Coexistence of bilateral pheochromocytoma and pancreatic islet cell tumor: report of a case and review of the literature.
A 14-year-old Japanese male with a previously undescribed combination of bilateral pheochromocytoma and an islet cell tumor of the pancreas is presented. The combination of endocrine neoplasms in this patient overlaps multiple endocrine neoplasia (MEN) Type 1 and Type 2. A total of 14 reported cases of MEN overlapping Type 1 and Type 2 is reviewed. Of the 14, 7 patients with acromegaly developed a paraganglioma(s), 2 patients with Sipple syndrome had a pituitary adenoma, and in the other 5 patients, an intestinal carcinoid or a pancreatic islet-cell tumor occurred in association with either a thyroid medullary carcinoma or a paraganglioma(s). We believe that the occurrence of MEN overlapping Type 1 and Type 2 is more than a fortuitous association, and can be explained on the basis of the neuroectodermal origin.
D007516:Adenoma, Islet Cell / Q000473:pathology*; D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000473:pathology*; D000328:Adult; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000473:pathology*; D010190:Pancreatic Neoplasms / Q000473:pathology*; D010235:Paraganglioma / Q000473:pathology; D009384:Paraneoplastic Endocrine Syndromes / Q000196:embryology / Q000473:pathology*; D010673:Pheochromocytoma / Q000473:pathology*