Pleuropulmonary blastoma is an uncommon primary malignant tumor of the lung. Surgery is the most important part of the treatment. Despite the use of chemotherapy with or without radiotherapy, the prognosis is poor. Here we report on the case of a 2.5 year-old boy presenting with pneumothorax who turned out to have a right hemithoracic mass. The patient underwent surgical resection of the mass. Pathologic diagnosis was type II pleuropulmonary blastoma. Postoperative thoracic CT showed nodular residual densities so the patient was given adjuvant combination chemotherapy with cisplatin and etoposide. He has been disease free for 12 months.
IndolfiP., CasaleF., CarliM., BisognoG., NinfoV., CecchettoG., BagnuloS., SantoroN., GiulianoM., Di TullioM.T.: Pleuropulmonary blastoma. Management and prognosis of 11 cases.Cancer, 89: 1396–1401, 2000.
2.
ManivelJ.C., PriestJ.R., WattersonJ., SteinerM., WoodsW.G., WickM.R., DehnerL.P.: Pleuropulmonary blastoma: the so-called pulmonary blastoma of childhood.Cancer, 62: 1516–1526, 1988.
3.
Di TullioM.T., IndolfiP., CasaleF., PettinatoG., MartoneA., MorgeraC.: Pleuropulmonary blastoma: Survival after intraocular recurrence.Med Pediatr Oncol, 33: 588–590, 1999.
4.
SchmaltzC., SauterS., OpitzO., HarmsD., KremensB., LohnerM., MetzK., BrandisM., NiemeyerC.: Pleuropulmonary blastoma: A case report and review of the literature.Med Pediatr Oncol, 25: 479–484, 1995.
