• Tatsuyoshi Ikeue, Issei Ohi, Susumu Noguchi, Akari Fukao, Satoshi Terashita, Sadao Horikawa, and Takakazu Sugita.
• Department of Respiratory Medicine, Japanese Red Cross Wakayama Medical Center, Japan.
• Intern. Med. 2016 Jan 1; 55 (17): 2463-7.

AbstractDesmoplastic small round cell tumor (DSRCT) is an aggressive mesenchymal tumor which primarily affects the abdomen. Even a multimodal approach rarely achieves durable remission and the optimal therapy for extended disease is unknown. We herein describe a rare case of DSRCT arising from the pleura in a 32-year-old man. Initial therapy, which included chemotherapy, surgery and radiotherapy, achieved a partial response for only two months. Although salvage chemotherapies had no effect, pazopanib treatment shrank the tumors and was well-tolerated on an outpatient basis. From the viewpoint of quality of life, pazopanib may therefore be a good therapeutic option for this aggressive disease.

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