A rare case of pleuropulmonary blastoma detected in fetus

Abstract

Pleuropulmonary blastoma (PPB) is among the rarest malignant tumors diagnosed in children. PPBs can be histopathologically classified into 3 types: cystic tumor (type I), mixed cystic and solid tumor (type II), and pure solid tumor (type III). We describe a case of type III PPB that was detected in a prenatal fetus, confirmed using histopathological methods. To the best of our knowledge, this is the first case describing a type III PPB detected in a fetus. Prenatal ultrasonography is an excellent tool for detecting pulmonary lesions during the diagnostic phase, and the possibility of PPB should be considered when solid tumors are detected. Early detection can allow for the performance of full resection, leading to a better prognosis for this cancerous tumor.

Keywords: Fetal pulmonary tumor; Pleuropulmonary blastoma; Prenatal diagnosis.

Fig. 1

Prenatal ultrasound: The axial plane reveals a heterogeneous tumor that shifts the mediastinum and causes pleural effusion (A). The parasagittal plane reveals an inverted diaphragm with ascites and skin edema (B). The tumor shows minimal vascularization (C). Umbilical Doppler showing an elevated vascular impedance (D). Open arrow: tumor, black arrowhead: heart, white arrow: pleural effusion, open white arrow: ascites, white arrowhead: skin edema.

Fig. 2

Post-term CT scanning: Mixed solid and cystic tumor located in the left thorax shows low attenuation and shifts the mediastinum toward the right. Open arrow: tumor, solid arrow: shifted heart, arrowhead: remaining left lung.

Fig. 3

Gross macroscopy of the tumor after resection.

Fig. 4

Immunohistochemistry: vimentin (+) (A), terminal deoxynucleotidyl transferase (−) (B).