title: [Pleuropulmonary blastoma in children: diagnosis and results of surgery and complex treatment].
abstract: 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.
authors.forename: R
authors.initials: R
authors.lastname: Drebov
authors.forename: O
authors.initials: O
authors.lastname: Brankov
authors.forename: V
authors.initials: V
authors.lastname: Mikhailova
authors.forename: M
authors.initials: M
authors.lastname: Kamenova
authors.forename: I
authors.initials: I
authors.lastname: Khristozova
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000627:therapeutic use*; D003609:Dactinomycin / Q000627:therapeutic use*; D018572:Disease-Free Survival; D004317:Doxorubicin / Q000627:therapeutic use*; D005500:Follow-Up Studies; D006801:Humans; D008175:Lung Neoplasms / Q000175:diagnosis / Q000188:drug therapy / Q000601:surgery*; D009364:Neoplasm Recurrence, Local / Q000601:surgery; D011013:Pneumonectomy; D018202:Pulmonary Blastoma / Q000175:diagnosis / Q000188:drug therapy / Q000601:surgery*; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D014750:Vincristine / Q000627:therapeutic use*
chemical_list: D003609:Dactinomycin; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide