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Somatostatin-Producing Neuroendocrine Tumor

MedGen UID:
52416
Concept ID:
C0037661
Neoplastic Process
Synonyms: Somatostatinoma; Somatostatinomas
Modes of inheritance:
Inheritance with unknown mechanism
MedGen UID:
831588
Concept ID:
CN227389
Intellectual Product
Source: Orphanet
Describes an inherited disorder with unknown mode of inheritance.
SNOMED CT: Somatostatin cell tumor (128642005); Somatostatinoma (128642005); Delta cell tumor (253006001); Somatostatinoma (253006001)
 
Orphanet: ORPHA97283

Definition

A rare, usually malignant neuroendocrine tumor arizing from delta cells. This neoplasm produces large amounts of somatostatin, which may result in a syndrome characterized by diarrhea, steatorrhea, weight loss, and gastric hyposecretion. Sixty percent are found in the pancreas and 40% in the duodenum or jejunum. The peak incidence occurs between 40 and 60 years of age; women are affected more than men by 2:1. [from NCI]

Recent clinical studies

Diagnosis

Guercioni G, Marmorale C, Siquini W, Fianchini M, Fianchini A, Landi E
Dig Dis Sci 2006 Oct;51(10):1767-72. doi: 10.1007/s10620-006-9222-1. PMID: 16967313

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