Pleuropulmonary blastoma.
We report a rare case of pleuropulmonary blastoma arising in a 3-year-old boy and involving the middle and upper lobes of the right lung. Radical resection of the mass was achieved by a bilobectomy. Microscopic examination of the histologic sections showed the typical findings of blastemal and mesenchymal areas with focal zones of rhabdomyoblastic and liposarcomatous differentiation. Monthly cycles of chemotherapy consisting of ifosfamide, vincristine and actinomycin D were given for 10 months after surgical resection. Our patient is presently alive and disease-free two years after diagnosis. As complete tumour ablation is essential to prevent local recurrence and allow any chance of survival, the favourable outcome of our patient is probably due to the radical resection of the neoplasm.
Granata
C
C
Department of Paediatric Surgery, Giannina Gaslini Institute, Genova, Italy. cgranata@panet.it
Gambini
C
C
Carlini
C
C
Repetto
P
P
Torre
M
M
Mazzola
C
C
Mattioli
G
G
Jasonni
V
V
D000970:Antineoplastic Agents / Q000627:therapeutic use; D017024:Chemotherapy, Adjuvant; D002675:Child, Preschool; D003609:Dactinomycin / Q000627:therapeutic use; D018572:Disease-Free Survival; D006801:Humans; D007069:Ifosfamide / Q000627:therapeutic use; D008175:Lung Neoplasms / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery*; D008297:Male; D010997:Pleural Neoplasms / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery*; D011013:Pneumonectomy; D018202:Pulmonary Blastoma / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery*; D014750:Vincristine / Q000627:therapeutic use
D000970:Antineoplastic Agents; D003609:Dactinomycin; D014750:Vincristine; D007069:Ifosfamide