Background. Tumors of the small bowel are rare, accounting for about 3-6% o
f all gastrointestinal neoplasms. However, diagnosis and treatment are diff
icult and an ongoing challenge. Methods: We retrospectively reviewed the me
dical records of 54 patients with tumors of the small intestine over a peri
od of 10 years to elucidate important factors for diagnosis, therapy and pr
ognosis. Results: 42 patients had malignant (36 primary, 6 secondary) and 1
2 had benign tumors. Histologically, adenocarcinoma (33%), leiomyosarcoma (
17%) and carcinoid (17%) were the most frequent malignancies found. All 12
benign lesions were either leiomyomas or adenomas. Initial symptoms were no
nspecific: abdominal pain in 67% and 50%, anemia in 38% and 58% and weight
loss in 38% and 42% in patients with malignant and benign tumors, respectiv
ely. Upper gastrointestinal series, endoscopy, computed tomography scan and
selective angiography were the most useful diagnostic tools. Resectability
rate for malignant tumors was 98%; curative resection was achieved in 51%.
Survival for malignant tumors has been poor: median postoperative survival
was 26.9 months, the 1- and 5-year survival rates were 42.9% and 20.8%, re
spectively. Conclusions: These results document the need for an aggressive
diagnostic work-up in these rare tumors with non-specific symptoms, as earl
y diagnosis and radical operative therapy are important prognostic factors.