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Presented by Ralph Hruban, M.D. and prepared by Jeremy Vincent M.D.
Case 2: The patient with neurofibromatosis underwent a Whipple resection for a mass centered in the duodenum.
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Question 1 of 1
1. Question
Week 508: Case 2
The patient with neurofibromatosis underwent a Whipple resection for a mass centered in the duodenum.images/1Alex/panc51.jpg
images/1Alex/panc52.jpg
images/1Alex/panc53.jpgCorrect
Answer: Somatostatinoma
Histology: This neoplasm was centered in the duodenum. The neoplastic cells form nests and trabeculae. The nuclei are uniform, and have a salt and pepper chromatin pattern. Numerous psamomma bodies are present.
Discussion: Patients with neurofibromatosis are at risk for a number of neoplasms. In the duodenum/ampulla, these include somatostatinomas, gastrointestinal stromal tumors, neurofibromas, and schwannomas. Somatostatinomas are extremely rare and usually do not cause an associated clinical syndrome. As seen in this case, they are often associated with psammoma bodies.
Reference(s):
-Periampuallary and duodenal neoplasms in neurofibromatosis type 1: two cases and an updated 20-year review of the literature yielding 76 cases. Relles D, Baek J, Witkiewicz A, Yeo CJ, J Gastrotintest Surg. 2010 Jun;14(6):1052-61. Epub 2010 Mar 19. Review. PMID: 20300877Incorrect
Answer: Somatostatinoma
Histology: This neoplasm was centered in the duodenum. The neoplastic cells form nests and trabeculae. The nuclei are uniform, and have a salt and pepper chromatin pattern. Numerous psamomma bodies are present.
Discussion: Patients with neurofibromatosis are at risk for a number of neoplasms. In the duodenum/ampulla, these include somatostatinomas, gastrointestinal stromal tumors, neurofibromas, and schwannomas. Somatostatinomas are extremely rare and usually do not cause an associated clinical syndrome. As seen in this case, they are often associated with psammoma bodies.
Reference(s):
-Periampuallary and duodenal neoplasms in neurofibromatosis type 1: two cases and an updated 20-year review of the literature yielding 76 cases. Relles D, Baek J, Witkiewicz A, Yeo CJ, J Gastrotintest Surg. 2010 Jun;14(6):1052-61. Epub 2010 Mar 19. Review. PMID: 20300877
