The association of neurofibromatosis, pheochromocytoma, and somatostatin-rich duodenal carcinoid tumor.
The association of neurofibromatosis and pheochromocytoma is well recognized; more recently, attention has been drawn to links between neurofibromatosis, pheochromocytoma, and ampullary somatostatin-rich carcinoid. Because of this association, the duodenum was explored during a recent laparotomy for resection of bilateral pheochromocytoma in a patient with von Recklinghausen's disease. A clinically unsuspected ampullary tumor was discovered; this proved to be in part a ganglioneuroma and in part a somatostatin-rich carcinoid. This paper presents full details of this carefully investigated and documented case and reviews the recent advances in this field. These studies lead us to conclude that: the clinical association of neurofibromatosis, pheochromocytoma, and D cell carcinoids ("somatostatinomas") of the ampullary region is confirmed; this association may be more common than has been previously thought, and the duodenum should be carefully examined in any patient with neurofibromatosis who undergoes laparotomy for pheochromocytoma.
D000310:Adrenal Gland Neoplasms / Q000378:metabolism / Q000473:pathology*; D002276:Carcinoid Tumor / Q000378:metabolism / Q000473:pathology*; D002395:Catecholamines / Q000378:metabolism; D004379:Duodenal Neoplasms / Q000378:metabolism / Q000473:pathology*; D005260:Female; D006651:Histocytochemistry; D006801:Humans; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000378:metabolism / Q000473:pathology*; D009456:Neurofibromatosis 1 / Q000378:metabolism / Q000473:pathology*; D010673:Pheochromocytoma / Q000378:metabolism / Q000473:pathology*; D013004:Somatostatin / Q000378:metabolism*; D014660:Vasoactive Intestinal Peptide / Q000378:metabolism
D002395:Catecholamines; D014660:Vasoactive Intestinal Peptide; D013004:Somatostatin