma associated with neurofibromatosis type 1: a single tertiary care centre experience | UKINETS2024 | UKI NETS 22nd National Conference | Endocrine Abstracts

  https://www.endocrine-abstracts.org/ea/0105/ea0105p29

  Phaeochromocytoma associated with neurofibromatosis type 1: a single tertiary care centre experience […] There is no current UK screening program for patients with NF-1 for pheochromocytoma/paraganglioma. […] The majority were asymptomatic, but would have been identified with biochemical testing, raising the question about the potential need for a structured screening pathway to identify pheochromocytoma early among patients with NF-1 to enable timely intervention to prevent potential metastatic disease. […] Our case series highlights the need for larger studies (potential with a national registry) to identify risk factors for more aggressive disease and the need for greater screening for NF1 patients.

• #1 A phaeochromocytoma crisis | SFEBES2018 | Society for Endocrinology BES 2018 | Endocrine Abstracts

  https://www.endocrine-abstracts.org/ea/0059/ea0059cmw2.2

  A phaeochromocytoma is a catecholamine secreting tumour arising from the adrenal medulla and a paraganglioma refers to its extra adrenal counterpart, which can develop from sympathetic or parasympathetic tissue anywhere from the skull base to the pelvis. Presenting symptoms of these rare tumours are most commonly related to catecholamine excess, and include headache, palpitations, paroxysmal hypertension, anxiety, abdominal pain and excessive sweating. A phaeochromocytoma crisis is a life-threatening endocrine emergency defined as catecholamine-induced hemodynamic instability including; labile blood pressure, tachyarrhythmias and the risk of subsequent cardiovascular collapse. […] Early cautious treatment with alpha-blockade remains the first line treatment option for a catecholamine crisis. However, invasive pressure monitoring and cross-specialty input from cardiology, endocrinology, intensive care and surgical colleagues is often crucial in achieving the best patient outcomes. This management session will involve a cased-based and interactive discussion focusing on: (i) potential presenting symptoms of a phaeochromocytoma crisis; (ii) risk factors and precipitants of a crisis; (iii) complications of a crisis; and, (iv) suitable management strategies.

• #1 Phaeochromocytoma | Deranged Physiology

  https://derangedphysiology.com/main/required-reading/endocrine-intensive-care/Chapter-231/phaeochromocytoma

  The management of phaeochromocytoma is discussed in an excellent article from the Royal Adelaide hospital. The key is to block the α-adrenoceptors first. Phenoxybenzamine is an exciting exotic substance used exclusively for this purpose, as it is a non-competitive α-antagonist. It binds irreversibly to α-receptors, inactivating them, and no concentration of catecholamines will ever displace it. This is good, because competitive inhibition of α-receptors in this context will face strong opposition from the thousand-times-increased concentration of serum catecholamines. […] The Adelaide paper does mention that their practice has been to use atenolol before giving phenoxybenzamine, so as to ablate the reflexive tachycardia which will result from its use. This is probably because the population reported on in the paper were stable pre-operative outpatients. In the context of an acute crisis, one is obliged to control the vasoconstriction first, using something like phentolamine or sodium nitroprusside.

• #1 Pheochromocytoma | Conditions | UCSF Health

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

  Pheochromocytoma is a rare tumor that usually develops from one of the body’s two adrenal glands, located above each kidney in the back of the upper abdomen. […] Surgery is the primary treatment for this disorder. The tumors are usually benign, but in the rare cases of a cancerous tumor, we may use a combination of surgical removal, chemotherapy and radiation therapy. […] Treatment of pheochromocytoma involves surgery, chemotherapy and radiation therapy. […] Once a diagnosis of pheochromocytoma has been established and the tumor located, the tumor will be surgically removed. One or both adrenal glands may be removed in an operation called adrenalectomy. […] Chemotherapy is called a systemic treatment because the drug enters the bloodstream, travels through the body and can kill cancer cells throughout the body. […] This treatment uses high-energy X-rays to kill cancer cells and shrink tumors. […] This treatment uses high doses of I-MIBG, which cancerous pheochromocytomas may absorb, resulting in shrinkage or even disappearance of the tumors.

