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Hypertensive pneumothorax with cystic lesions: Pleuropulmonary blastoma in an infant
Rossella Lamberti1, Michele Ghezzi1, Francesca Riccaboni1
1Pediatric Department, "Vittore Buzzi" Children's Hospital, 20154, Milan, Italy.
Insights
Pleuropulmonary blastoma (PPB), a rare pediatric lung cancer, can mimic congenital cystic pulmonary malformation (CPAM). This case highlights the importance of re-evaluating initial diagnoses for accurate treatment of pediatric lung lesions.
Area of Science:
- Pediatric Oncology
- Thoracic Surgery
- Diagnostic Imaging
Background:
- Pleuropulmonary blastoma (PPB) is a rare pediatric malignancy.
- Congenital cystic pulmonary malformation (CPAM) is a key differential diagnosis.
- Early diagnosis and treatment are crucial for pediatric lung diseases.
Observation:
- A neonate presented with respiratory failure and bilateral lung cysts.
- Initial diagnosis was CPAM, but lesions progressed.
- Histological revision confirmed type I PPB at nine months of age.
Findings:
- The patient underwent multiple pulmonary resections and chemotherapy.
- Despite treatment, lesions increased in size, posing a risk of pneumothorax.
- Surgical intervention and continued monitoring were necessary.
Implications:
- This case underscores the diagnostic challenges in differentiating PPB from CPAM in infants.
- Accurate histological re-evaluation is vital for appropriate management of pediatric lung tumors.
- Long-term surveillance is essential for patients with PPB due to potential recurrence and progression.
Abstract:
Pleuropulmonary blastoma (PPB) is a rare primitive malignant lung cancer that occurs in pediatric age. Its main differential diagnosis is congenital cystic pulmonary malformation (CPAM). A 30-day-old infant with respiratory failure obtained a chest x-ray and a computed tomography scan (CT) which revealed hypertensive pneumothorax with multifocal bilateral cysts. After thoracic drainage, the patient underwent multiple thoracoscopic pulmonary resections. The first histological diagnosis was of type 2 CPAM. During the radiological follow-up, an increase in the number and dimension of the lesions was detected. Thus, a histological revision was performed, leading to the diagnosis of type I PPB, at nine months. The patient subsequently underwent chemotherapy. At the five-year follow-up appointment, chest magnetic resonance (MR) and CT scans showed a dimensional increase in size of the lesions, with the risk of recurrent pneumothorax. An upper right lobectomy and wedge resection of the residual cysts were performed. Control MR scans showed normalization of the lung parenchyma and the patient showed substantial clinical improvement.
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