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Int J Surg Pathol. 2014 Aug;22(5):393-400. doi: 10.1177/1066896914537683. Epub 2014 Jun 5.

Hereditary Pheochromocytoma.

Author information

1
Faculdade de Medicina, Universidade do Porto, Porto, Portugal.
2
Faculdade de Medicina, Universidade do Porto, Porto, Portugal Assistente Hospitalar de Cirurgia Geral do Centro Hospitalar S. João, Porto, Portugal.
3
Faculdade de Medicina, Universidade do Porto, Porto, Portugal Assistente Hospitalar Graduado de Cirurgia Geral, Diretor do Serviço de Cirurgia Geral, Serviço de Cirurgia Geral do Hospital Pedro Hispano, Senhora da Hora, Portugal ataveira@med.up.pt.

Abstract

INTRODUCTION:

Pheochromocytomas (PHEO) and paragangliomas (PGL) are rare neuroendocrine tumors with an estimated occurrence of 2 to 5 patients per million per year and an incidence of about 1 per 100 000 in the general population. These tumors may arise sporadically or be associated to various syndromes, namely multiple endocrine neoplasia type 2, neurofibromatosis type 1, Von Hippel-Lindau syndrome, and hereditary paraganglioma-pheochromocytoma syndromes.

OBJECTIVES:

This article aims to review the current epidemiology, pathogenesis, clinical presentation, and genetic aspects of syndromes associated with hereditary PHEO/PGL.

METHODS:

The literature research, conducted at PubMed database, included review articles, published from February 2009 to February 2014, written in English or Portuguese, using as query: "Hereditary AND Pheochromocytoma."

CONCLUSION:

These tumors can be part of a myriad hereditary conditions that are not yet fully understood. Nevertheless, important systemic symptoms and even fatal outcomes can occur. Knowledge of these hereditary conditions can ensure a more efficient detection, treatment, and even prevention of these neuroectodermal tumors, thus new tests and studies should be conducted.

KEYWORDS:

hereditary pheochromocytoma; paraganglioma; pheochromocytoma

PMID:
24903423
DOI:
10.1177/1066896914537683
[Indexed for MEDLINE]

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