A pleuropulmonary blastoma type III in a 4-month-old infant: First case report in Syria

Abstract

Introduction: Pleuropulmonary blastoma is a rare, aggressive intrathoracic neoplasm of early childhood.

Case presentation: We report a case of a 4-month-old male baby who has presented with recurrent respiratory infections since birth. A surgical team was consulted due to abnormal opacification observed on a chest X-ray. An enhanced-contrast CT scan of the chest revealed a heterogenous, well-delineated mass measuring about 3,8 × 6 cm in the posterior mediastinum. A left posterolateral thoracotomy was performed. The mass was separated from the lung parenchyma, located behind the parietal pleura, and adherent to the chest wall and superior ribs. The lesion was totally removed. Histologically, the lesion was a pleuropulmonary blastoma type III. Currently, the patient is on a 6-month course of chemotherapy.

Clinical discussion: The aggressive, insidious behavior of PPB requires a high index of suspicion for diagnosis. The clinical manifestations and imaging modalities are atypical and nonspecific. However, PPB should be kept in mind when a huge solid or cystic mass is observed in the lung field on imaging.

Conclusion: Extrapulmonary pleuropulmonary blastoma is a very rare entity characterized by highly aggressive behavior and a poor prognosis. Early excision of thoracic cystic lesions in children is warranted regardless of the symptoms to avoid future mishaps.

Keywords: Case report; Congenital pulmonary airway malformation; Neoplasm; Pediatric; Pleuropulmonary blastoma.

Fig. 1

An X-ray of the chest showing a spherical opacity filling the upper 2/3 of the left chest.

Fig. 2

CT scan of the chest demonstrated a heterogeneous, well-defined mass measuring approximately 3,8 × 6 cm in the posterior mediastinum and compressing the left lung.

Fig. 3

Intraoperative images of the surgical excision of the tumor. A: The tumor was recognized immediately upon accessing the left thoracic cavity through 4th intercostal space. B: The tumor was extrapulmonary and not attached to lung parenchyma after ongoing dissection. C: Advanced stage of dissection with releasing most of the tumor mass outside the field.

Fig. 4

The surgical specimen of the neoplasm after removal.

Fig. 5

A chest X-ray revealed a complete expansion of the left lung with no abnormalities after one month of excision.

Fig. 6

Histopathological images of the surgical specimen of the PPB type III: A& B with low power magnification (H&E $\times$200), and C with high power magnification (H&E $\times$400) showing a heterogeneous solid tumor composed of mixture of primitive blastomatous and sarcomatous elements. D Ki67 immunohistochemical staining revealing high mitotic activity of the tumor ($\times$400).