Carcinoid / Well Differentiated Neuroendocrine Neoplasm / Tumor of the Ileum, Distal Jejunum and Cecum
Clinical
- Five year survival
- No liver metastases at presentation 70%
- Liver metastases at presentation 35%
- Deaths due to disease continue to occur beyond 5 years
- Carcinoid syndrome is usually present only if the liver is involved by metastases
- 15% of patients also have a non-endocrine neoplasm, usually gastrointestinal adenocarcinoma
- Less frequently associated with MEN-1 than are gastric and duodenal endocrine neoplasms
- Patients with multiple jejuno-ileal carcinoids have a decreased survival
- Histopathologic features are predictive of behavior
- Benign
- Non-functioning
- Well differentiated
- ≤1 cm in greatest dimension
- Confined to mucosa and submucosa
- No involvement of muscularis propria
- No vascular invasion
- Uncertain malignant behavior
- As for benign, but with:
- >1 but ≤2 cm in greatest dimension and/or
- Vascular invasion
- Low grade malignant
- Non-functioning and well differentiated, with
- Invasion of muscularis propria or beyond and/or
- Metastasis and/or
- >2 cm in greatest dimension (not uniformly accepted)
- OR, functioning and well differentiated