• #1 Med-Surg Nursing: Pheochromocytoma & Hyperaldosteronism

  https://leveluprn.com/blogs/medical-surgical-nursing/endocrine-system-13-pheochromocytoma-hyperaldosteronism?srsltid=AfmBOoqpLvt-idOIYAL7BZc4aB5n_ttBoIIwXDAEsYKLR3EbWV_fRjpm

  Heres something important to remember about the nursing care for a patient with pheochromocytoma: do not palpate the abdomen. Although abdomen palpation is a step in physical assessment, it should not be done on this patient because it can cause a sudden release of catecholamines, which can trigger severe hypertension which is very dangerous for this patient. […] One important thing for you to remember as a nurse is you never want to palpate the abdomen of a patient who is suspected of having pheochromocytoma because that can trigger the sudden release of catecholamines, which, in turn, can trigger severe hypertension in the patient, which can be very dangerous.

• #1 Post-Surgery Care for Pheochromocytoma Patients

  https://www.rupahealth.com/post/post-surgery-care-for-pheochromocytoma-patients

  Patients and caregivers should be educated about the signs of post-operative complications. For example: […] Signs of infection include fever, increased pain at the surgical site, redness, or unusual drainage. […] Uncontrolled blood pressure, extreme fatigue, or dizziness suggest adrenal insufficiency or other hormonal imbalances. Patients with these symptoms should contact their healthcare provider immediately. […] Recovery after surgery can be emotionally challenging. Patients may experience anxiety, mood swings, or fatigue as their body adjusts to hormonal changes. Emotional and psychological support, including counseling or support groups, can help manage the emotional aspects of recovery, promoting a positive healing process. […] To manage adrenal insufficiency, hormone replacement therapy may be prescribed, usually in the form of cortisol or hydrocortisone. These medications help restore normal hormone levels and ensure the body functions properly. Regular blood tests will be needed to monitor hormone levels and adjust medications as necessary.

• #1 Post-Surgery Care for Pheochromocytoma Patients

  https://www.rupahealth.com/post/post-surgery-care-for-pheochromocytoma-patients

  Patients should be educated about the signs of recurrence, such as unexplained high blood pressure, headaches, or excessive sweating. […] In addition to medical follow-ups, emotional support plays a key role in long-term recovery. Support groups can provide a space for patients to connect with others who have gone through similar experiences, which can help alleviate feelings of isolation. […] Counseling may also be beneficial, as it helps patients cope with the stress and anxiety that can come with managing a long-term condition. By staying informed and engaged in follow-up care, patients can improve their quality of life and increase the chances of early detection if issues arise.

• #1 Management of phaeochromocytoma and paraganglioma in patients with germline SDHB pathogenic variants: an international expert Consensus statement | Nature Reviews Endocrinology

  https://www.nature.com/articles/s41574-023-00926-0

  R1. We recommend that all major treatment and management decisions of patients with SDHB PPGLs should be carried out in an expert, interdisciplinary team conference to optimize care (Grade 1, very low). […] The management and treatment of patients with SDHB PPGLs pose a challenge to clinicians in many disciplines. These tumours are heterogeneous and can manifest in many organs with high rates of recurrence, local aggressive growth and metastasis over time. Most specialists across various disciplines have limited experience in this area of SDHB PPGLs and, therefore, international experts in this committee are convinced that an interdisciplinary discussion of management decisions in patients with SDHB PPGLs is an optimal approach. This approach also facilitates personalized tailoring of management in specific clinical situations, including plans for individualized surveillance and follow-up. However, this recommendation cannot be supported by evidence from well-designed clinical studies because such studies do not exist. Therefore, management decisions based on discussions in an interdisciplinary team with expertise in SDHB PPGLs are required to potentially achieve the most favourable outcomes for patients.

• #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.

• #2 Pheochromocytoma – Nurses Revision

  https://nursesrevisionuganda.com/pheochromocytoma/

  Pheochromocytoma is a tumor that produces excessive amounts of catecholamines, including adrenaline (epinephrine) and noradrenaline (norepinephrine). […] The primary goals of managing pheochromocytoma are; to control symptoms, stabilize blood pressure, ultimately remove the tumor. […] Pre-operative Care: Monitoring: Frequent monitoring of vital signs (blood pressure, heart rate). Medication Administration: Ensure accurate and timely administration of alpha and beta blockers. Patient Education: Provide clear instructions about medications and potential side effects. […] Post-operative Care: Monitoring: Continuous monitoring of vital signs. Fluid Management: Careful management of fluid balance to prevent hypotension or fluid overload. Pain Management: Administer pain medication as prescribed. Wound Care: Monitor incision site for signs of infection. Hormone Replacement: If bilateral adrenalectomy was performed, initiate hormone replacement therapy and educate the patient on how to take the medications.

