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Revista chilena de pediatría

versión impresa ISSN 0370-4106

Resumen

SAEZ B., Josefina; PATTILLO S., Juan Carlos  y  VULETIN S., José Fernando. Pleuro-pulmonary blastoma presenting as a chest wall deformity: a case report. Rev. chil. pediatr. [online]. 2018, vol.89, n.2, pp.231-235. ISSN 0370-4106.  http://dx.doi.org/10.4067/S0370-41062018000200231.

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.

Palabras clave : Lung neoplasm; pleuropulmonary blastoma; chest wall deformity.

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