The association of neurofibromatosis, pheochromocytoma, and somatostatin-rich duodenal carcinoid tumor.

Abstract

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.

• Authors: M H Wheeler, I R Curley, E D Williams
• Journal: Surgery (Surgery) Vol. 100 Issue 6 Pg. 1163-9 (Dec 1986) ISSN: 0039-6060 [Print] United States
• PMID: 2878497 (Publication Type: Case Reports, Journal Article)

Chemical References

• Catecholamines
• Vasoactive Intestinal Peptide
• Somatostatin

Topics

• Adrenal Gland Neoplasms (metabolism, pathology)
• Carcinoid Tumor (metabolism, pathology)
• Catecholamines (metabolism)
• Duodenal Neoplasms (metabolism, pathology)
• Female
• Histocytochemistry
• Humans
• Middle Aged
• Neoplasms, Multiple Primary (metabolism, pathology)
• Neurofibromatosis 1 (metabolism, pathology)
• Pheochromocytoma (metabolism, pathology)
• Somatostatin (metabolism)
• Vasoactive Intestinal Peptide (metabolism)