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334 DERMATOFIBROSARCOMA PROTUBERANS AND SMALL BOWEL ADENOCARCINOMA: COINCIDENTAL OCCURRENCE OR GENETIC ASSOCIATION?
  1. A. Sumrall1,
  2. N. Soni1
  1. 1University of Mississippi Medical Center, Jackson, MS

Abstract

Evidence from the US National Cancer Institute indicates that the incidence of multiple primary neoplasms has doubled over the last 20 years. A suspected genetic predisposition of patients with dermatofibrosarcoma protuberans (DFSP) to second primary tumors has been previously reported. We report the first case of DFSP associated with adenocarcinoma of the small intestine. A 41-year-old African American man was admitted to the University of Mississippi Medical Center in November 2005 with melena and hematochezia. His medical history included diabetes mellitus, anemia, GERD, and DFSP with sacral and right inguinal involvement. The sacral mass was resected several times and was ultimately treated with radiation for 6 months. DFSP recurred in his right groin and was excised in March 2005. At presentation, he recalled an unintentional 50-pound weight loss over the past year and admitted to a 19-pack-year smoking history. He denied odynophagia, dysphagia, hematemesis, hemorrhoids, or change in bowel habits. Physical examination was remarkable for pedal edema bilaterally, splenomegaly, and mild hepatomegaly. His right inguinal region had mild scar tissue, and the sacrum showed a well-healed incision with postradiation changes. Laboratory examination showed a profound microcytic anemia (hematocrit 18.5). EGD revealed chronic gastritis and the presence of Helicobacter pylori. A small bowel follow-through study showed a luminal filling defect in the midjejunum. The CT scan of the abdomen and pelvis was initially reported as being unremarkable, but following the small bowel study, the CT scan was reread. A 7.3 × 6.0 cm soft tissue mass was seen in the lumen of the small bowel. In the operating room, he was found to have an intussusception of the small bowel surrounding the exophytic mass. Pathology determined the mass to be a well-differentiated adenocarcinoma, measuring 9 × 5 × 8 cm. Although these two primary cancers initially seemed unrelated, it was felt to be unusual that a young patient would have two rare cancers without a genetic predisposition. A literature review disclosed a possible genetic link between the two malignancies via tumor suppressor gene p53 and human MSH2. As our knowledge of cancer genetics improves and genetic testing becomes more available, relationships between different primary cancers will be better understood.

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