• #2 Perioperative care of phaeochromocytoma | PPT

  https://www.slideshare.net/slideshow/perioperative-care-of-phaeochromocytoma-62265098/62265098

  This document discusses the perioperative care of patients with phaeochromocytoma. It covers the classification, aetiology, incidence, symptoms, diagnosis, localization and preoperative preparation of phaeochromocytoma. For preoperative preparation, the key goals are controlling blood pressure through adrenergic blockade, optimizing volume status and cardiac function, and correcting metabolic disturbances. Intraoperatively, the anaesthetic technique aims to prevent catecholamine release while managing surges in blood pressure from tumour handling and treating hypotension after tumour removal. […] The objectives of preoperative care include: Arterial pressure control, Reversal of chronic circulating volume depletion, Heart rate and arrhythmia control, Assessment and optimization of myocardial function, Reversal of glucose and electrolyte disturbances.

• #2 Phaeochromocytoma (Investigations and Treatment)

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

  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. […] 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 Phaeochromocytoma – Symptoms, diagnosis and treatment | BMJ Best Practice

  https://bestpractice.bmj.com/topics/en-gb/163

  Phaeochromocytoma is a rare neuroendocrine tumour of the adrenal glands. It can present with palpitations, diaphoresis, pallor, and paroxysmal hypertension. […] Treatment includes medical therapy for hypertension and surgical excision of tumour (open or laparoscopic adrenalectomy). For unresectable tumours, alternative approaches include chemotherapy or radiopharmaceuticals. […] Complications include hypertensive crisis, myocardial infarction, and hypotension.

• #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. 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.

• #2

  https://www.clinicsinsurgery.com/full-text/cis-v2-id1517.php

  The goals of preoperative management are to reduce the possibility of a catecholamine surge and its effect on target organs upon anesthesia induction and tumor manipulation while ensuring stability in postoperative care. […] Although no studies have specifically addressed the practice of fluid and electrolytes resuscitation in preparation for surgery, many highly recommend it. Chronically high levels of catecholamines from PPGLs contracts intravascular volume. Salt or saline loading should be instituted in preoperative patients to expand the intravascular volume. It also allows patients to increase their pre-operative alpha-blockade further before achieving orthostatic hypotension and it may reduce post-operation hypotension. […] Reducing anxiety prior to induction is crucial to prevent apprehension from causing catecholamine surges. Long-acting benzodiazepines the night prior and IV midazolam prior to transfer on the day of surgery can reduce the risk of hypertensive crises during induction.

• #2 Perioperative care of phaeochromocytoma | PPT

  https://www.slideshare.net/slideshow/perioperative-care-of-phaeochromocytoma-62265098/62265098

  Early phaeochromocytoma surgery saw mortality rates of up to 45% Severe intraoperative hypertension, Strokes, Arrhythmias, Myocardial ischaemia, Left ventricular failure, and Refractory hypotension after tumour resection. […] Preoperative adrenergic blockade achieves the following objectives: Lowers blood pressure, Increases intravascular volume, Reduces the chance of hypertensive crises during induction and tumour manipulation, Allows resensitisation of adrenergic receptors, Reduces myocardial dysfunction in the perioperative period. […] Commonly used -blockers include Phenoxybenzamine and Doxazosin. […] Calcium channel blockers inhibit norepinephrine-induced calcium influx and have been utilized for haemodynamic control before surgery. […] Intraoperative management should avoid drug-induced catecholamine release, avoid catecholamine release induced by anaesthetic or surgical manoeuvres, minimize haemodynamic responses to tumour handling, and treat episodes of hypotension, particularly after tumour devascularization. […] Postoperative care includes managing patients in an ITU/HDU, extubation at the end of surgery if uncomplicated intraoperatively, and ensuring invasive arterial pressure monitoring in a high dependency environment for at least 24 hours after the procedure.

