Radiation Therapy for Pleuropulmonary Blastoma: A Case Report and Review of Literature

Abstract

Objectives

This study aims to (1) describe a case of pleuropulmonary blastoma (PPB), and (2) discuss the role of radiation therapy in PPB management through a review of existing literature.

Methods

We described a case of PPB and conducted a comprehensive search through PubMed, Scopus, Cochrane, and the Grey Literature Report databases. Data on the role of radiation therapy, particularly its timing and outcomes, were reviewed and synthesized.

Results

A 4-year-old female with recurrent respiratory symptoms was diagnosed with PPB. A month after complete excision of the thoracic tumor and right upper lobectomy, recurrence was observed. Despite adjuvant chemotherapy, there was disease progression. The patient underwent subtotal re-excision followed by radiation therapy, receiving 4500 cGy to the grossly apparent tumor and 3600 cGy to the postoperative tumor bed in 180 cGy fractions concurrently with chemotherapy. Significant tumor regression was observed post-radiation therapy, with no significant adverse effects. The patient remains well three years after treatment.

Discussion

The role of upfront radiation therapy for PPB is unclear. Although registry data suggest it has no impact on overall survival, it is often considered when surgical resection is incomplete or when tumors demonstrate chemotherapy resistance. Additionally, radiation therapy may be utilized in treating PPB metastases, including those to the brain. The good long-term control in our patient suggests that its role requires further exploration.

Conclusion

Integration of radiation therapy in a multimodal treatment approach for recurrent PPB yielded favorable outcomes, suggesting it is a viable option warranting further investigation.