Korean J Hepatobiliary Pancreat Surg.  2000 Feb;4(1):191-195.

Pheochromocytoma with pancreatic islet cell tumor: a case report

• Ju YT
• Jung EJ
• Park SH
• Hong SC

Abstract

A 17-years old woman with a combination of unilateral pheochromocytoma and an asymptomatic islet cell tumor of the pancreas is presented. The unusual coincidence of pheochromocytoma and pancreatic islet cell tumor in the patient is of interest as a possible crossover between MEN I and MEN II. It has been suggested that MEN I represents an abnormality of the APUD(amine precursor uptake and decarboxylation) cells of ectodermal origin. However, the possibility of a common progenitor cell in the neural crest for all APUD cells has been suggested by Pearse and Polak(1971). Recent clinical reports suggest that overlap between the two syndroms may occur. The overlapping of elements of the classical endocrine neoplasia should alert clinicians to the possibility of such associations in any particular patients. Since islet cell tumors may occur in association with pheochromocytomas and may be clinically silent, exploration of the pancreas during surgery for pheochromocytomas would seem to be useful.

Keyword

pheochromocytoma; pancreatic islet cell tumor; MEN

MeSH Terms

Adenoma, Islet Cell
Adolescent
APUD Cells
Ectoderm
Female
Humans
Islets of Langerhans*
Male
Multiple Endocrine Neoplasia Type 1
Multiple Endocrine Neoplasia Type 2a
Neural Crest
Pancreas
Pheochromocytoma*
Pyrus
Stem Cells