Anti-hypertensive treatment in pheochromocytoma and paraganglioma: current management and therapeutic features

  title={Anti-hypertensive treatment in pheochromocytoma and paraganglioma: current management and therapeutic features},
  author={Alberto Mazza and Michela Armigliato and Maria Cristina Marzola and Laura Schiavon and Domenico Montemurro and Giorgio Vescovo and Marco Zuin and Sotirios Chondrogiannis and Roberta Ravenni and Giuseppe Opocher and Patrick M Colletti and Domenico Rubello},
Pheochromocytoma (PH) and paraganglioma (PG) are neuroendocrine neoplasms arising from chromaffin cells of the adrenal medulla and the sympathetic ganglia, respectively. Although are unusual cause of hypertension (HT) accounting for at most 0.1–0.2 % of cases, they may lead to severe and potentially lethal hypertensive crisis due to the effects of the released catecholamines. However, both PH and PG may be asymptomatic as ~30 % of subjects are normotensive or have orthostatic hypotension and in… 

Pheochromocytoma and Paraganglioma

Pheochromocytomas and paragangliomas are tumors of the chromaffin cells of the adrenal medulla and autonomic nervous system, respectively that are a rare cause of secondary hypertension, cardiomyopathy, myocardial infarction, stroke, and death.

Anaesthetic Management of Extramedullary Pheochromocytoma

The high incidence of pheochromocytoma in families as a primary disease, in association with multiple endocrine neoplasia or other familial diseases such as Von Hippel Lindau syndrome and neurofibromatosis I, indicates the need for genetic counseling.

Pheochromocytoma and paraganglioma—an update on diagnosis, evaluation, and management

Since genetic mutations have been reported in tumor susceptibility genes in nearly 50% of patients with PPGLs, genetic counselling and testing should be considered in all patients with a confirmed tumor.

Treatment of pheochromocytomas and paragangliomas: genetic approach?

Pheochromocytomas/PGLs are neuroendocrine tumors derived from the chromaffin tissue of the adrenal medulla or from extra-adrenal sympathetic and parasympathetic paraganglia that affect one in 2500–6500 people, with 500–1600 patients diagnosed annually in the USA.

Therapeutic goals in patients with pheochromocytoma: a guide to perioperative management

A multidisciplinary team approach is best throughout the perioperative period to prevent potential complications that arise and understand the pathophysiology of pheochromocytoma, the pharmacology of medications used, and recognizing postoperative complications.

Approach to the Patient: Perioperative Management of the Patient with Pheochromocytoma or Sympathetic Paraganglioma.

The underlying evidence and the pros and cons of presurgical medical preparation are discussed, and the areas of uncertainty and controversies in this field are addressed.

Medical Management of Pheochromocytoma

Patients with catecholamine-secreting pheochromocytomas and paragangliomas require medical treatment to control blood pressure and tachyarrhythmias and to prepare the patient for surgical tumor

Preoperative Management of Pheochromocytoma and Paraganglioma

The pathogenesis of this disease is reviewed, the current approaches and evidence available for preoperative management are discussed, and the results of the latest studies which compared the efficacies of preoperatively management with or without α adrenergic-receptor antagonists are summarized to facilitate better understanding of the preoperativemanagement of PPGL for the physicians.

Severe hypertension complicating pregnancy, pheochromocytoma: A rare case report

Pheochromocytoma should be considered if a patient has labile hypertension, hypertension resistant to treat by antihypertensive therapy, or paroxysmal symptoms (“spells”).

Successful management of a third-trimester pregnancy complicated by pheochromocytoma: case report

The specific and unique pharmacological treatment with doxazosin, the use of corticosteroids and a close monitoring of fetal well-being which proved being an effective approach are described.



Pheochromocytoma: an approach to antihypertensive management.

  • E. Bravo
  • Medicine
    Annals of the New York Academy of Sciences
  • 2002
The question debated most often regarding medical therapy of pheochromocytoma is whether antihypertensive treatment regimens other than nonspecific alpha-blockade are just as effective and safe.

[Hypertension secondary to multiple malignant paragangliomas].

In this patient the diagnosis of retroperitoneal multiple paragangliomas was made two years after the beginning of the typical symptoms, and the treatment was confirmed at six months follow-up, in consideration of the persistently high norepinephrine plasma levels.

Pheochromocytoma in Pregnancy: A Case Series and Review

It is imperative that physicians who manage patients with pheochromocytoma familiarize themselves with special considerations in relation to pheolamine producing tumor during pregnancy, as well as understanding the indications of when women with chronic or de novo hypertension during gestation should undergo the special tests used to diagnose and how to manage the disease once diagnosed.

Urapidil in the Preoperative Treatment of Pheochromocytomas: A Safe and Cost-effective Method

Perioperative treatment with the selective α1 blocker urapidil remains a simple and cost effective method in the treatment regime of patients with pheochromocytoma.

Pheochromocytoma and pregnancy: a deceptive connection.

  • J. Lenders
  • Medicine
    European journal of endocrinology
  • 2012
Despite all technical diagnostic and therapeutic progress over the last decades, the key factor for further reduction of maternal and fetal mortality is early awareness and recognition of the potential presence of a pheochromocytoma in a pregnant patient with hypertension.

Severe paroxysmal hypertension (Pseudopheochromocytoma)

  • S. Mann
  • Medicine, Psychology
    Current hypertension reports
  • 2008
An approach to understanding and successfully treating paroxysmal hypertension and three forms of intervention, alone or in combination, appear successful: antihypertensive therapy with agents directed at the sympathetically mediated blood pressure elevation, psychopharmacologic interventions including anxiolytic and/or antidepressant agents; and psychological intervention, particularly reassurance and increased psychological awareness.

Pheochromocytoma/Paraganglioma: Review of Perioperative Management of Blood Pressure and Update on Genetic Mutations Associated With Pheochromocytoma

This review focuses on the perioperative management of pheochromocytoma and paragangliomas and the clinical implications of the associated genetic mutations.

CASE REPORT PHEOCHROMOCYTOMA IN PREGNANCY Experience of treatment with phenoxybenzamine in three patients

It is concluded that pheochro- mocytoma diagnosed during the 1st trimester is probably best managed by tumor resection as soon as possible, to avoid the risk of abortion during surgery in cases diagnosed in the 2nd trimester.

Prevalence of clinically unsuspected pheochromocytoma. Review of a 50-year autopsy series.

Pheochromocytoma and Paraganglioma in Children: A Review of Medical and Surgical Management at a Tertiary Care Center

Patients with a known genetic mutation or familial pheochromocytoma/paraganglioma were more likely to achieve resection with negative microscopic margins and had improved disease-specific mortality rates.