somatostatinoma

Definition: Somatostatinoma is a very rare endocrine tumors. Less than 200 have been reported in the literature, 80 of which linked to NF1.

At the time of the diagnosis, 75 to 80 percent of the patients are metastatics, either in the liver or with nodes metastases.

Surgery is the only treatment.

Images

 https://twitter.com/mariaramir/status/727964314599100417

Localization

 pancreatic somatostatinoma
 ampullary somatostatinoma (Vater ampulla)

References

 Hamissa S, Rahmouni A, Coffin C, Wolkenstein P. CT detection of an ampullary somatostatinoma in a patient with von Recklinghausen’s disease. AJR Am J Roentgenol. 1999 Aug;173(2):503-4.

 Krejs GJ, Orci L, Conlon JM, Ravazzola M, Davis GR, Raskin P, et al.
Somatostatinoma syndrome. Biochemical, morphologic and clinical features. N Engl J Med. 1979 Aug 9;301(6):285-92.

 Bakkevold KE, Arnesjo B, Kambestad B. Carcinoma of the pancreas and papilla of Vater: presenting symptoms, signs, and diagnosis related to stage and tumour site. A prospective multicentre trial in 472 patients. Norwegian Pancreatic Cancer Trial. Scand J Gastroenterol. 1992 Apr;27(4):317-25.

 Ponchon T, Berger F, Chavaillon A, Bory R, Lambert R. Contribution of endoscopy to diagnosis and treatment of tumors of the ampulla of Vater. Cancer. 1989 Jul 1;64(1):161-7.

 Behranwala KA, Spalding D, Wotherspoon A, Fisher C, Thompson JN. Small bowel gastrointestinal stromal tumours and ampullary cancer in Type 1 neurofibromatosis. World J Surg Oncol. 2004;2:1.

 Ricci JL. Carcinoid of the ampulla of Vater. Local resection or pancreaticoduodenectomy. Cancer. 1993 Feb 1;71(3):686-90.