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JMD 2002, Vol. 4, No. 3
Copyright © 2002 American Society for Investigative Pathology & Association for Molecular Pathology

A Novel Type of EWS-CHOP Fusion Gene in Two Cases of Myxoid Liposarcoma

Taisuke Hosaka*{dagger}, Yasuaki Nakashima{ddagger}, Katsuyuki Kusuzaki§, Hiroaki Murata§, Tomitaka Nakayama{dagger}, Takeharu Nakamata*{dagger}, Tomoki Aoyama*{dagger}, Takeshi Okamoto*{dagger}, Kohichi Nishijo*{dagger}, Nobuhito Araki, Tadao Tsuboyama{dagger}||, Takashi Nakamura{dagger} and Junya Toguchida*{dagger}

From the Institute for Frontier Medical Sciences * Department of Orthopedic Surgery {dagger} and Pathology, {ddagger} Faculty of Medicine, and College of Medical Technology, || Kyoto University, Kyoto; the Department of Orthopedic Surgery, § Kyoto Prefectural University of Medicine, Kyoto; and the Department of Orthopedic Surgery, Osaka Medical Center for Cancer and Cardiovascular Diseases, Osaka, Japan

Fusion genes consisting of TLS/FUS and CHOP or EWS and CHOP are characteristic markers for myxoid/round cell liposarcomas (MLS/RCLS). Several different structures of the fusion genes were reported in the case of the TLS/FUS-CHOP form, whereas only one type of structure has so far been found for the EWS-CHOP form, which consisted of exons 1 to 7 of the EWS and exons 2 to 4 of the CHOP gene. Here we describe a novel type of EWS-CHOP fusion gene in two cases of MLS/RCLS, which were found in a consecutive analysis of 21 cases. This fusion gene consisted of exons 1 to 10 of the EWS and exons 2 to 4 of the CHOP gene. The two cases with this fusion gene shared several clinical features, such as a large tumor mass, rapid and invasive growth, and local recurrence within 12 months after surgical resection. Histopathological findings also showed common features characterized by the diffuse proliferation of small spindle cells with a primitive mesenchymal appearance. The association of these clinical and histopathological features suggests a distinct biological property for this rare type of fusion product.




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