Tumors of the small intestine with special reference to the rare benign disease of diffuse, nodular-polyploid lipomatosis

Tumors of the small intestine are very rare. Two-thirds of all neoplasms that occur in the small bowel are malignant. Only 1-3% of all malignancies of the bowel are detected in the small intestine. One-third of the tumors in small intestine are benign and lipomas belong to this category. They make up about 1.5-6.5% of all gastrointestinal neoplasms. Lipomatosis of the small intestine was first described by Hellstrom in 1906 but until now, fewer than 30 cases have been reported. The diffuse, nodular-polypoid lipomatosis of the small intestine is extremely rare. It signifies either the presence of multiple lipomas in one segment of the small intestine or of the entire small intestine. We present in this paper a case report of this rare disease, including its diagnosis, therapy and subsequent follow-up. In the discussion we review the literature for the period 1906-2005. (author)