Background: Tumors of the small intestine represent a small fraction of gastrointestinal tract neoplasms and might be missed if the referring physician and radiologist are not actively looking for a tumor. The choice of an optimal imaging protocol for detecting tumors in the jejunum and ileum is crucial. The differential diagnosis should include both benign (GIST, lipoma, hemangioma, neural tumors) and malignant (adenocarcinoma, carcinoid, lymphoma and metastases) tumors. Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor, and is never on the initial differential diagnosis of a small intestine tumor. In this article we present a case of jejunal AFH and a literature review of radiological findings in malignant and non-malignant tumors of the small intestine.
Clinical case: A 68-year-old male presented with a 1 month history of weight loss, lack of appetite, and pain in the right iliac and lumbar regions. Past medical history was significant for an adrenal tumor of unspecified origin and adrenalectomy in 2005. CT showed a non-homogenous infiltration located posterior to the ligament of Treitz and a polypoid exophytic mass. A biopsy was obtained during enteroscopy. Histological and immunohistochemical analysis confirmed an AFH. The adrenal tumor specimens were retrospectively re-analyzed and showed similar morphology and immunohistochemistry, therefore it was concluded that the neoplasm arose in the adrenal gland and metastasized to the jejunum.
Conclusions: In this report we presented to our knowledge the first case of an AFH of the adrenal gland which later metastasized to the jejunum. AFH has several characteristic findings on MR imaging: a double-rim sign, fluid-fluid levels, and marginal infiltrating strings of tumor tissue. To distinguish between benign and malignant tumors of the small intestine it is crucial to evaluate the number, location, vascularity, calcifications, growth and enhancement patterns, mesenteric and extra-intestinal involvement of the tumors.
Keywords: angiomatoid fibrous histiocytoma, small intenstine, small bowel, neoplasms, tumors, imaging, radiology