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Pleuropulmonary blastoma in a child presenting with spontaneous pneumothorax
Jamaree Teeratakulpisarn1, Surapon Wiangnon, Jiraporn Srinakarin
1Departments of Pediatrics, Faculty of Medicine, Khon Kaen University, Khon Kaen 40002, Thailand.
Insights
A rare pediatric lung tumor, pleuropulmonary blastoma, was diagnosed in a young boy presenting with pneumothorax. Successful surgical removal and chemotherapy led to a favorable outcome with no signs of recurrence.
Area of Science:
- Pediatric Oncology
- Thoracic Surgery
- Pulmonology
Background:
- Pleuropulmonary blastoma (PPB) is a rare malignant tumor in children.
- Early diagnosis and treatment are crucial for improving patient outcomes.
Observation:
- A 27-month-old boy presented with respiratory distress and spontaneous pneumothorax.
- Imaging revealed a cystic and solid mass in the right lower lung with hydropneumothorax.
Findings:
- Histopathology confirmed a mixed cystic and solid type of pleuropulmonary blastoma.
- Complete surgical resection of the mass was achieved.
- Adjuvant chemotherapy included vincristine, cyclophosphamide, doxorubicin, and dactinomycin.
Implications:
- This case highlights the importance of considering PPB in pediatric patients with respiratory symptoms and pneumothorax.
- Multidisciplinary management involving surgery and chemotherapy can lead to successful treatment.
- Long-term surveillance is necessary to monitor for recurrence or metastasis.
Abstract:
The authors described a 27-month-old boy with the diagnosis of pleuropulmonary blastoma who presented with spontaneous pneumothorax. The child was admitted to our hospital with the chief complaint of respiratory distress for 8 months. Initial chest X-ray revealed tension pneumothorax on the right side. After chest tube insertion to the right side, a repeated chest X-ray showed minimal pleural effusion and a mass-like lesion at the right lower lung field. Computed tomography (CT) of the chest showed a cavity with intramural mass confined in the right lower lung accompanied with hydropneumothorax. The surgery revealed a cystic and solid mass occupying the right pleural space medially displacing the right lower lung. Total removal of the mass was performed, the histopathologic findings revealed a mixed cystic and solid type of pleuropulmonary blastoma which was composed of primitive blastema with multidirectional differentiation. Combination chemotherapy consisting of vincristine, cyclophosphamide, doxorubicin and dactinomycin was administered two weeks after surgery. The child has been well for almost 6 months since the surgery, without any signs of metastasis or recurrence.