de Castro Cláudio Galvão, de Almeida Simone Geiger, Gregianin Lauro José, Loss Jiseh Fagundes, Rivero Luís Fernando, Schwartsmann Gilberto, Brunetto Algemir Lunardi
Pediatric Oncology Unit, Hospital de Clinicas de Porto Alegre, Rua Ramiro Barcelos 2350, 3rd Floor, Porto Alegre, RS, Brazil 90035-003.
J Pediatr Hematol Oncol. 2003 Jan;25(1):78-81. doi: 10.1097/00043426-200301000-00016.
Pleuropulmonary blastoma (PPB) is a rare and aggressive malignant tumor of the lung. Approximately 80 cases of PPB have been published, and in only three cases high-dose chemotherapy with autologous hematopoietic stem cell transplantation (HSCT) was applied. A 5-year-old girl presenting with cough, fever, and shortness of breath was referred to the authors in March 1999. A computed tomography scan of the chest showed a tumor mass in the left hemithorax. The lesion was biopsied and the histopathologic report suggested the diagnosis of PPB. The patient received chemotherapy comprising vincristine, actinomycin D, and cyclophosphamide with only a minor response, and treatment was switched to ifosfamide, carboplatin, and etoposide, which produced a partial response. Tumor resection was performed, but margins were positive for PPB. Due to the high risk of recurrence, the authors elected to administrate high-dose chemotherapy using melphalan, etoposide, and carboplatin, followed by autologous HSCT. The patient achieved complete hematologic recovery, and reimaging after HSCT showed no evidence of disease. She relapsed 4 months later and died about 9 months after the completion of high-dose therapy. The role of high-dose chemotherapy and autologous HSCT is likely to be limited in PPB.
肺胸膜母细胞瘤（PPB）是一种罕见的侵袭性肺恶性肿瘤。已发表的PPB病例约有80例，其中仅有3例应用了大剂量化疗联合自体造血干细胞移植（HSCT）。1999年3月，一名出现咳嗽、发热和呼吸急促症状的5岁女孩被转诊至作者处。胸部计算机断层扫描显示左半胸有一个肿瘤肿块。对该病变进行了活检，组织病理学报告提示诊断为PPB。患者接受了包含长春新碱、放线菌素D和环磷酰胺的化疗，但反应轻微，随后改用异环磷酰胺、卡铂和依托泊苷，产生了部分反应。进行了肿瘤切除术，但切缘PPB呈阳性。由于复发风险高，作者选择使用美法仑、依托泊苷和卡铂进行大剂量化疗，随后进行自体HSCT。患者实现了完全血液学恢复，HSCT后再次成像未发现疾病迹象。4个月后她复发，在大剂量治疗完成约9个月后死亡。大剂量化疗和自体HSCT在PPB中的作用可能有限。