• #2 Perioperative Management of Pheochromocytoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK589634/

  Pheochromocytoma (PCC) is a rare catecholamine-secreting tumor of adrenal origin. […] This activity entails pre-operative optimization of patients for resection of PCC. […] Post-operative management of patients has been discussed. […] Pre-op preparation and optimization are essential to decrease peri-operative morbidity and mortality in pheochromocytoma. […] It is vital to optimize intravascular volume to decrease peri-operative hemodynamic fluctuation. […] A detailed preoperative assessment by an experienced anesthesiologist is required to evaluate cardiovascular status, adequacy of alpha and beta blockade, volume resuscitation, and any other comorbidities. […] Minimally-invasive adrenalectomy via retroperitoneal or transperitoneal approach is the gold standard for pheochromocytoma resection. […] An interprofessional team consisting of an experienced surgeon, anesthesiologist, and endocrinologist is required. […] Pre-operative optimization with alpha-blocking agents and close hemodynamic monitoring in the peri-operative period is essential for the best outcome.

• #2 37 | How to confidently care for a pheochromocytoma patient as a vet nurse — Veterinary Internal Medicine Nursing

  https://www.veterinaryinternalmedicinenursing.com/blog/episode-37

  During the initial diagnostic period, our nursing focus is on the careful management of hypertension, accurate blood pressure measurement, and monitoring for those hypertensive crisis or collapse episodes. […] The majority of our nursing care is during surgery and in recovery. Under anaesthesia, these patients require careful monitoring. Placing an arterial catheter is recommended to facilitate direct blood pressure measurement. The patient should also have a central venous catheter or multiple IV catheters, including a rescue line or emergency line, that can be easily reached at all times during a hypertensive/tachycardic crisis under GA. […] A continuous ECG should also be placed and assessed throughout the anaesthetic for any cardiac arrhythmias. Antiarrhythmic and antihypertensive medications should be given as needed, and careful communication between the surgeon and anaesthesia team is vital – we should know if the surgeon is about to handle the tumour, for example, to ensure we have emergency drugs on hand.

• #2 37 | How to confidently care for a pheochromocytoma patient as a vet nurse — Veterinary Internal Medicine Nursing

  https://www.veterinaryinternalmedicinenursing.com/blog/episode-37

  In recovery, our nursing attention turns to ICU care and monitoring. Well monitor ECG and blood pressure frequently for the first 24 hours and then reduce the intensity of our monitoring as needed, alongside providing general nursing care. […] So thats my guide to managing patients with pheochromocytomas! Though these tumours are rare, we do see them, and given the associated risks, its vital that we understand what they are and how we can safely manage them as nurses. This includes careful monitoring, creating a stress-free environment, and intensive care alongside general nursing.

• #2 PERIOPERATIVE CARE OF PHAEOCHROMOCYTOMA – Anaesthesia News

  https://anaesthesianews.wordpress.com/2023/03/17/perioperative-care-of-phaeochromocytoma/

  Relief from anxiety prior to anesthetic induction is a key component, as apprehension can predispose to catecholamine surges. […] Aside from standard American Society of Anesthesiologists monitors, intra-arterial monitoring is the gold standard in the pheochromocytoma resection for beat-to-beat blood pressure monitoring. […] Persistent hypertension heralds more sinister causes such as incomplete tumour resection or metastatic disease. […] Lifelong steroid replacement is indicated if a bilateral adrenalectomy has been performed, but steroid supplementation is rarely required otherwise. […] Regular blood glucose monitoring and appropriate titration of dextrose infusions is therefore recommended.

• #2 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. […] A team of doctors and other professionals discuss the best treatment and care for you. They are called a multidisciplinary team (MDT). […] You may need to travel to a hospital that specialises in phaeochromocytoma for treatment. […] You usually have this done as keyhole surgery by a specialist surgeon. […] The anaesthetic and surgery can make the tumour release large amounts of adrenaline and noradrenaline. […] These drugs block the effect of the hormones on your blood pressure and help keep it stable. […] You will need to have hormone replacement if you have both adrenal glands removed. […] You have regular check ups after your treatment. […] It is important to go to your follow up appointments so that if it does come back the cancer is picked up sooner.

• #2 Post-Surgery Care for Pheochromocytoma Patients

  https://www.rupahealth.com/post/post-surgery-care-for-pheochromocytoma-patients

  A balanced diet with adequate protein, vitamins, and minerals is essential to aid in tissue healing and regain energy after surgery. […] Patients should avoid foods that may trigger hormonal spikes, such as caffeine or alcohol, as these can interfere with blood pressure control. […] By following these dietary and exercise guidelines, patients can promote long-term recovery and improve their quality of life. […] After surgery for pheochromocytoma, regular follow-ups with an endocrinologist are important. Ongoing monitoring helps detect any signs of tumor recurrence, even years after surgery. […] Patients typically undergo a combination of tests, including imaging (CT or MRI scans) and blood or urine tests, to check for elevated hormone levels, known as biochemical markers. These tests are usually done every few months in the first year after surgery and less frequently after that, depending on the patient’s health status and the risk of recurrence.

