Case studySegmental xanthomatosis of the small intestine. A case report and review of the literature
Introduction
Xanthomatosis is characterized by the accumulation of lipid-laden, foamy macrophages resulting in the formation of plaques or nodules in tissues, particularly in the skin. Xanthomas occur more frequently in the skin and tendons but may occur in other places, for example, gastrointestinal tract, bladder, prostate, and arytenoepiglottic fold [1]. The stomach is the most frequent location of xanthomatosis in the gastrointestinal tract, and it rarely occurs in the small intestine [2], [3], [4], [5], [6], [7], colon [1], or simultaneously in both [4]. In many cases, these lesions are associated with familial or acquired conditions that result in hyperlipidemia or lymphoproliferative disorders. However, in other cases, no underlying predisposing condition can be identified. We report a case of segmental xanthomatosis of the small intestine in a patient with history of Ewing sarcoma of the right hip that was treated with radiation therapy. The literature on xanthomatosis of the small intestine was reviewed, and 6 previously published cases were found in the English literature since 1961 [2], [3], [4], [5], [6], [7].
Section snippets
Report of a case
A 22-year-old man with a history of Ewing sarcoma diagnosed at 4 years of age underwent radiation therapy for the right hip from ages 4 to 6 years. He did well from that time until 2 years ago, when he began complaining of abdominal pain and distension after eating, with progressing symptoms of intestinal obstruction. His bowel became nonfunctional, and he was on home total parenteral nutrition for 3 to 4 months. With a clinical impression of radiation enteritis, the patient was hospitalized
Macroscopic examination
The specimen was received in 10% buffered formalin and consisted of the distal small intestine and cecum. The small intestine segment was 55 cm in length and 4 cm in circumference. The small intestine mucosal surface was pale yellow and studded with small elevated protuberances ranging from 0.1 to 0.2 cm (Fig. 1). These lesions were diffusely distributed on the small bowel surface mucosa, involving 50 cm of the resected specimen, without involvement of the ileocecal valve, cecum, or resection
Discussion
Xanthomatous lesions have been described in different sites of the gastrointestinal (GI) tract (esophagus, stomach, small bowel, and colon). In the GI tract, the stomach is by far the most common site of occurrence. Reports of xanthomatous lesions of the small bowel are rare [2], [3], [4], [5], [6], [7]. These lesions have been described as multiple, small, and diffuse mucosal surface nodules in a segment of the bowel (Coletta and Sturgill [2] and the current case); as bandlike infiltration
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Histiocytic disorders of the gastrointestinal tract
2013, Human PathologyCitation Excerpt :Based on the limited number of reports, it seems that there is a predominance of males. The age ranges from roughly 9 to 70 years, with a mean age around 50 years [5,58-65]. Most cases were localized in the small intestine, followed by the cecum and ascending colon (Fig. 1).
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