Other Pathology

There is increased incidence of adenocarcinoma in:

— familial adenomatous polyposis coli, particularly periampullary related to duodenal adenomas. It is a significant cause of mortality in FAPC

— hereditary non-polyposis colon cancer

— Peutz-Jegher's polyposis (beware epithelial misplacement/pseudo-invasion); rare

— coeliac disease

— Meckel's diverticulum: rare—also carcinoid tumour, GIST, leiomyosarcoma

Coeliac disease/ulcerative jejunitis/gluten-induced intestinal or enteropathy-associated T cell lymphoma (EATCL): change in or lack of responsiveness to a gluten-free diet or presentation as an ulcerative/ perforated jejunitis or an abdominal mass in an older patient can indicate onset of EATCL. Stricture—carcinoid, metastases. Intussusception—carcinoid, lymphoma.

Multifocal—carcinoid, lymphoma, malignant melanoma, metastases. Meckel's diverticulum—carcinoid, adenocarcinoma, leiomyomatous tumours.

Immunophenotype

Small bowel adenocarcinoma is CAM5.2, AE1/AE3, CEA, CK20, CDX-2 positive. About 50% are also CK7 positive.

Prognosis

Small bowel adenocarcinoma is unusual, being 50 times less common than large bowel carcinoma. Seventy percent occur in the duodenum, particularly the periampullary region. Presentation is late due to the fluid content of the bowel. Many patients already have transmural spread and lymph node metastases and the majority subsequently die from their disease. Five-year survival rates are approximately 10-20% with the most important prognostic indicator being depth of spread or stage of disease. Surgical resection is the treatment of choice.

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