• #2 Pheochromocytoma | Stanford Health Care

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

  Pheochromocytomas are usually benign (noncancerous), but they require specialized care. […] These tumors release hormones that can cause life-threatening symptoms that include hypertension (high blood pressure), heart palpitations (rapid heartbeat), and headache. […] Surgery is the primary treatment for benign and cancerous pheochromocytomas. […] After surgery, people need ongoing treatment. […] Your doctor will monitor you carefully for ongoing effects from the tumor or pheochromocytoma cells elsewhere in your body. […] Genetic counseling plays a crucial role in managing pheochromocytomas. […] Our Endocrine Tumor Program includes a nationally recognized specialist in genetic testing and counseling specifically for endocrine tumors, including pheochromocytomas. […] Because many pheochromocytomas have a genetic cause, everyone diagnosed with pheochromocytoma tumors should have genetic testing. […] 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 goal is to catch other tumors as early as possible, so that you receive appropriate care.

• #2 Management of phaeochromocytoma and paraganglioma in patients with germline SDHB pathogenic variants: an international expert Consensus statement | Nature Reviews Endocrinology

  https://www.nature.com/articles/s41574-023-00926-0

  R1. We recommend that all major treatment and management decisions of patients with SDHB PPGLs should be carried out in an expert, interdisciplinary team conference to optimize care (Grade 1, very low). […] The management and treatment of patients with SDHB PPGLs pose a challenge to clinicians in many disciplines. These tumours are heterogeneous and can manifest in many organs with high rates of recurrence, local aggressive growth and metastasis over time. Most specialists across various disciplines have limited experience in this area of SDHB PPGLs and, therefore, international experts in this committee are convinced that an interdisciplinary discussion of management decisions in patients with SDHB PPGLs is an optimal approach. This approach also facilitates personalized tailoring of management in specific clinical situations, including plans for individualized surveillance and follow-up. However, this recommendation cannot be supported by evidence from well-designed clinical studies because such studies do not exist. Therefore, management decisions based on discussions in an interdisciplinary team with expertise in SDHB PPGLs are required to potentially achieve the most favourable outcomes for patients.

• #2 Pheochromocytoma NCLEX Review Notes

  https://www.registerednursern.com/pheochromocytoma-nclex-review-notes/

  When taking care of a patient with pheochromocytoma, it is very important the nurse knows how to recognize the typical signs and symptoms seen in these conditions, how it is diagnosed, nursing interventions, and patient education. […] Monitor vital signs: blood pressure, heart rate […] Monitor for hypertensive crisis: 180 systolic or 120 diastolic if blood pressure is too high for a long period of time this can cause damage to vital organs kidneys, eyes, brain, heart. […] Provide a calm and cool environment. no overstimulation! […] Administer pre-opt (prior to adrenalectomy) alpha-adrenergic blockers (Cardura, Minipress, Hyrtin): work by blocking noradrenaline, reduces catecholamines. […] Education for patient with pheochromocytoma: Eat high calorie diet: burning fats at a rapid rate […] Avoid stimulant substances: energy drinks, caffeine products, or smoking (due to vasoconstriction) […] For patient going for an adrenalectomy: educate about having to take hormone replacement medications after surgery and taking alpha-adrenergic prior to surgery (usually 2 weeks before surgery).

• #2 PERIOPERATIVE CARE OF PHAEOCHROMOCYTOMA – Anaesthesia News

  https://anaesthesianews.wordpress.com/2023/03/17/perioperative-care-of-phaeochromocytoma/

  Early phaeochromocytoma surgery saw mortality rates of up to 45%, with severe intraoperative hypertension, strokes, arrhythmias, myocardial ischaemia, left ventricular failure, and refractory hypotension after tumour resection. […] Although the evidence base has recently been questioned, preoperative -block is standard practice and aims to provide preoperative arterial pressure control with subsequent restoration of blood volume (which may be titrated using serial haematocrits). […] The optimal duration of -blockade therapy is undetermined and may range from 3 days to 2 weeks or longer. […] In addition to pharmacological control, a high sodium diet and fluid intake are also recommended to help restore blood volume. […] Tachyarrhythmias may result from epinephrine/dopamine-secreting tumours or be secondary to -blockade.

• #2 Phaeochromocytoma | Deranged Physiology

  https://derangedphysiology.com/main/required-reading/endocrine-intensive-care/Chapter-231/phaeochromocytoma

  Overall, one’s management should be guided by some sort of international consensus guidelines, which take the following shape: Control of hypertension: Rapidly acting α-1 antagonist: phentolamine, Slowly acting non-competitive α-1 antagonist: phenoxybenzamine, β-antagonist (after α-antagonist). Maintenance of circulating volume in the face of vasodilation: IV fluid replacement. Control of AF: Verapimil, diltiazem, or amiodarone. Assessment of myocardial damage: ECG, TTE, CK and troponin, Radionuclide imaging – the test of choice currently is 123I-labeled meta-iodobenzylguanide scintigraphy.

• #2 Post-Surgery Care for Pheochromocytoma Patients

  https://www.rupahealth.com/post/post-surgery-care-for-pheochromocytoma-patients

  Patients should be educated about the signs of recurrence, such as unexplained high blood pressure, headaches, or excessive sweating. […] In addition to medical follow-ups, emotional support plays a key role in long-term recovery. Support groups can provide a space for patients to connect with others who have gone through similar experiences, which can help alleviate feelings of isolation. […] Counseling may also be beneficial, as it helps patients cope with the stress and anxiety that can come with managing a long-term condition. By staying informed and engaged in follow-up care, patients can improve their quality of life and increase the chances of early detection if issues arise.

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

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

  Your hospital team, including your specialist nurse will help support you. […] Talking to other people who have the same type of tumour can also help. […] Cancer Research UK nurses provide general information and support. […] The Association for Multiple Endocrine Neoplasia Disorders (AMEND) is a patient group which was set up to support people affected by multiple endocrine neoplasia disorders. […] Neuroendocrine UK help support people with neuroendocrine tumours and their families.

• #3 Perioperative care of phaeochromocytoma | PPT

  https://www.slideshare.net/slideshow/perioperative-care-of-phaeochromocytoma-62265098/62265098

  Early phaeochromocytoma surgery saw mortality rates of up to 45% Severe intraoperative hypertension, Strokes, Arrhythmias, Myocardial ischaemia, Left ventricular failure, and Refractory hypotension after tumour resection. […] Preoperative adrenergic blockade achieves the following objectives: Lowers blood pressure, Increases intravascular volume, Reduces the chance of hypertensive crises during induction and tumour manipulation, Allows resensitisation of adrenergic receptors, Reduces myocardial dysfunction in the perioperative period. […] Commonly used -blockers include Phenoxybenzamine and Doxazosin. […] Calcium channel blockers inhibit norepinephrine-induced calcium influx and have been utilized for haemodynamic control before surgery. […] Intraoperative management should avoid drug-induced catecholamine release, avoid catecholamine release induced by anaesthetic or surgical manoeuvres, minimize haemodynamic responses to tumour handling, and treat episodes of hypotension, particularly after tumour devascularization. […] Postoperative care includes managing patients in an ITU/HDU, extubation at the end of surgery if uncomplicated intraoperatively, and ensuring invasive arterial pressure monitoring in a high dependency environment for at least 24 hours after the procedure.

• #3

  https://www.clinicsinsurgery.com/full-text/cis-v2-id1517.php

  Multiple successful strategies exist for the preoperative management of PPC patients. Even when patients are normotensive, preoperative preparation is required. Phenoxybenzamine effectively controls intraoperative hemodynamics but leads to prolonged postoperative hypotension. It also can lead to reflex tachycardia, central sedation and orthostatic hypotension. Selective alpha inhibitors such as urapidil, prazosin, terazosin, and doxazosin have less intraoperative hemodynamic control but also less postoperative hypotension and a decreased side effect profile. They have varying half-lives which are shorter than phenoxybenzamine, making them easier to titrate. Selective alpha inhibitors are currently more cost effective and are more widely available than phenoxybenzamine in many regions. Reflex tachycardia after the initiation of alpha-blockade may require beta-blockers such as metoprolol or atenolol. Dihydropyrimidine calcium channel blockers (i.e. amlodipine, nifedipine, and nicardipine) are widely available and effective at controlling intraoperative hemodynamics, particularly in smaller and less active tumors. They also can be added to an alpha-blockade for patients with poorly controlled hypertension. Metyrosine, while it should not be used as a monotherapy, is an excellent additional therapy that has been shown to reduce complications.

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

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

  In the preoperative period, the goal is to optimize the BP and normalize the intravascular volume. […] Patients are encouraged to be well hydrated with a high sodium diet prior to surgery. […] Selective or non-selective alpha blockade is always initiated first in preoperative preparation of patients with PPGLs. […] The communication between the anesthesiologist and the surgeon is paramount to a safe outcome. […] The surgical approaches for patients with pheochromocytoma include open and minimally invasive techniques. Minimally invasive techniques can be approached either laparoscopically or robotically. […] 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. Patients who have ongoing hemodynamic instability may require a monitored bed with telemetry or the intensive care unit. […] The extent of adrenalectomy can be dictated by genetic and familial predisposition.

• #3 PERIOPERATIVE CARE OF PHAEOCHROMOCYTOMA – Anaesthesia News

  https://anaesthesianews.wordpress.com/2023/03/17/perioperative-care-of-phaeochromocytoma/

  Relief from anxiety prior to anesthetic induction is a key component, as apprehension can predispose to catecholamine surges. […] Aside from standard American Society of Anesthesiologists monitors, intra-arterial monitoring is the gold standard in the pheochromocytoma resection for beat-to-beat blood pressure monitoring. […] Persistent hypertension heralds more sinister causes such as incomplete tumour resection or metastatic disease. […] Lifelong steroid replacement is indicated if a bilateral adrenalectomy has been performed, but steroid supplementation is rarely required otherwise. […] Regular blood glucose monitoring and appropriate titration of dextrose infusions is therefore recommended.

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

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

  Patients with hypertension prior to surgery often have rapid and significant improvement immediately after surgery. Patients often worry that removing an adrenal gland means their quality of life will be affected or they will need to be on hormone replacement after surgery. But that is not the case. The other adrenal gland produces all of the hormones that the body needs, and hormone replacement is not needed after adrenalectomy for pheochromocytoma. […] For patients who have non-cancerous tumors that are removed with surgery, more than 90% are doing well with no recurrence after 5 years. Tumors come back in less than 10% of patients, most often in cases where the tumor was malignant or the capsule of the tumor was broken during surgery. 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.

• #3 Managing Your Pheochromocytoma – Symptoms & Treatment | Carle.org

  https://carle.org/conditions/pheochromocytoma

  More than 90% of pheochromocytomas are in the adrenal glands and can be cured with surgery. Medicines to control blood pressure should be given before surgery. […] Temporary low and high blood pressure readings can occur while the tumor is being removed. […] DO tell your health care provider if you had pheochromocytomas before or family members have endocrine tumors. Your family may need screening blood or urine tests. […] DO call your health care provider if you have vision changes, severe headache, weakness on one side of the body, chest pains, or increasing palpitations. […] DO call your health care provider if you have ankle swelling, shortness of breath, or weakness or dizziness when standing. […] DO call your health care provider if symptoms return after surgery. […] DONT do strenuous exercise until your pheochromocytoma has been removed. […] DONT expect that your high blood pressure will be completely normal after the operation. Some permanent changes may have already occurred in the kidneys and blood vessels.

• #3 Pheochromocytoma Symptoms, Treatment, Diagnosis & More

  https://pheopara.org/education/pheochromocytoma

  Before undergoing surgery, the patient must be adequately blocked with medication. Going under anesthesia without being blocked is very dangerous. Anesthesia can cause tumors to release massive amounts of catecholamines. Manipulation of the tumor during surgery can also cause this release, which may result in a hypertensive crisis and even death. This is why it is not recommended for patients to have a biopsy of the tumor if pheo or para is suspected. […] A pheochromocytoma urine test and/or plasma tests should be repeated 2-8 weeks after surgery to check for any remaining disease. Long-term regular follow-up is recommended for all patients. Yearly urine or plasma tests for pheochromocytoma should be performed for life to detect remaining disease, return of the disease, or the development of metastases. For most people, follow-up CT or MRI is not needed if urine and plasma test results are normal. Exceptions to this may include the identification of a genetic mutation or if the primary tumor was large.

