Hereditary neuroendocrine tumor of small intestine

Orpha code: 456333OMIM code:

Definition

A rare inherited cancer-predisposing syndrome characterized by occurrence of multiple synchronous primary carcinoids of the small intestine. Clinical presentation is otherwise indistinguishable from sporadic carcinoids and includes abdominal pain, flushing, and diarrhea, often becoming manifest only after a long asymptomatic period. Most patients present with low grade tumors. Occurrence of pulmonary carcinoids has also been reported.

Disease data
Classification

Disease

Synonyms
Hereditary neuroendocrine tumor of small bowel
Hereditary neuroendocrine tumor of small bowel
ORPHA code
456333
OMIM code
-
ICD10 code
C17.9
ICD11 code
-

No additional description.

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