Sclerosing epithelioid fibrosarcoma of the cecum: a radiation-associated tumor in a previously unreported site.
Data from the nuclear reactor explosion in Chernobyl and the atomic bomb detonations in Hiroshima and Nagasaki demonstrated an association between ionizing radiation and tumoriogenesis. There is a significant association between external beam radiation and radiation-induced sarcoma. Sclerosing epithelioid fibrosarcoma is a rare form of malignant fibrosarcoma that is low grade and indolent with distinct immunohistopathologic characteristics that usually occurs in the soft tissues of the extremities. A 62-year-old man from Kiev who aided in the cleanup at Chernobyl presented with crampy abdominal pain, nausea, and vomiting. His workup revealed a cecal mass, and the final pathology from his laparotomy confirmed sclerosing epithelioid fibrosarcoma with metastasis to the liver. In addition to a review of the literature, we report the first case of sclerosing epithelioid fibrosarcoma arising from the large bowel. Exposure to ionizing radiation from Chernobyl could have played a role in the development of his tumor.
Frattini
Jared C
JC
Department of Surgery, Yale University School of Medicine, 333 Cedar St, New Haven, CT 06520, USA.
Sosa
Julie Ann
JA
Carmack
Susanne
S
Robert
Marie E
ME
D002432:Cecum / Q000473:pathology* / Q000528:radiation effects*; D047852:Chernobyl Nuclear Accident; D005354:Fibrosarcoma / Q000473:pathology*; D006801:Humans; D007414:Intestinal Neoplasms / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009381:Neoplasms, Radiation-Induced / Q000473:pathology*