Mutafoğlu-Uysal Kamer, Kargi Aydanur, Sarialioğlu Faik, Olgun Nur, Kovanlikaya Arzu
Department of Pediatric Oncology, Institute of Oncology, Dokuz Eylül University, Faculty of Medicine, Izmir, Turkey.
Turk J Pediatr. 2002 Jul-Sep;44(3):244-7.
Malignant mesothelioma is a very rare tumor in childhood. Presently, treatment of this disease continues to be frustrating and prognosis remains poor. We here report a pediatric case of malignant pleural mesothelioma who gave a complete response to ICE-VAC chemotherapy regimen and achieved a long-term survival. An eight-year-old girl underwent exploratory thoracotomy and decortication because of a unilateral loculated and multicystic pleural effusion. Histopathological diagnosis was sarcomatoid pleural malignant mesothelioma. After decortication, chemotherapy with ICE (ifosfamide, carboplatin, etoposide) - VAC (vincristine, adriamycin, cyclophosphamide) combination was started. Six courses of chemotherapy resulted in complete clinical and radiological tumor response. She did not receive any further therapy and remains disease-free three years after the first remission. ICE-VAC chemotherapy combination resulted in a complete tumor response and a long-term disease-free survival for the presented case. The efficacy of this chemotherapy regimen in malignant mesothelioma needs to be documented in future trials.
恶性间皮瘤在儿童时期是一种非常罕见的肿瘤。目前,这种疾病的治疗仍然令人沮丧,预后仍然很差。我们在此报告一例儿童恶性胸膜间皮瘤病例,该病例对ICE-VAC化疗方案产生了完全缓解,并实现了长期生存。一名8岁女孩因单侧局限性多囊性胸腔积液接受了 exploratory thoracotomy(开胸探查术)和胸膜剥脱术。组织病理学诊断为肉瘤样胸膜恶性间皮瘤。胸膜剥脱术后,开始使用ICE(异环磷酰胺、卡铂、依托泊苷)-VAC(长春新碱、阿霉素、环磷酰胺)联合化疗。六个疗程的化疗导致临床和影像学上肿瘤完全缓解。她没有接受任何进一步的治疗,首次缓解三年后仍无疾病。ICE-VAC化疗联合方案使本病例肿瘤完全缓解并长期无病生存。这种化疗方案在恶性间皮瘤中的疗效需要在未来的试验中得到证实。