An Autopsy Case of Periaortic Malignant Fibrous Histiocytoma with Multiple Metastases to the Small Intestine

A 67-year-old woman was admitted to our hospital with severe abdominal pain. Emergency laparotomy was performed, and intestinal perforation was diagnosed. Wide resection of the distal ileum and cecum with ileostomy was performed. Resected specimens revealed three small perforated ulcers and two pedunculated tumors in the small intestine. The ulcers and tumors were diagnosed histopathologically and immunohistochemically as storiform-pleomorphic-type malignant fibrous histiocytoma (MFH) . The patient died 1 month after surgery due to liver and renal failure with aortic occlusion. At the primary site, an encapsulated mass (4.0×1.0×2.0 cm) was situated around the wall of the aorta at autopsy. The grayish white tumor tightly encircled half of the circumference at the level of the celiac axis. It is very unusual for the metastatic foci of MFH to be found before discovery of the primary focus. In addition gastrointestinal (GI) involvement from MFH has rarely been reported. We found 11 cases of metastasis of MFH to the small intestine in the English language literature. Our case is the first reported case of MFH causing perforation of the small intestine.