Duodenal somatostatinoma

MedGen UID:: 234205
•Concept ID:: C1333320
•: Neoplastic Process

Synonyms:	duodenal delta cell somatostatin producing tumor; duodenal delta cell somatostatin producing tumour; Duodenal Somatostatin Producing Tumor; duodenal somatostatin producing tumor; duodenal somatostatin producing tumour; Duodenal Somatostatin-Producing NET; duodenal somatostatin-producing NET; Duodenal Somatostatin-Producing Neuroendocrine Tumor; duodenal somatostatin-producing neuroendocrine tumor; duodenal somatostatin-producing neuroendocrine tumour; Duodenal Somatostatinoma; duodenal somatostatinoma

Monarch Initiative:	MONDO:0004236

Definition

A somatostatin-producing neuroendocrine tumor that arises from the duodenum. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVDuodenal somatostatinoma

Neoplasm of the gastrointestinal tract
- Digestive system neuroendocrine neoplasm
  - Digestive system neuroendocrine tumor, grade 1/2
    - Intestinal Neuroendocrine Tumor
      - Small intestine neuroendocrine tumor, well differentiated, low or intermediate grade
        Duodenal neuroendocrine tumor, well differentiated, low or intermediate grade
        Duodenal somatostatinoma

Professional guidelines

PubMed

Duodenal neuroendocrine tumors: Classification, functional syndromes, diagnosis and medical treatment.

Hoffmann KM, Furukawa M, Jensen RT
Best Pract Res Clin Gastroenterol 2005 Oct;19(5):675-97. doi: 10.1016/j.bpg.2005.05.009. PMID: 16253893

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Recent clinical studies

Etiology

Functional Imaging Signature of Patients Presenting with Polycythemia/Paraganglioma Syndromes.

Janssen I, Chen CC, Zhuang Z, Millo CM, Wolf KI, Ling A, Lin FI, Adams KT, Herscovitch P, Feelders RA, Fojo AT, Taieb D, Kebebew E, Pacak K
J Nucl Med 2017 Aug;58(8):1236-1242. Epub 2017 Mar 23 doi: 10.2967/jnumed.116.187690. PMID: 28336782 Free PMC Article

Duodenal somatostatinoma: a case report and review of 31 cases with special reference to the relationship between tumor size and metastasis.

Tanaka S, Yamasaki S, Matsushita H, Ozawa Y, Kurosaki A, Takeuchi K, Hoshihara Y, Doi T, Watanabe G, Kawaminami K
Pathol Int 2000 Feb;50(2):146-52. doi: 10.1046/j.1440-1827.2000.01016.x. PMID: 10792774

Small duodenal somatostatinoma with high plasma somatostatin level.

Takizawa H, Kaneko H, Koike M, Hirose S, Asakura H
Am J Gastroenterol 1997 Jun;92(6):1063-4. PMID: 9177540

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Diagnosis

Duodenal somatostatinoma presenting as obstructive jaundice with the coexistence of a gastrointestinal stromal tumour in neurofibromatosis type 1: a case with review of the literature.

Thavaraputta S, Graham S, Rivas Mejia AM, Lado-Abeal J
BMJ Case Rep 2019 Jan 10;12(1) doi: 10.1136/bcr-2018-226702. PMID: 30635305 Free PMC Article

Functional Imaging Signature of Patients Presenting with Polycythemia/Paraganglioma Syndromes.

Duodenal somatostatinoma: a case report and review.

Kim JA, Choi WH, Kim CN, Moon YS, Chang SH, Lee HR
Korean J Intern Med 2011 Mar;26(1):103-7. Epub 2011 Mar 2 doi: 10.3904/kjim.2011.26.1.103. PMID: 21437171 Free PMC Article

Von Recklinghausen's neurofibromatosis associated with duodenal somatostatinoma. A case report and review of the literature.

Cappelli C, Agosti B, Braga M, Cumetti D, Gandossi E, Rizzoni D, Agabiti Rosei E
Minerva Endocrinol 2004 Mar;29(1):19-24. PMID: 15258554

Duodenal somatostatinoma: a case report and review of 31 cases with special reference to the relationship between tumor size and metastasis.

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Therapy

Duodenal somatostatinoma and erythrocytosis in a patient with von Hippel-Lindau disease type 2A.

Karasawa Y, Sakaguchi M, Minami S, Kitano K, Kawa S, Aoki Y, Itoh N, Sakurai A, Miyazaki M, Watanabe T, Akimoto M, Arakura N, Kiyosawa K
Intern Med 2001 Jan;40(1):38-43. doi: 10.2169/internalmedicine.40.38. PMID: 11201368

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