Sclerosing Epithelioid Fibrosarcoma of the Cecum: A Radiation-Associated Tumor in a Previously Unreported Site

Author:

Frattini Jared C.1,Sosa Julie Ann1,Carmack Susanne1,Robert Marie E.1

Affiliation:

1. From the Departments of Surgery (Drs Frattini and Sosa) and Pathology (Drs Carmack and Robert), Yale University School of Medicine, New Haven, Conn

Abstract

Abstract 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.

Publisher

Archives of Pathology and Laboratory Medicine

Subject

Medical Laboratory Technology,General Medicine,Pathology and Forensic Medicine

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