Gastrointestinal autonomic nerve tumours--report of a case and review of literature

Abstract

Background: Gastrointestinal autonomic nerve tumours are uncommon stromal tumours of the intestinal tract. They can involve any part of the gastrointestinal system, but are very rarely seen in the rectum.

Case presentation: We report a unique case of rectal schwannoma with associated synchronous adenocarcinoma of the splenic flexure and adenoma of the descending colon. A 70-year-old patient was admitted with complaint of bleeding per rectum and investigations revealed the presence of a large submucosal rectal lesion in addition to the colonic pathologies. Following panproctocolectomy with permanent spout ileostomy, histopathology and immunohistochemistry confirmed the rectal lesion to be a schwannoma.

Conclusion: Literature review of the few reported cases has suggested radical surgical excision to be the best approach. Prognosis tends to be favourable after resection.

Figure 1

Imaging of the large bowel 1a) CT scan showing the large lesion in the posterior and left lateral wall of the rectum and 1b) MRI imaging: the distal rectal wall lesion (GANT) is shown to be intramural and free from adjoining structures.

Figure 2

Barium enema a) the apple core lesion at the splenic flexure and b) the extrinsic compression in the distal rectum caused by schwannoma.

Figure 3

Resected specimen showing malignant tumour at the splenic flexure, polyp in the descending colon and rectal schwannoma.

Figure 4

Photomicrograph showing a) cellular (Antoni A) and b) myxoid (Antoni B) areas (hematoxylin and eosin ×40).

Figure 5

Immunohistochemical staining of the rectal lesion with a) CD34, b) S100, c) Vimentin and d) NSE (original magnification ×40).