Kukkady A, Upadhyay V, Pease P W, Chan Y F
Department of Surgery, Starship Hospital, Grafton, Auckland, New Zealand.
Pediatr Surg Int. 2000;16(8):595-8. doi: 10.1007/s003830000359.
Pleuropulmonary blastoma (PPB) is a rare malignant neoplasm affecting children. A retrospective review was carried out of patients diagnosed as having PPB at one institute over a period of 16 years. The presentation, diagnosis, treatment, and family history were studied. PPB usually presents with symptoms and signs of respiratory tract infection, and the diagnosis may be delayed. There are no distinguishing clinical features or imaging studies. The diagnosis is made on histologic examination of tumour material and is sometimes difficult to differentiate from benign cystic lung lesions. The treatment is primarily complete excision of the tumour, followed in some cases by intense chemotherapy. PPB is a strong predictor of the presence of tumours in close relatives. Four patients treated at our institute are discussed along with a review of the literature.
肺胸膜母细胞瘤（PPB）是一种罕见的儿童恶性肿瘤。对某机构16年间诊断为PPB的患者进行了回顾性研究。对其临床表现、诊断、治疗及家族史进行了分析。PPB通常表现为呼吸道感染的症状和体征，诊断可能会延迟。没有特异性的临床特征或影像学检查方法。诊断依靠肿瘤组织的组织学检查，有时难以与良性肺囊性病变相鉴别。治疗主要是肿瘤的完整切除，部分病例随后进行强化化疗。PPB是近亲患肿瘤的一个有力预测指标。本文讨论了在我们机构接受治疗的4例患者，并对相关文献进行了综述。
