[Pleuropulmonary blastoma in children: diagnosis and results of surgery and complex treatment].

This is a report on a retrospective study of experience had with complex treatment of pleuropulmonary blastoma in ten children aged 2-11 years, covering a 27-year period. In all patients diagnosis is made on the ground of clinical symptomatology, imaging methods and histological findings. All patients undergo surgery, as follows: tumor extirpation (n = 3), lobectomy (n = 2) and pneumonectomy (n = 5). Three deaths occur in the early postoperative period (30%). In the remainder postoperative chemotherapy is conducted. Histologically the solid variant (type 3 according to Dehner's classification) is predominant. There are three recurrences (42.8%) among the cases with simple tumor extirpation (2) and lobectomy (1), followed by successful pneumonectomy (two patients) and atypical pulmonary resection (one patient). At long-term follow-up, five patients (50%) are still alive for periods ranging from 1 to 11 years postoperatively. Pleuropulmonary blastoma is a surgical rarity in children. The most important factors for long-term survivorship are both radical surgery, and adequate chemotherapy.