Materiały źródłowe

• #1 Pheochromocytoma: Causes, Symptoms & Treatment

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

  Unfortunately, you cant prevent developing a pheochromocytoma. However, if youre at risk for developing a pheochromocytoma due to certain inherited syndromes and genes, genetic counseling can help screen for pheochromocytoma and potentially help you catch it in its early phases. […] Talk to your healthcare provider if you have any first-degree relatives (siblings and parents) that have been diagnosed with pheochromocytoma and/or any of the following genetic conditions: Multiple endocrine neoplasia 2 syndrome, Von Hippel-Lindau (VHL) disease, Neurofibromatosis type 1 (NF1), Hereditary paraganglioma syndrome, Carney-Stratakis dyad, Carney triad.

• #1 Hereditary Paraganglioma and Pheochromocytoma Panel Test – PreventionGenetics

  https://www.preventiongenetics.com/testInfo?val=Hereditary-Paraganglioma-and-Pheochromocytoma-Panel

  Individuals with a clinical or family history of hereditary PGL/PCC syndrome should be tested early. Early diagnosis may improve patient prognosis through regular screening and treatment for early-onset malignancies. Early detection through surveillance and removal of tumors may prevent or minimize complications related to mass effects, catecholamine hypersecretion, and malignant transformation. […] Early diagnosis may improve patient prognosis through regular screening and treatment for early-onset malignancies. Early detection through surveillance and removal of tumors may prevent or minimize complications related to mass effects, catecholamine hypersecretion, and malignant transformation.

• #1 Pheochromocytoma – USZ

  https://www.usz.ch/en/disease/pheochromocytoma/

  The causes of pheochromocytoma are unknown in many cases. Therefore, you cannot prevent the tumor in the adrenal medulla in sporadic cases. There are also no special early detection measures for pheochromocytoma. […] However, hereditary factors play a role in around 25 to 40 percent of people with a pheochromocytoma. Regular check-ups are advisable, for example for healthy family members with the corresponding genetic changes: […] If you have a known genetic disease such as neurofibromatosis type 1, multiple endocrine neoplasia type 2 a/b, Von Hippel-Lindau syndrome or paraganglioma syndrome, you must have your disease monitored closely and regularly by an endocrinologist. In this way, a pheochromocytoma can be detected and treated at an early stage. […] As pheochromocytoma is a rare disease, treatment in a specialized center is advisable. In our adrenal consultation at the USZ, you will be advised on the necessary clarifications (genetic examination) and the subsequent follow-up care (regular imaging, screening for other tumors depending on the underlying disease).

• #1

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

  Pheochromocytomas and paragangliomas are catecholamine-producing neoplasms that can cause life-threatening hemodynamic instability, particularly intraoperatively, when the tumor is manipulated. Preoperative medical management reduces both preoperative morbidity and mortality. The current review discusses the latest literature on preoperative management. Preoperative strategies include a nonselective alpha-antagonist, selective alpha antagonists, calcium channel blockers, tyrosine hydroxylase inhibitors, and fluid and salt loading. […] The goal of preoperative care is to prevent a sudden release of catecholamines or attenuate the response to a release of catecholamines perioperatively. There are many ways to achieve this. […] Preoperative preparation should be initiated, even in normotensive or asymptomatic patients.

• #1

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

  The totality of evidence suggests that all patients received preoperative blockade until orthostatic hypotension is achieved. […] Even when patients are normotensive, preoperative preparation is required. […] 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. […] 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. […] Metyrosine, while it should not be used as a monotherapy, is an excellent additional therapy that has been shown to reduce complications. […] Although no studies have specifically addressed the practice of fluid and electrolytes resuscitation in preparation for surgery, many highly recommend it. […] Reducing anxiety prior to induction is crucial to prevent apprehension from causing catecholamine surges.

• #1

  https://scibasejournals.org/clinical-and-medical-case-reports/1007.html

  Preoperative blood pressure control in phaeochromocytoma is an uphill task using multiple medications leading to post operative hypotension. […] It is well known that complete prevention of perioperative hypertension and tachycardia cannot be achieved by any dose or combination of drugs. […] We thus achieved adequate preoperative blood pressure control with no untoward complications intra and postoperatively. […] Perioperative blood pressure control can be achieved with the help of preoperative epidural infusion of drugs instead of systemic antihypertensive medications in a case of surgical resection of a phaeochromocytoma. […] Further research is needed on this intervention.

