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Pleuropulmonary blastoma: a single-institution experience
Rajkumar Venkatramani1, Marcio H Malogolowkin, Larry Wang
1Division of Hematology/Oncology, Department of Pathology and Laboratory Medicine, Children's Hospital Los Angeles, Los Angeles, CA 90027, USA. rvenkatramani@chla.usc.edu
Insights
Pleuropulmonary blastoma (PPB), a rare childhood lung cancer, requires prompt diagnosis. Complete surgical removal offers the best chance for survival, with radiation therapy not always necessary for a cure.
Area of Science:
- Pediatric Oncology
- Thoracic Surgery
- Medical Malignancies
Background:
- Pleuropulmonary blastoma (PPB) is a rare intrathoracic mesenchymal malignancy exclusively affecting young children.
- This study reviews cases diagnosed between 1979 and 2009, categorizing them by type (I, II, III).
Purpose of the Study:
- To evaluate the efficacy of upfront complete tumor resection versus neoadjuvant chemotherapy and surgery for pediatric PPB.
- To determine prognostic factors influencing survival in patients with PPB.
Main Methods:
- Retrospective analysis of 12 pediatric patients diagnosed with PPB.
- Treatment modalities included upfront surgery, neoadjuvant chemotherapy followed by surgery, and radiation therapy.
- Chemotherapy regimens varied, with most patients receiving standard agents and some receiving additional agents.
Main Results:
- Upfront complete tumor resection was successful in 5 of 6 patients.
- The 5-year event-free survival was 33% ± 14%, and overall survival was 42% ± 14%.
- Gross total resection correlated with survival, while gross residual disease led to mortality. Radiation was not required for all survivors.
Conclusions:
- A high index of suspicion for PPB is crucial in pediatric patients with intrathoracic sarcoma.
- Gross total resection is the most critical factor for achieving a cure in PPB.
- Selected pediatric PPB patients may not require radiation therapy post-surgery.
Abstract:
Pleuropulmonary blastoma (PPB) is a rare primary intrathoracic mesenchymal malignancy that occurs exclusively in early childhood. Twelve patients were diagnosed with PPB (1 type I, 5 type II, and 6 type III) between 1979 and 2009 at our institution. Upfront complete tumor resection was successful in 5 of 6 patients. Six patients had biopsy followed by neoadjuvant chemotherapy, 2 had complete tumor resection, and 2 had microscopic residual disease after surgery. All patients received vincristine, dactinomycin, and cyclophosphamide chemotherapy. Eight received additional chemotherapy with doxorubicin, cisplatin, etoposide, or ifosfamide. Three patients received local irradiation. The 5-year event-free and overall survivals were 33% ± 14% and 42% ± 14%, respectively. Median time to progression was 8 months. Five of 9 patients with gross total resection survived, whereas all 3 with gross residual disease died. Three of 5 survivors did not receive radiation. A high index of suspicion for PPB must be maintained in all patients diagnosed with intrathoracic sarcoma in early childhood. Gross total resection is necessary for cure, and selected patients do not require radiation therapy.
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