A Case Report of Pleuropulmonary Blastoma in Childhood

Park, Hye Rim; Sohn, Jin Hee; Hong, Ki Woo

Abstract

Classic pulmonary blastoma is a variant of carcinosarcoma which is seen almost exclusively in adults. By contrast, most cases of pulmonary blastoma in children have been described as having an exclusive mesenchymal composition, which was proposed as pleuropulmonary blastoma. Recently we experienced a case of pleuropulmonary blastoma, type 1. This 27-month-old male baby was transferred to our hospital due to the left tension pneumothorax. Chest CT revealed a subpleural pulmonary cystic lesion on the left upper lung and an open cystectomy was performed. Histologically the lesion was composed of variable-sized cystic structures lined with a single layer of respiratory-type epithelium. the underlying stroma was composed of sheets of small, round to oval, primitive tumor cells. Some of them had eccentric, eosinophilic cytoplasm, suggestive of rhabdomyoblastic differentiation. These rhabdomyoblastic cells were fuchsinophilic and positive with desmin and vimentin on immunohistochemistry.