Clinical significance of the association of the Von Hippel-Lindau disease with pheochromocytoma and pancreatic apudoma

The American Journal of the Medical Sciences
J L MulshineR W Gifford

Abstract

Von Hippel-Lindau disease (VHLD) is a rare disorder known to occur with pheochromocytoma. We report a case with this association and also with pancreatic apudoma. We believe that this is the fifth reported case of this triad, which represents an overlap in the multiple endocrine neoplasia (MEN) classification. Many recent reports of overlap of MEN syndromes challenge the validity of this classification. New understanding of the embryologic origin of cells of endocrine organs has led to the elucidation of the APUD (amine precursor uptake and decarboxylation)-neuroendocrine system. Integration of the APUD system may be important to understanding the pathogenesis of the MEN-like syndromes as well as their relationship to the neurocutaneous syndromes. Any endocrine tumor or neurocutaneous syndrome should be evaluated to exclude a spectrum of possible associations.

Citations

Mar 21, 2006·Journal of Hepato-biliary-pancreatic Surgery·Akihiro OsawaToshiaki Oharazeki
Oct 23, 2002·Clinical Endocrinology·B MukhopadhyayS L Chew
Nov 1, 1990·American Journal of Medical Genetics·R N Schimke

Related Concepts

Angiomatosis
Apudoma
Von Hippel-Lindau Syndrome
Malignant Neoplasm of Pancreas
Genealogical Tree
Pheochromocytoma, Extra-Adrenal

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