• #1

  https://journals.lww.com/joacp/fulltext/2020/36010/peri_operative_management_of_pheochromocytoma_with.9.aspx

  The main objective of our retrospective observational study was to evaluate the efficacy of pre-operative preparation by continuous IV infusion of Urapidil in the prevention of hypertensive peaks (HTN) (SAP 180 mmHg), and tolerance, represented by hypotension (SAP 80 mmHg) during the peri-operative management of 79 cases (75 patients) of PCC ablation. […] The relatively large sample size of our cohort with the same anesthetic and surgical management shows that the use of Urapidil in the peri-operative period of these patients is safe and efficient to prevent HTN and hypotension. As compared to other blockers given orally, the IV route is an interesting alternative. It permits patients to have controlled blood pressure and heart rate with a single drug upon arrival in the operating room. It facilitates the treatment of hemodynamic disorders, fits the surgical imperatives by allowing a shorter hospital stay. Further studies are warranted to confirm these data.

• #1

  https://link.springer.com/article/10.1007/s11845-015-1383-5

  A multidisciplinary team approach is best throughout the perioperative period to prevent potential complications that arise. […] In addition to the pharmacologic and volume recommendations, a multidisciplinary discussion allows for seamless implementation of an organized plan of care.

• #1 PERSPECTIVE: Challenging preoperative α-blockade in phaeochromocytoma surgery: beyond tradition, towards ‘safer surgery’ in: Endocrine Connections Volume 14 Issue 5 (2025)

  https://ec.bioscientifica.com/view/journals/ec/14/5/EC-25-0139.xml

  The rarity of phaeochromocytomas has left a gap in evidence supporting guideline recommendations for preoperative -blockade dose-escalation. […] Despite these outcomes, randomized-controlled trials (RCTs) are warranted before considering omitting preoperative -blockade dose-escalation. […] The survey furthermore revealed diverse opinions regarding study design and perioperative threats, emphasizing the importance of an inclusive, multidisciplinary approach in future research. […] The diverse perspectives regarding study design and perioperative threats, alongside the predominant monodisciplinary focus of current guidelines led by endocrinologists, emphasize the necessity of a multidisciplinary approach in future research, particularly when conducting RCTs. […] In conclusion, recent studies highlight the evolving understanding of phaeochromocytoma management. More research, preferably RCTs, is needed to improve treatment strategies and patient outcomes. Our survey suggests a non-inferiority-designed RCT that only excludes patients with severe comorbidities and incorporates clinical outcome measures as most suitable design.

• #1 A case of anaphylaxis in phaeochromocytoma | SFEBES2019 | Society for Endocrinology BES 2019 | Endocrine Abstracts

  https://www.endocrine-abstracts.org/ea/0065/ea0065p56

  Introduction: The physiological actions of catecholamines have led to the empirical use of adrenaline in the management of anaphylaxis, with alpha-adrenergic activation increasing peripheral vascular resistance and beta-adrenergic activation relaxing bronchial smooth muscle and inhibiting histamine release. […] Hence blockade of these same receptors is required for symptom control, prevention of end organ damage and to facilitate safer operative management. […] Conclusion: Anaphylaxis in patients with phaeochromocytoma can occur despite supraphysiological levels of catecholamines. Possible reasons for this are the down regulation of adrenergic receptors due to chronic exposure to catecholamines, and the effect of optimised alpha blockade, leading to severe anaphylaxis upon exposure to a trigger.

• #1 Pheochromocytoma crisis and systemic corticosteroids

  https://www.medsafe.govt.nz/profs/PUArticles/December2022/Pheochromocytoma-crisis-systemic-corticosteroids.html

  Pheochromocytoma crisis has been reported following the administration of systemic corticosteroids to patients with pheochromocytoma. […] Medsafe has asked sponsors of systemic corticosteroid medicines to update their data sheets with a warning for pheochromocytoma crisis. […] Pheochromocytoma crisis, which can be fatal, has been reported after administration of systemic corticosteroids. Corticosteroids should only be administered to patients with suspected or identified pheochromocytoma after an appropriate risk/benefit evaluation.

