Radical resection of pleuropulmonary blastoma

Pleuropulmonary blastoma (PPB) is a rare intrathoracic neoplasm of early childhood that occurs in the lung or visceral pleura. We present a case of a 5-year-old boy with a cough, left-sided chest pain and progressive dyspnea on exertion. The patient had had an operation for a congenital lung cyst of the left lower lobe, six months prior to the present admission. A CT showed a heterogeneously enhancing mass lesion filling the left hemithorax. CT-guided fine needle aspiration cytology of the mass was inconclusive. En bloc resection of the mass along with left lower lobe, the involved ribs and left diaphragm was performed. The diaphragmatic defect was repaired using prolene mesh. Histopathology of the resected specimen showed solid and cystic areas consistent with type II PPB. The patient received adjuvent chemotherapy and was doing well 23 months after surgery. Radical resection of the tumor followed by adjuvent chemotherapy provides the only hope of salvaging cases of type II or type III PPB.