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复发性或转移性肝母细胞瘤患儿的治疗结果分析

Analysis of treatment outcome for children with recurrent or metastatic hepatoblastoma.

作者信息

Matsunaga Tadashi, Sasaki Fumiaki, Ohira Mutsuro, Hashizume Kohei, Hayashi Akira, Hayashi Yutaka, Mugishima Hideo, Ohnuma Naomi

机构信息

Department of Pediatric Surgery, Graduate School of Medicine, Chiba University, Chiba, Japan.

出版信息

Pediatr Surg Int. 2003 May;19(3):142-6. doi: 10.1007/s00383-002-0906-0. Epub 2003 May 24.

Abstract

For better total survival rate of children with hepatoblastoma, the therapeutic strategy for recurrent or metastatic hepatoblastoma should be improved. From 1991 to 1999, 134 cases of hepatoblastoma were treated by surgery and combination chemotherapy of cisplatin (CDDP) and THP-Adriamycin (THP-ADR) based on the JPLT-1 protocol. In 114 non-metastatic cases, 90 primary liver tumors were resected completely by partial hepatectomy, but 12 recurrences were observed in the liver (4 cases) and the lungs (8 cases). Distant metastases on the diagnosis were observed in 20 cases. The treatment outcome of these 12 recurrent and 20 metastatic tumors was analyzed. In four recurrent liver tumors, surgical resection was performed in all four cases, and all the patients were alive and well. In eight recurrent lung tumors, surgical resection was performed completely in six cases with unilateral lung disease, and five of the six patients were alive and well. In stage IV tumors, the survival rate of the patients having primary tumors within two hepatic sections was significantly higher than that of the patients having primary tumors over three hepatic sections. Active surgical intervention to lung metastases and a more intensive chemotherapy to facilitate complete resection of primary hepatic tumor could improve survival rate of children with refractory hepatoblastoma.

摘要

为提高肝母细胞瘤患儿的总生存率,应改进复发性或转移性肝母细胞瘤的治疗策略。1991年至1999年,根据JPLT - 1方案,134例肝母细胞瘤患儿接受了手术及顺铂(CDDP)和吡柔比星(THP - ADR)联合化疗。在114例非转移性病例中,90例原发性肝肿瘤通过肝部分切除术完全切除,但观察到12例复发,其中肝脏复发4例,肺复发8例。诊断时发现20例有远处转移。对这12例复发性肿瘤和20例转移性肿瘤的治疗结果进行了分析。在4例复发性肝肿瘤中,4例均进行了手术切除,所有患者均存活且状况良好。在8例复发性肺肿瘤中,6例单侧肺部病变患者进行了完全手术切除,6例患者中有5例存活且状况良好。在IV期肿瘤中,原发性肿瘤位于两个肝段以内的患者生存率明显高于原发性肿瘤超过三个肝段的患者。对肺转移进行积极的手术干预以及采用更强化的化疗以促进原发性肝肿瘤的完全切除,可提高难治性肝母细胞瘤患儿的生存率。

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