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

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

  Pheochromocytomas are adrenal tumors that are found in the in center, or medulla area, of the adrenal gland whereas paragangliomas (also referred to as extra-adrenal pheochromocytomas) are found outside the adrenal gland in the peripheral nervous system, most commonly along the aorta or in the neck by the carotid artery. Both can be either benign or malignant, and sometimes that distinction can be difficult to diagnose. […] The only treatment for a pheochromocytoma or paraganglioma is surgery to remove your adrenal gland (which includes your adrenal nodule), which should be performed by a specialized adrenal, endocrine surgeon. […] All patients with functional, hormone-producing paragangliomas and pheochromocytomas should be started on an alpha-blocker 7-14 days before surgery. […] 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. […] Additionally, all patients should be referred for genetic testing.

• #3 Management of phaeochromocytoma and paraganglioma in patients with germline SDHB pathogenic variants: an international expert Consensus statement | Nature Reviews Endocrinology

  https://www.nature.com/articles/s41574-023-00926-0

  R22. We suggest active surveillance for patients without symptoms who have a low tumour burden or otherwise indolent tumour behaviour, in whom treatment is not currently deemed beneficial (Grade 2, very low). […] R23. We recommend adrenoceptor blockers for the treatment of catecholamine-associated manifestations associated with SDHB PPGLs (Grade 1, low). […] R24. We recommend that medications that might elicit a catecholamine crisis in catecholamine-secreting SDHB PPGLs should be avoided (Grade 1, very low). […] R25. We recommend active surveillance in patients with very slowly progressing and/or stable SDHB PPGLs (usually for over 6-12 months) without relevant symptoms or signs (Grade 1, low). […] R26. We recommend chemotherapy with cyclophosphamide, vincristine and dacarbazine as the first-line therapy for rapidly progressive SDHB PPGLs or for patients with high visceral tumour burden, or potentially as a second-line therapy if there is rapid progression following other systemic therapies (Grade 1, low).

• #3 Pheochromocytoma – Nurses Revision

  https://nursesrevisionuganda.com/pheochromocytoma/

  Long-Term Follow-Up: Monitoring: Regular monitoring of blood pressure, catecholamine levels, and imaging studies to detect recurrence. Genetic Counseling: Offer genetic counseling and testing, especially for patients with a family history of pheochromocytoma or associated genetic syndromes. […] Nursing Care Plan: Pheochromocytoma: Assessment: Patient presents with hypertension, palpitations, headaches, excessive sweating, and anxiety. […] Risk for Hypertensive Crisis related to excessive catecholamine secretion as evidenced by severe hypertension, palpitations, and headaches. […] Patient will verbalize understanding of the disease and treatment plan. […] Patient will express reduced fear and anxiety about surgery. […] Patient will adhere to preoperative and postoperative care instructions.

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

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

  Your hospital team, including your specialist nurse will help support you. […] Talking to other people who have the same type of tumour can also help. […] Cancer Research UK nurses provide general information and support. […] The Association for Multiple Endocrine Neoplasia Disorders (AMEND) is a patient group which was set up to support people affected by multiple endocrine neoplasia disorders. […] Neuroendocrine UK help support people with neuroendocrine tumours and their families.

• #3 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. […] A team of doctors and other professionals discuss the best treatment and care for you. They are called a multidisciplinary team (MDT). […] You may need to travel to a hospital that specialises in phaeochromocytoma for treatment. […] You usually have this done as keyhole surgery by a specialist surgeon. […] The anaesthetic and surgery can make the tumour release large amounts of adrenaline and noradrenaline. […] These drugs block the effect of the hormones on your blood pressure and help keep it stable. […] You will need to have hormone replacement if you have both adrenal glands removed. […] You have regular check ups after your treatment. […] It is important to go to your follow up appointments so that if it does come back the cancer is picked up sooner.

• #4 Management of phaeochromocytoma and paraganglioma in patients with germline SDHB pathogenic variants: an international expert Consensus statement | Nature Reviews Endocrinology

  https://www.nature.com/articles/s41574-023-00926-0

  R27. We recommend that targeted radionuclide therapy with iodine-131 meta-iodobenzylguanidine ([131I]MIBG) or peptide receptor radionuclide therapy (PRRT) should be considered as a first-line treatment in patients with inoperable SDHB PPGL if there is slow-to-moderate progression with moderate-to-high tumour burden. [131I]MIBG or PRRT might be considered for patients with metastatic disease as a first-line treatment if there are signs and symptoms owing to uncontrolled catecholamine excess (such as hypertension, tachyarrhythmias and other cardiovascular events) or if there are mass-related effects (Grade 1, low).

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