Pleuropulmonary blastoma

Pleuropulmonary blastoma (PPB) is a rare and aggressive intrathoracic malignancy that typically affects children. In general, PPB is a dysembryonic neoplasm of thoraco-pulmonary mesenchyme, which arises from the lung, pleural surface, or both. Herein we report a 1.5-year-old patient with PPB, where complete surgery was not possible due to involvement of vital structures (i.e., lungs, aorta, and pleura) by the tumor, as demonstrated by radiographic studies. Thereafter, neoadjuvant chemotherapy with six courses of ICE/VAC (= ifosfamide, carboplatin, etoposide/vincristine, actinomycin-D, cyclophosphamide) was implemented. Thereafter, We observed a significant 60% reduction in tumor burden. The patient survived for two years, manifesting marked improvement. Ultimately, our study confirmed that PPB is a rare childhood malignancy that appears to respond promisingly to neoadjuvant chemotherapy.