Pleuropulmonary blastoma: Cystic tumour misdiagnosed as an emphysematous bulla

Pleuropulmonary blastoma is a dysontogenetic neoplasm of childhood, histologically characterized by a primitive variably mixed blastematous and sarcomatous appearance. It presents a pattern of rapid growth and is associated with a poor prognosis. We report the case of a 13-month-old girl, consulting...

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Bibliographic Details
Published in:Respiratory medicine CME 2010, Vol.3 (2), p.120-122
Main Authors: Chadli-Debbiche, A., Ben Brahim, E., Jouini, R., Labbene, N., Sayed, S., Ben Ayed, M., Kaâbar, N.
Format: Article
Language:English
Subjects:
Childhood
Cystic pulmonary lesion
Dysontogenetic neoplasm
Histology
Pleuropulmonary blastoma
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Summary:Pleuropulmonary blastoma is a dysontogenetic neoplasm of childhood, histologically characterized by a primitive variably mixed blastematous and sarcomatous appearance. It presents a pattern of rapid growth and is associated with a poor prognosis. We report the case of a 13-month-old girl, consulting for wheezing, dyspnea and loss of appetite. Chest X-ray revealed a mediastinal deviation with a clear appearance of the right lung. Computed tomography showed a voluminous emphysematous bulla of the right lung and atelectasis of the lower lobe. The final diagnosis was made on the basis of histological features of a cystic tumour, showing blastematous elements associated with sarcomatous compound confirming the diagnosis of pleuropulmonary blastoma type I. Subsequent chemotherapy was performed. Four years after the operation, the child is well with no evidence of disease recurrence or metastasis.
ISSN:1755-0017
1755-0017
DOI:10.1016/j.rmedc.2009.02.007