Malignant Somatostatinoma

MedGen UID:: 224755
•Concept ID:: C1266015
•: Neoplastic Process

Synonyms:	Delta Cell Carcinoma; Somatostatin cell tumor, malignant; Somatostatinoma, malignant
SNOMED CT:	Somatostatin cell tumor, malignant (128643000); Somatostatinoma, malignant (128643000)

Definition

A neuroendocrine tumor arising from delta cells which produce somatostatin. It displays vascular invasion and metastasizes to other anatomic sites. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVMalignant Somatostatinoma

Abnormality of the digestive system
- Digestive system neoplasm
  - Neoplasm of the gastrointestinal tract
    - Digestive system neuroendocrine neoplasm
      - Digestive system neuroendocrine tumor, grade 1/2
        Somatostatinoma
        Malignant Somatostatinoma
        Malignant Pancreatic Somatostatinoma

Professional guidelines

PubMed

Diabetes Mellitus Secondary to Endocrine Diseases: An Update of Diagnostic and Treatment Particularities.

Popoviciu MS, Paduraru L, Nutas RM, Ujoc AM, Yahya G, Metwally K, Cavalu S
Int J Mol Sci 2023 Aug 11;24(16) doi: 10.3390/ijms241612676. PMID: 37628857 Free PMC Article

Pancreatic neuroendocrine tumors: contemporary diagnosis and management.

Parbhu SK, Adler DG
Hosp Pract (1995) 2016 Aug;44(3):109-19. Epub 2016 Jul 18 doi: 10.1080/21548331.2016.1210474. PMID: 27404266

Pancreatic neuroendocrine tumors: clinical features, diagnosis and medical treatment: advances.

Ito T, Igarashi H, Jensen RT
Best Pract Res Clin Gastroenterol 2012 Dec;26(6):737-53. doi: 10.1016/j.bpg.2012.12.003. PMID: 23582916 Free PMC Article

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Recent clinical studies

Etiology

Serum levels of alpha-1-antitrypsin in pancreatic islet cell tumors.

Prinz RA, Sandberg L
J Med 1984;15(3):177-83. PMID: 6098624

See all (1)

Diagnosis

Malignant somatostatinoma presenting with diabetic ketoacidosis and inhibitory syndrome: pathophysiologic considerations.

Theodoraki A, Khoo B, Hamda A, Grillo F, Meyer T, Bouloux PM
Endocr Pract 2010 Sep-Oct;16(5):835-7. doi: 10.4158/EP10109.CR. PMID: 20497932

Diagnostic usefulness of FDG-PET for malignant somatostatinoma of the pancreas.

Suzuki H, Kuwano H, Masuda N, Hashimoto S, Kanoh K, Nomoto K, Shimura T, Katoh H
Hepatogastroenterology 2008 Jul-Aug;55(85):1242-5. PMID: 18795665

Ampullary somatostatinomas and jejunal gastrointestinal stromal tumor in a patient with Von Recklinghausen's disease.

Bettini R, Falconi M, Crippa S, Capelli P, Boninsegna L, Pederzoli P
World J Gastroenterol 2007 May 21;13(19):2761-3. doi: 10.3748/wjg.v13.i19.2761. PMID: 17569151 Free PMC Article

Somatostatin receptor scintigraphy of malignant somatostatinoma with indium-111-pentetreotide.

Schillaci O, Annibale B, Scopinaro F, delle Fave G, Colella AC
J Nucl Med 1997 Jun;38(6):886-7. PMID: 9189135

Brain metastasis from malignant pancreatic somatostatinoma. Case report.

Abe T, Oshida K, Matsumoto K, Iida M, Sanno N
J Neurosurg 1996 Oct;85(4):681-4. doi: 10.3171/jns.1996.85.4.0681. PMID: 8814175

See all (6)

Therapy

Carcinoid syndrome due to a malignant somatostatinoma.

Ozbakir O, Keleştimur F, Oztürk F, Sözüer E, Unal A, Patiroğlu TE, Güven K
Postgrad Med J 1995 Nov;71(841):695-8. doi: 10.1136/pgmj.71.841.695. PMID: 7494779 Free PMC Article

Malignant pancreatic somatostatinoma in a patient with dermatitis herpetiformis and coeliac disease.

Levi S, Bjarnason I, Swinson CM, Polak JM, Murray W, Levi AJ
Digestion 1988;39(1):1-6. doi: 10.1159/000199601. PMID: 2899527

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