Two Cases of Pleuropulmonary Blastomas in Children / 대한소아혈액종양학회지

ABSTRACT

Pleuropulmonary blastoma (PPB) is a rare primary malignant neoplasm with poor prognosis in children. PPB originates from the lung, the pleura, or the mediastinum. Histologically, it is characterized by a primitive, mixed blastematous, sarcomatous appearance and the absence of epithelial cell. Initial presenting symptoms are cough, fever and dyspnea. We experienced two cases of PPB (type I and type II). Complete surgical removal is always required for the treatment and chemotherapy and radiotherapy is needed as adjuvant therapy. We report two cases of pleuropulmonary blastoma treated with surgical removal, chemotherapy (vincristine, actinomycin D, cyclophosphamide) and radiotherapy.