Atypical presentation of type 2 Pleuropulmonary Blastoma as a second cancer in a 5-year-old boy: A case report

Abstract

ABSTRACT Pleuropulmonary Blastoma (PPB) presents unique challenges due to its rarity, diverse clinical behaviors, and association with germline DICER1 mutations, emphasizing the need for advanced diagnostics and multidisciplinary management. This case describes the atypical presentation of type 2 PPB, the early development of a second cancer, and the need for vigilance to ensure optimal patient outcomes. We report a 5-year-old boy with type 2 PPB, previously managed for Wilms’ tumor who developed bilateral coin lesions discovered in the lungs during the follow-up, requiring surgical resection with histological confirmation of type 2 PPB. He was treated with IVADo regimen (ifosfamide, vincristine, actinomycin D, and doxorubicin) and is currently on follow-up. The early development and recognition of a rare second cancer with diverse clinical behaviors are dependent on focused surveillance post-treatment.