Survival with combined modality therapy after intracerebral recurrence of pleuropulmonary blastoma

Background. We present and discuss the successful treatment of pleuropulmonary blastoma metastatic to the brain using a multimodality regimen with surgery, high-dose chemotherapy and radiation therapy. Procedure. A 3-year-old boy referred to our institution with bilateral pulmonary cysts was diagnosed with pleuropulmonary blastoma (PPB). Initial treatment included surgery and multiagent chemotherapy with vincristine, dactinomycin, cyclophosphamide, cisplatin, and doxorubicin. One year after the completion of therapy, his PPB recurred as an intracerebral metastasis, and required further treatment with a multimodality salvage regimen. The child was successfully treated with a sub-total surgical resection, followed by high-dose cyclophosphamide, and radiation therapy. He is now disease-free 24 months later. Results. Intracerebral metastases of PPB have been a uniformly fatal complication of this tumor. Postsurgical chemotherapy and radiation therapy appear to have contributed to the prolonged survival and potential for cure in our patient. Conclusions. The use of this multimodality regimen may be warranted in other patients with recurrent PPB metastatic to the brain.