辛泽尔-吉迪恩综合征中的难治性骶尾部生殖细胞肿瘤。
Refractory sacrococcygeal germ cell tumor in Schinzel-Giedion syndrome.
作者信息
Kishimoto Kenji, Kobayashi Ryoji, Yonemaru Nozomi, Yamamoto Hiroshi, Tsujioka Takao, Sano Hirozumi, Suzuki Daisuke, Yasuda Kazue, Suzuki Masahiko, Ando Akiko, Tonoki Hidefumi, Iizuka Susumu, Uetake Kimiaki, Kobayashi Kunihiko
机构信息
*Department of Pediatrics, Sapporo Hokuyu Hospital Departments of †Pediatrics ‡Pediatric Surgery, Tenshi Hospital, Sapporo §Department of Pediatrics, Obihiro-Kosei General Hospital, Obihiro, Japan.
出版信息
J Pediatr Hematol Oncol. 2015 May;37(4):e238-41. doi: 10.1097/MPH.0000000000000236.
We describe a boy with Schinzel-Giedion syndrome who developed refractory sacrococcygeal germ cell tumor with elements of embryonal carcinoma and immature teratoma. He developed local recurrence soon after tumor resection. The tumor was highly resistant to platinum-based combination chemotherapy, local irradiation, and salvage chemotherapy. Frequent infections resulted in a delay in treatment, although apparent fragility had not been observed clinically. He died from tumor progression at 32 months of age. Intensification of chemotherapy does not seem to be feasible for tumors in patients with Schinzel-Giedion syndrome.
我们描述了一名患有申泽尔-吉迪恩综合征的男孩，他患上了具有胚胎癌和未成熟畸胎瘤成分的难治性骶尾部生殖细胞肿瘤。肿瘤切除后不久便出现局部复发。该肿瘤对铂类联合化疗、局部放疗及挽救性化疗均具有高度耐药性。尽管临床上未观察到明显的脆弱性，但频繁感染导致治疗延误。他在32个月大时因肿瘤进展而死亡。对于申泽尔-吉迪恩综合征患者的肿瘤，强化化疗似乎并不可行。
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