Type Ii Pleuropulmonary Blastoma Responsive to Multimodal Therapy

Abstract

Pleuropulmonary blastoma is a rare and aggressive primary intrathoracic neoplasm of children. T'he prognosis is extremely poor with frequent metastasis to the brain and bone. A 4-year-old boy successfully treated with multimodal therapy despite unfavorable prognostic factors is presented. The authors support the use of aggressive treatment of pleuropulmonary blastoma with surgery, chemotherapy, and radiotherapy.

Rare Pediatric Tumors

“…96 Drug regimens parallel those used for other pediatric sarcomas and include ifosfamide/carboplatin/etoposide (ICE regimen), and ifosfamide/vincristine/dactinomycin (Actinomycin-D)/ doxorubicin (IVADo regimen). 111 Five-year survival rates are approximately 80% for patients with type I tumors and 45% for patients with types II and III. 97 RT may be indicated in unresectable tumors, although large treatment volumes and young patient age often hinder the use of this modality.…”

Thirteen-Year-Old Child With a Malignant Chest Tumor Clinicopathologic Conference

“…Total resection of PPB performed at any time of treatment appears to provide a better outcome, whereas extrapulmonary involvement at diagnosis worsens the prognosis [41]. Multiple reports have addressed the need for combination chemotherapy for PPB [41,[43][44][45][46][47][48][49][50], although there is no worldwide agreement on the optimal regimen [45]. Radiation therapy is recommended at some institutions for patients with type II or III lesions with known areas of residual disease [51].…”

Biphasic Tumors of the Lung

“…Other workers have suggested that pleuropulmonary blastoma type II may respond to multimodal therapy. 67 The aggressive nature of pleuropulmonary blastoma types II and III also has been demonstrated by documentation of metastatic disease to the central nervous system. 68,69 More recently, Priest and coworkers 70 stated that a rigorous surveillance schedule for pleuropulmonary blastoma type I might detect early recurrences and may be an alternative to adjuvant chemotherapy.…”