Neurofibromatosis-Pheochromocytoma-Duodenal Carcinoid Syndrome

From their own experience and from the literature, Griffiths et al. (1983) collected 3 patients with duodenal carcinoid tumor in association with neurofibromatosis and pheochromocytoma and 4 patients with duodenal carcinoid with either von Recklinghausen disease or pheochromocytoma. The duodenal carcinoids had an unusual morphology. They reacted only weakly to normal silver impregnation techniques and had an unusual glandular pattern, and 3 of them contained psammoma bodies. Three were strongly positive on immunolocalization with an antibody to somatostatin (which stains normal pancreatic D cells). Griffiths et al. (1983) suggested that neurofibromatosis, pheochromocytoma, and duodenal carcinoid constitute a distinct and specific multiple endocrine neoplasia syndrome. They suggested that it might be termed MEN IIIa, with MEN IIIb being assigned to the von Hippel-Lindau syndrome (193300) with pheochromocytoma and islet cell tumors. In only 1 of the cases was the neurofibromatosis familial. It appears that the cutaneous involvement was typical of von Recklinghausen disease (162200) in all the cases. Obstructive jaundice (due to the duodenal carcinoid) was the manner of clinical presentation in 2 of the cases. Symptoms of somatostatin secretion by the carcinoid were not observed.