Abstract
Pneumoblastoma is a rare primary childhood tumor. We report the observation of an infant aged 2 years and 8 months who presented with dry cough and dyspnea. The physical examination found mixed pleural effusion syndrome on the right. The chest X-ray revealed a right pneumothorax. Biology has shown leukocytosis at 16,000/mm3. The CT scan revealed parenchymal air cystic lesions affecting the outer segment of the middle lobe mimicking a pulmonary malformation. Thoracic drainage brought back 100 ml of the fluid. Two months later, when a pyopneumothorax appeared, a medium lobectomy was performed. Pathological study specimen showed a high-grade type II pneumoblastoma The extension assessment identified a secondary hepatic location. Chemotherapy has been indicated. This observation illustrates the diagnosis challenge of pneumoblastoma in children.
Highlights
Pneumoblastoma (PB) is an extremely rare primary malignant tumor in children
We report an unusual observation after the consent of the parents of a hydropneumothorax revealing pneumoblastoma in a girl mimicking pulmonary malformation
A right middle lobectomy was performed because we suspected a cystic adenomatoid malformation of the right middle lobe. rough an open thoracotomy, we found a gelatinous component in the right chest originated from the middle right lobe extending to the pleura and diaphragm; a middle lobectomy was performed with resection of the gelatinous component (Figure 3). e specimen weighed 194 g and measured 16 cm
Summary
Pneumoblastoma (PB) is an extremely rare primary malignant tumor in children. It represents 0.25 to 0.5% of all lung tumors [1] with a very serious prognosis. There are three types of PB [2]: type I is a purely cystic, bullous lesion, type II combines solid and cystic plaques, and type III is exclusively solid. E clinical features of PB are usually nonspecific encompassing pneumothorax or respiratory distress [3] sometimes leading to delayed diagnosis. X-ray images are often confused with those of a congenital lung defect. We report an unusual observation after the consent of the parents of a hydropneumothorax revealing pneumoblastoma in a girl mimicking pulmonary malformation
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