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Pleuropulmonary Blastoma in Children: Three Distinct Cases with their Diagnostic and Therapeutic Pathways
Avilash Gourav Sahu1, Aditya Arvind Manekar1, Subrat Kumar Sahoo1
1Department of Pediatric Surgery, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India.
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.
