Vol 6, No 3 (2002)
Review paper
Published online: 2002-07-01
Pheochromocytoma - Disease that Can Stimulate Many Different Syndromes
Włodzimierz Januszewicz, Aleksander Prejbisz, Andrzej Januszewicz, Mariola Pęczkowska
Nadciśnienie tętnicze 2002;6(3):217-228.
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.
Keywords
pheochromocytoma; hypertension; diagnosis; treatment
Title
Pheochromocytoma - Disease that Can Stimulate Many Different Syndromes
Journal
Arterial Hypertension
Issue
Vol 6, No 3 (2002)
Article type
Review paper
Pages
217-228
Published online
2002-07-01
Page views
1085
Article views/downloads
4198
Bibliographic record
Nadciśnienie tętnicze 2002;6(3):217-228.
Keywords
pheochromocytoma
hypertension
diagnosis
treatment
Authors
Włodzimierz Januszewicz
Aleksander Prejbisz
Andrzej Januszewicz
Mariola Pęczkowska