open access

Vol 6, No 3 (2002)
REVIEV
Published online: 2002-07-01
Get Citation

Pheochromocytoma - Disease that Can Stimulate Many Different Syndromes

Włodzimierz Januszewicz, Aleksander Prejbisz, Andrzej Januszewicz, Mariola Pęczkowska
Nadciśnienie tętnicze 2002;6(3):217-228.

open access

Vol 6, No 3 (2002)
REVIEV
Published online: 2002-07-01

Abstract

Pheochromocytoma is a rare but important tumour of chromaffin cells. Clinical and biochemical manifestation are mainly caused by excess of circulating catecholamines and hypertension. Manifestations mimics many conditions, which may result in erroneous diagnoses and improper treatment. Most pheochromocytomas are sporadic and up to 25 percent are hereditary. Pheochromocytoma can be a component of multiple endocrine neoplasia type 2 (MEN-2), von Hippel-Lindau disease, familial paragangliomas syndrome and neurofibromatosis type 1. Diagnosis of pheochromocytoma depends on biochemical evidence of catecholamine production by the tumor. Recent findings have demonstrated high sensitivity of plasma and urinary levels of metanephrines for detecting pheochromocytoma. An algorithm for clinical diagnostic steps is presented. Surgical resection by laparoscopic or open adrenalectomy can be curative. Drug treatment prior to and during surgery is mandatory. Lifelong follow-up after surgical removal of pheochromocytoma is recommended. Routine analysis for mutations of susceptibility genes for pheochromocytoma is indicated to identify pheochromocytoma-associated syndromes.

Abstract

Pheochromocytoma is a rare but important tumour of chromaffin cells. Clinical and biochemical manifestation are mainly caused by excess of circulating catecholamines and hypertension. Manifestations mimics many conditions, which may result in erroneous diagnoses and improper treatment. Most pheochromocytomas are sporadic and up to 25 percent are hereditary. Pheochromocytoma can be a component of multiple endocrine neoplasia type 2 (MEN-2), von Hippel-Lindau disease, familial paragangliomas syndrome and neurofibromatosis type 1. Diagnosis of pheochromocytoma depends on biochemical evidence of catecholamine production by the tumor. Recent findings have demonstrated high sensitivity of plasma and urinary levels of metanephrines for detecting pheochromocytoma. An algorithm for clinical diagnostic steps is presented. Surgical resection by laparoscopic or open adrenalectomy can be curative. Drug treatment prior to and during surgery is mandatory. Lifelong follow-up after surgical removal of pheochromocytoma is recommended. Routine analysis for mutations of susceptibility genes for pheochromocytoma is indicated to identify pheochromocytoma-associated syndromes.
Get Citation

Keywords

pheochromocytoma; hypertension; diagnosis; treatment

About this article
Title

Pheochromocytoma - Disease that Can Stimulate Many Different Syndromes

Journal

Arterial Hypertension

Issue

Vol 6, No 3 (2002)

Pages

217-228

Published online

2002-07-01

Bibliographic record

Nadciśnienie tętnicze 2002;6(3):217-228.

Keywords

pheochromocytoma
hypertension
diagnosis
treatment

Authors

Włodzimierz Januszewicz
Aleksander Prejbisz
Andrzej Januszewicz
Mariola Pęczkowska

Important: This website uses cookies. More >>

The cookies allow us to identify your computer and find out details about your last visit. They remembering whether you've visited the site before, so that you remain logged in - or to help us work out how many new website visitors we get each month. Most internet browsers accept cookies automatically, but you can change the settings of your browser to erase cookies or prevent automatic acceptance if you prefer.

By "Via Medica sp. z o.o." sp.k., ul. Świętokrzyska 73, 80–180 Gdańsk

tel.:+48 58 320 94 94, faks:+48 58 320 94 60, e-mail: viamedica@viamedica.pl