Pleuropulmonary Blastoma in Children: Three Distinct Cases with their Diagnostic and Therapeutic Pathways
Year: 2026
Type: article
Study design: Case Report
Abstract: Background: Pleuropulmonary blastoma (PPB) is a rare but aggressive pediatric tumor arising from the lung or pleura. Histologically, it consists of primitive blastomatous elements variably admixed with sarcomatous tissue. PPB is classified into four subtypes: type I (cystic), type Ir (regressing cystic), type II (mixed cystic and solid), and type III (predominantly solid). Imaging: PPB lacks characteristic imaging findings, making multimodality imaging essential for establishing a differential diagnosis, distinguishing the tumor from congenital airway malformations and benign cystic lesions, and identifying the specific subtype to guide management. Management: Treatment typically involves multimodal therapy, with surgery and chemotherapy forming the cornerstone. Early recognition and accurate subtype differentiation are critical for optimal outcomes. Case Summary: We present a series of three cases, each representing a different PPB subtype, all of which were successfully managed according to their specific pathological type.
Institution: All India Institute of Medical Sciences Bhubaneswar
Language: English
Subfield: Surgery
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W7119482815