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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]

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: 37628857Free PMC Article
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: 23582916Free PMC Article
The current management of pancreatic neuroendocrine tumors.
Ellison TA, Edil BH
Adv Surg 2012;46:283-96. doi: 10.1016/j.yasu.2012.04.002. PMID: 22873046

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

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
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: 17569151Free 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
Serum levels of alpha-1-antitrypsin in pancreatic islet cell tumors.
Prinz RA, Sandberg L
J Med 1984;15(3):177-83. PMID: 6098624

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: 7494779Free 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

Prognosis

Atypical thymic carcinoid and malignant somatostatinoma in type I multiple endocrine neoplasia syndrome: case report.
Lin FC, Lin CM, Hsieh CC, Li WY, Wang LS
Am J Clin Oncol 2003 Jun;26(3):270-2. doi: 10.1097/01.COC.0000020584.56294.72. PMID: 12796599

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