Gastrointestinal stromal tumours of the small intestine, including duodenum (GIST)

Gastrointestinal stromal tumour more commonly occurs in the stomach. GIST is by far the most common stromal tumour in the duodenum2.

Epidemiology

Age range was from 10 to 88 years, median 56 years2.

Clinical features

The most common manifestation is with bleeding, usually chronic, causing anaemia, less often acute with melena2. The small intestines are the most common site for GIST in association with type I neurofibromatosis, in which context they are commonly multiple2.

Histopathology

The pathology resembles that at other sites. A feature not observed outside the duodenum is a haemangioma-like vascular proliferation, which is sometimes glomeruloid2.

Immunohistochemistry

Expression of SMA and S-100 appears to be more common in GISTs of the small bowel than in those of the stomach, with less common expression of CD34.

Differential diagnosis

• undifferentiated sarcoma - CD117 negative, these tumours are more pleomorphic

• smooth muscle tumours

• rare Schwannomas

Prognosis

Patients with recurrence, metastasis or died of disease:

Spread occurs within the abdominal cavity, to liver, bones or lungs, rarely ovaries, never to lymph nodes.

References

1 Chambonniere, M. L., Mosnier-Damet, M., Mosnier, J. F. Expression of microtubule-associated protein tau by gastrointestinal stromal tumors. Human Pathol 2001;32:1166-1173.

2 Miettinen, M., J. Kopczynski, et al. (2003). "Gastrointestinal stromal tumors, intramural leiomyomas, and leiomyosarcomas in the duodenum: a clinicopathologic, immunohistochemical, and molecular genetic study of 167 cases." Am J Surg Pathol 27(5): 625-41.

3 Hasegawa, T., Y. Matsuno, et al. (2002). "Gastrointestinal stromal tumor: consistent CD117 immunostaining for diagnosis, and prognostic classification based on tumor size and MIB-1 grade." Hum Pathol 33(6): 669-76.

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This page last revised 28.1.2006.

©SMUHT/PW Bishop