Journal of Indian Association of Pediatric Surgeons · 2026
Pleuropulmonary Blastoma in Children: Three Distinct Cases with their Diagnostic and Therapeutic Pathways.
СтатьяНазвание и аннотация: Перевод в очереди
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Sahu Avilash Gourav, Manekar Aditya Arvind, Sahoo Subrat Kumar, Tripathy Bikasha Bihary, Mohanty Manoj Kumar, Sable Mukund Namdev, Sahoo Debasish, Mishra Pritinanda
Аннотация
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.
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.