title: Central nervous system recurrence of desmoplastic small round cell tumor following aggressive multimodal therapy: A case report.
abstract: Patients with desmoplastic small round cell tumors (DSRCTs) have an extremely poor outcome despite the use of aggressive therapy. The current study presents the case of 16-year-old male with metastatic DSRCT, in which multimodal therapy, including intensive chemotherapies using frequent autologous stem cell support, gross resection of primary and metastatic lesions, and whole abdominopelvic intensity-modulated radiation therapy, was administered. Subsequent to these treatments, there was no evidence of active disease. However, cerebellar and pineal body lesions, and bone metastasis to the left humerus were detected 1 year and 2 months after the initial diagnosis. Combination chemotherapy with irinotecan and temozolomide was initially effective against the central nervous system (CNS) metastatic lesions; however, the patient succumbed due to progressive CNS disease after seven courses of combination chemotherapy. Additional studies are required to accumulate information regarding CNS recurrence of DSRCT.
authors: Department of Pediatrics, Graduate School of Medicine, Kyoto University, Kyoto 606-8507, Japan.
authors: Katsutsugu
authors: K
authors: Umeda
authors: Department of Pediatrics, Graduate School of Medicine, Kyoto University, Kyoto 606-8507, Japan.
authors: Satoshi
authors: S
authors: Saida
authors: Department of Pediatrics, Graduate School of Medicine, Kyoto University, Kyoto 606-8507, Japan.
authors: Hideki
authors: H
authors: Yamaguchi
authors: Department of Surgery, Graduate School of Medicine, Kyoto University, Kyoto 606-8507, Japan.
authors: Shinya
authors: S
authors: Okamoto
authors: Department of Orthopedic Surgery, Graduate School of Medicine, Kyoto University, Kyoto 606-8507, Japan.
authors: Takeshi
authors: T
authors: Okamoto
authors: Department of Pediatrics, Graduate School of Medicine, Kyoto University, Kyoto 606-8507, Japan.
authors: Itaru
authors: I
authors: Kato
authors: Department of Pediatrics, Graduate School of Medicine, Kyoto University, Kyoto 606-8507, Japan.
authors: Hidefumi
authors: H
authors: Hiramatsu
authors: Department of Pediatrics, Otsu Red Cross Hospital, Otsu, Shiga 520-8511, Japan.
authors: Tsuyoshi
authors: T
authors: Imai
authors: Department of Radiation Oncology, Aichi Cancer Center Hospital, Nagoya, Aichi 464-8681, Japan.
authors: Takeshi
authors: T
authors: Kodaira
authors: Department of Pediatrics, Graduate School of Medicine, Kyoto University, Kyoto 606-8507, Japan.
authors: Toshio
authors: T
authors: Heike
authors: Human Health Science, Graduate School of Medicine, Kyoto University, Kyoto 606-8507, Japan.
authors: Souichi
authors: S
authors: Adachi
authors: Department of Pediatrics, Graduate School of Medicine, Kyoto University, Kyoto 606-8507, Japan.
authors: Ken-Ichiro
authors: KI
authors: Watanabe
keywords: autologous stem cell transplantation; central nervous system; desmoplastic small round cell tumor; multimodal therapy