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仅对化疗有反应的转移至右心房的肝母细胞瘤。

Hepatoblastoma metastatic to the right atrium responding to chemotherapy alone.

作者信息

Kesik Vural, Yozgat Yilmaz, Sari Erkan, Kocaoğlu Murat, Kismet Erol, Koseoglu Vedat

机构信息

Department of Pediatric Oncology, Gulhane Military Medical Academy, School of Medicine, Etlik, Ankara, Turkey.

出版信息

Pediatr Hematol Oncol. 2009 Nov;26(8):583-8. doi: 10.3109/08880010903116678.

DOI:10.3109/08880010903116678
PMID:19954368
Abstract

An 18-month-old boy presented with abdominal pain and distension. On physical examination there was a 10 x 7-cm mass in the right upper abdominal quadrant. His alpha-fetoprotein level was 175,000 IU/mL. Abdominal magnetic resonance findings revealed hepatomegaly with multiple tumor masses involving nearly all the segments of the liver (PRETEXT IV). The tumor extended through the inferior vena cava and filled 2/3 of the right atrium. Echocardiography revealed normal cardiac function. Histopathologic findings after liver biopsy were consistent with hepatoblastoma. After 6 courses of chemotherapy including cisplatin and doxorubicin (PLADO, SIOPEL protocol), the cardiac tumor regressed completely. The patient's primary tumor was then fully resected; no cardiac surgery was performed. After surgery the AFP level was 4 IU/mL and echocardiography revealed normal cardiac function with no residual tumor. The patient has been in remission for 31 months postdiagnosis.

摘要

一名18个月大的男孩出现腹痛和腹胀症状。体格检查发现右上腹有一个10×7厘米的肿块。他的甲胎蛋白水平为175,000 IU/mL。腹部磁共振成像结果显示肝脏肿大,多个肿瘤肿块几乎累及肝脏的所有节段(PRETEXT IV)。肿瘤延伸至下腔静脉并占据右心房的2/3。超声心动图显示心脏功能正常。肝脏活检后的组织病理学结果与肝母细胞瘤相符。经过包括顺铂和阿霉素在内的6个疗程化疗(PLADO,SIOPEL方案)后,心脏肿瘤完全消退。随后对患者的原发性肿瘤进行了完整切除;未进行心脏手术。术后甲胎蛋白水平为4 IU/mL,超声心动图显示心脏功能正常,无残留肿瘤。该患者确诊后已缓解31个月。

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