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[Pleuro-pulmonary blastoma presenting as a chest wall deformity: a case report]
Josefina Sáez B1, Juan Carlos Pattillo S1, José Fernando Vuletin S1
1Facultad de Medicina, Pontificia Universidad Católica de Chile, Chile.
Insights
Pleuropulmonary blastoma (PPB), a rare childhood lung cancer, can present atypically. This case highlights PPB manifesting as a chest wall deformity, emphasizing early diagnosis for successful treatment.
Area of Science:
- Pediatric Oncology
- Thoracic Surgery
- Diagnostic Imaging
Background:
- Pleuropulmonary blastoma (PPB) is the most common pediatric lung malignancy, typically affecting children aged 1-4.
- PPB subtypes (I, II, III) correlate with survival and prognosis.
- Respiratory symptoms are common, but atypical presentations occur.
Observation:
- A one-year-old male presented with a chest wall deformity at ten months.
- Imaging revealed a giant right hemithorax cyst.
- Respiratory symptoms manifested only upon hospital admission.
Findings:
- Surgical excision of the right upper lobe was performed.
- Biopsy confirmed a type I pleuropulmonary blastoma.
- Complete surgical excision achieved successful treatment.
Implications:
- PPB diagnosis is crucial for differentiating from cystic lung malformations in children.
- Early recognition and complete surgical excision are vital for favorable outcomes.
- Routine thoracic imaging follow-up is recommended post-treatment.
Introduction:
Pleuropulmonary blastema (PPB) is the most common primary malignancy of the lungs in childhood. It occurs more frequently in children between one and four years of age, and respiratory symptoms are a common manifestation. Three types have been defined (type I, II and III), which are related to survival and prognosis.
Objective:
To report the first case of a patient with a PPB who presented with a chest wall deformity.
Case Report:
One year old male patient who had a chest wall deformity at ten months of age. Imaging revealed a giant cyst in the right hemithorax. He did not develop respiratory symptoms until hospital admission. A right upper lobectomy was perfor med and the biopsy confirmed a type I pleuropulmonary blastoma. He was considered successfully treated with complete surgical excision and routine follow-up with thoracic imaging is conducted.
Conclusions:
PPB is a very rare cancer that needs to be considered in the differential diagnosis of cystic lung diseases in children. The recognition of this lesion as a malignant tumour rather than a developmental cystic malformation is vital so the child can receive complete excision and appropriate follow-up care.
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