Asynchronous bilateral pulmonary pathology: left-sided congenital pulmonary airway malformation with contralateral malignant pleuropulmonary blastoma in an adolescent.
Ranganath T Ganga, Praveen Kumar Dubey, Mudalsha Ravina et al.
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Case in brief
An adolescent boy with a history of left-sided congenital pulmonary airway malformation (CPAM) presented with dry cough, right-sided chest pain, and breathlessness. Imaging revealed right-sided pleural effusion, pulmonary nodules, and skeletal metastases. A thoracoscopic biopsy confirmed pleuropulmonary blastoma (PPB) type III. Despite aggressive chemotherapy, the disease progressed rapidly, leading to a fatal outcome.
What made this case unusual
The case illustrates the rare occurrence of contralateral malignant pleuropulmonary blastoma in a patient with a history of congenital pulmonary airway malformation.
Diagnostic / clinical pearl
Clinicians should be aware of the potential for malignant transformation in patients with congenital pulmonary airway malformation, particularly in the presence of respiratory symptoms.
Why it matters
This case underscores the importance of early surgical intervention for CPAM and the need for vigilant screening in patients with a family history of CPAM or DICER1 mutations.