Christosova Iskra R, Avramova Boryana E, Drebov Rosen S, Shivachev Hristo I, Kamenova Margarita A, Bobev Dragan G, Brankov Ognyan G
Specialized Hospital for Pediatric Oncohematology, Sofia, Sofia, Bulgaria.
University Hospital for Emergency Medicine "Pirogov," Sofia, Clinic of Pediatric Surgery, Sofia, Bulgaria.
Pediatr Pulmonol. 2015 Jul;50(7):698-703. doi: 10.1002/ppul.23047. Epub 2014 Apr 1.
Pleuropulmonary blastoma (PPB) is a rare and potentially aggressive intrathoracic disembryonic neoplasm typically occurring in children less than 6 years of age. We assessed the relative incidence, clinical characteristics, treatment outcome, and the prognostic factors for long-term survival in patients with PPB treated at our institution over a 25-year period, and compared these data with reports in the literature. From 1985 to 2010, 11 children (4 males and 7 females), with a median age of 5.4 years (range, 1-12 years) were treated at our hospital. Here we described the main characteristics of these patients, the diagnostic methods, and treatment modalities used. During a median follow-up period of 80, 9 months, the overall survival (OS) and disease-free survival (DFS) rates were 54, 6% and 45, 5%, respectively. Two patients survived for more than 20 years. The main prognostic factors for long-term survival were the diseases type I and II and treatment with radical surgery. Our results show that in order to improve the prognosis of patients with PPB a timely in our opinion and accurate diagnosis needs to be established and treatment should be offered according to the disease type and extend of dissemination.
肺胚细胞瘤（PPB）是一种罕见且具有潜在侵袭性的胸腔内胚胎发育异常性肿瘤，通常发生于6岁以下儿童。我们评估了在我院接受治疗25年的PPB患者的相对发病率、临床特征、治疗结果及长期生存的预后因素，并将这些数据与文献报道进行比较。1985年至2010年，我院共治疗11例儿童患者（4例男性，7例女性），中位年龄5.4岁（范围1 - 12岁）。在此我们描述了这些患者的主要特征、诊断方法及所用治疗方式。在中位随访期80.9个月时，总生存率（OS）和无病生存率（DFS）分别为54.6%和45.5%。2例患者存活超过20年。长期生存的主要预后因素为疾病的I型和II型以及根治性手术治疗。我们的结果表明，为改善PPB患者的预后，我们认为需要及时、准确地做出诊断，并应根据疾病类型和播散范围进行治疗。
