Umeda Katsutsugu, Saida Satoshi, Yamaguchi Hideki, Okamoto Shinya, Okamoto Takeshi, Kato Itaru, Hiramatsu Hidefumi, Imai Tsuyoshi, Kodaira Takeshi, Heike Toshio, Adachi Souichi, Watanabe Ken-Ichiro
Department of Pediatrics, Graduate School of Medicine, Kyoto University, Kyoto 606-8507, Japan.
Department of Surgery, Graduate School of Medicine, Kyoto University, Kyoto 606-8507, Japan.
Oncol Lett. 2016 Jan;11(1):856-860. doi: 10.3892/ol.2015.3928. Epub 2015 Nov 17.
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.
尽管采用了积极的治疗方法，促结缔组织增生性小圆细胞肿瘤（DSRCT）患者的预后仍然极差。本研究报告了一名16岁患有转移性DSRCT的男性病例，对其实施了多模式治疗，包括使用频繁自体干细胞支持的强化化疗、对原发灶和转移灶进行大体切除以及全腹盆腔调强放射治疗。这些治疗后，没有疾病活动的证据。然而，在初次诊断后1年零2个月时，发现了小脑和松果体病变以及左肱骨骨转移。伊立替康和替莫唑胺联合化疗最初对中枢神经系统（CNS）转移性病变有效；然而，在七个疗程的联合化疗后，患者因进行性CNS疾病死亡。需要进一步的研究来积累关于DSRCT中枢神经系统复发的信息。