• #2 Pheochromocytoma – USZ

  https://www.usz.ch/en/disease/pheochromocytoma/

  The causes of pheochromocytoma are unknown in many cases. Therefore, you cannot prevent the tumor in the adrenal medulla in sporadic cases. There are also no special early detection measures for pheochromocytoma. […] However, hereditary factors play a role in around 25 to 40 percent of people with a pheochromocytoma. Regular check-ups are advisable, for example for healthy family members with the corresponding genetic changes: […] If you have a known genetic disease such as neurofibromatosis type 1, multiple endocrine neoplasia type 2 a/b, Von Hippel-Lindau syndrome or paraganglioma syndrome, you must have your disease monitored closely and regularly by an endocrinologist. In this way, a pheochromocytoma can be detected and treated at an early stage. […] As pheochromocytoma is a rare disease, treatment in a specialized center is advisable. In our adrenal consultation at the USZ, you will be advised on the necessary clarifications (genetic examination) and the subsequent follow-up care (regular imaging, screening for other tumors depending on the underlying disease).

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

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

  There’s no way to prevent a pheochromocytoma from developing. But those at risk of developing the tumor due to certain inherited conditions or genes can seek genetic testing to screen for it. Talk to your doctor if you have a parent or sibling with paraganglioma or pheochromocytoma or one of the following conditions: […] Multiple endocrine neoplasia, type 2 (MEN2) […] Von Hippel-Lindau disease […] Neurofibromatosis 1 (NF1) […] Carney-Stratakis dyad […] Carney triad.

• #2

  https://journals.lww.com/joacp/fulltext/2020/36010/peri_operative_management_of_pheochromocytoma_with.9.aspx

  Surgery for pheochromocytoma (PCC) can cause excessive catecholamine release with severe hypertension. Alpha blockade is the mainstay of preoperative management. The aim of this study was to evaluate the efficacy and tolerance of intra-venous (IV) urapidil, a competitive short acting 1 receptor antagonist, in the prevention of peri-operative hemodynamic instability of patients with PCC. […] To prevent these potentially fatal events, pre-operative management is necessary. Scholten et al. demonstrated that emergency surgery in untreated patients leads to more post-operative complications, intensive care unit admissions and longer hospital stay than treated patients. As a result, European and Americans endocrine societies have issued recommendations for the peri-operative management of PCC in 2014 with mandatory initiation of blocker, even in normotensive patients, to prevent an unpredictable per-operative hemodynamic instability.

• #2 Perioperative hemodynamic instability in patients undergoing laparoscopic adrenalectomy for pheochromocytoma – Pisarska – Gland Surgery

  https://gs.amegroups.org/article/view/11816/html

  Currently the gold standard in the treatment of pheochromocytoma is preoperative use of alpha-blockers and laparoscopic surgery. […] It is generally accepted that every patient with suspected pheochromocytoma requires appropriate preoperative optimization with antihypertensive drugs and blood volume expansion fluids. […] In summary, appropriate preoperative alpha or/and beta-blockade allows significant reduction in cardiovascular morbidity and mortality as demonstrated by Goldstein et al. from 69% to 3%. […] According to previously published studies such combination allows better blood pressure stabilization, reduced intraoperative blood loss and volume replacement during surgery compared to alpha-blockers alone. […] In conclusion, hemodynamic instability is one of the most commonly diagnosed adverse events in patients undergoing laparoscopic adrenalectomy for pheochromocytoma.

• #2 Perioperative hemodynamic instability in patients undergoing laparoscopic adrenalectomy for pheochromocytoma – Pisarska – Gland Surgery

  https://gs.amegroups.org/article/view/11816/html

  Appropriate preoperative assessment and treatment with antihypertensive drugs significantly reduces the incidence of hemodynamic changes during and after surgery, thus minimizing serious complications and mortality. […] To minimize morbidity and mortality a close cooperation between the surgeon and anesthetist is mandatory in every patient with suspected or confirmed pheochromocytoma.

• #3 Pheochromocytoma – USZ

  https://www.usz.ch/en/disease/pheochromocytoma/

  The causes of pheochromocytoma are unknown in many cases. Therefore, you cannot prevent the tumor in the adrenal medulla in sporadic cases. There are also no special early detection measures for pheochromocytoma. […] However, hereditary factors play a role in around 25 to 40 percent of people with a pheochromocytoma. Regular check-ups are advisable, for example for healthy family members with the corresponding genetic changes: […] If you have a known genetic disease such as neurofibromatosis type 1, multiple endocrine neoplasia type 2 a/b, Von Hippel-Lindau syndrome or paraganglioma syndrome, you must have your disease monitored closely and regularly by an endocrinologist. In this way, a pheochromocytoma can be detected and treated at an early stage. […] As pheochromocytoma is a rare disease, treatment in a specialized center is advisable. In our adrenal consultation at the USZ, you will be advised on the necessary clarifications (genetic examination) and the subsequent follow-up care (regular imaging, screening for other tumors depending on the underlying disease).

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