Somatostatinoma

Somatostatinoma (SSoma) is an extremely rare pancreatic neuroendocrine tumor or duodenal endocrine tumor (see these terms) that originates either in the pancreas (50%) or the gastrointestinal tract (50%) and mainly presents with non-specific symptoms of abdominal pain, weight loss, jaundice and diarrhea but, in approximately 20% of pancreatic cases, leads to a somatostatin hypersecretion syndrome (somatostatinoma syndrome) characterized by diabetes mellitus, cholelithiasis, steatorrhea and hypochlorhydria.

Paraganglioma

A carotid body tumor (also called paraganglionoma or chemodectoma) is a tumor found in the upper neck at the branching of the carotid artery. They arise from the chemoreceptor organ (paraganglion) located in the adventitia of the carotid artery bifurcation.

Total: 8

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PMID (PMCID)
24276449	MIXED_SAMPLE	Infant
	Mosaicism in HIF2A-related polycythemia-paraganglioma syndrome.
	Buffet A, Smati S, Mansuy L, Menara M, Lebras M, Heymann MF, Simian C, Favier J, Murat A, Cariou B, Gimenez-Roqueplo AP. J Clin Endocrinol Metab. 2014;99(2):E369-73.
	Recently, HIF2A somatic mutations were found in several patients with polycythemia and paraganglioma, pheochromocytoma, or somatostatinoma, suggesting the occurrence of a de novo postzygotic HIF2A mutation that has not been demonstrated clearly.
24819565	MALE
	Polycythemia and paraganglioma with a novel somatic HIF2A mutation in a male.
	Toyoda H, Hirayama J, Sugimoto Y, Uchida K, Ohishi K, Hirayama M, Komada Y. Pediatrics. 2014;133(6):e1787-91.
	Recently, a new syndrome of paraganglioma, somatostatinoma, and polycythemia has been discovered (known as Pacak-Zhuang syndrome).
23509317	FEMALE	Adult
	New syndrome of paraganglioma and somatostatinoma associated with polycythemia.
	Pacak K, Jochmanova I, Prodanov T, Yang C, Merino MJ, Fojo T, Prchal JT, Tischler AS, Lechan RM, Zhuang Z. J Clin Oncol. 2013;31(13):1690-8.
	New syndrome of paraganglioma and somatostatinoma associated with polycythemia.
22931260 (3432945)	FEMALE	Adult
	Somatic HIF2A gain-of-function mutations in paraganglioma with polycythemia.
	Zhuang Z, Yang C, Lorenzo F, Merino M, Fojo T, Kebebew E, Popovic V, Stratakis CA, Prchal JT, Pacak K. N Engl J Med. 2012;367(10):922-30.
	Here we report two novel somatic gain-of-function mutations in the gene encoding hypoxia-inducible factor 2945 (HIF2A) in two patients, one presenting with paraganglioma and the other with paraganglioma and somatostatinoma, both of whom had polycythemia.
22931260 (3432945)	FEMALE	Adult
	Somatic HIF2A gain-of-function mutations in paraganglioma with polycythemia.
	Zhuang Z, Yang C, Lorenzo F, Merino M, Fojo T, Kebebew E, Popovic V, Stratakis CA, Prchal JT, Pacak K. N Engl J Med. 2012;367(10):922-30.
	Here we report two novel somatic gain-of-function mutations in the gene encoding hypoxia-inducible factor 2945 (HIF2A) in two patients, one presenting with paraganglioma and the other with paraganglioma and somatostatinoma, both of whom had polycythemia.
20300877	MIXED_SAMPLE	Adult
	Periampullary 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 Gastrointest Surg. 2010;14(6):1052-61.
	Tumor type was reported as somatostatinoma (40%), GIST (34%), adenocarcinoma (8%), carcinoid (6%), neurofibroma (5%), schwannoma (4%), or gangliocytic paraganglioma (3%).
15851380	OTHER	Middle Aged
	Duodenal somatostatinoma: clinical and immunohistochemical patterns--difficult differential diagnosis in regard to gangliocytic paraganglioma: report of a case.
	Bastian PJ, Eidt S, Koslowsky TC, Wulke AP, Siedek M. Eur J Med Res. 2005;10(3):135-8.
	Duodenal somatostatinoma: clinical and immunohistochemical patterns--difficult differential diagnosis in regard to gangliocytic paraganglioma: report of a case.
2894342	FEMALE	Middle Aged
	Synchronous duodenal neuroendocrine tumours in von Recklinghausen's disease--a case report of co-existing gangliocytic paraganglioma and somatostatin-rich glandular carcinoid.
	Stephens M, Williams GT, Jasani B, Williams ED. Histopathology. 1987;11(12):1331-40.
	This case strengthens the known association of glandular duodenal somatostatinoma with von Recklinghausen's disease and phaeochromocytoma and, in the light of a previous case report, suggests that von Recklinghausen's disease and gangliocytic paraganglioma may co-exist more commonly than expected.