Pleuropulmonary blastoma: case report in a child 8 years old

Abstract

Pleuropulmonary blastoma is a very rare primary fetal tumor of the lung and pleura in young children and is associated with a mutation in the DICER1 gene. Based on the histological picture, three types of this tumor are distinguished: cystic (type I), solid cystic (II), solid (type III). Treatment is surgical followed by polychemotherapy. The prognosis is unfavorable.
 The authors bring to the attention of colleagues a description of a clinical case of pleuropulmonary blastoma in an 8-year-old child, diagnosed against the background of an acute respiratory viral infection. He fell ill acutely with a rise in temperature and an unproductive cough. The condition was regarded as a course of an acute respiratory viral infection, symptomatic therapy was carried out. After 8 days from the onset of respiratory viral infection, there is a sharp deterioration in the condition in the form of shortness of breath, refusal to eat and drink, and weakness. The child is hospitalized in a Central District Hospital, differential diagnosis with pneumonia is carried out. According to the examination (plain radiograph and CT scan of the chest), a large volumetric formation with even contours is visualized in the right lung. The patient is transferred to a specialized hospital in St. Petersburg. After an MRI of the chest organs with contrast, taking a biopsy material and performing a histological examination, the patient was diagnosed with a tumor of the right lung pleuropulmonary blastoma type II.
 This clinical case of a rare malignant tumor in an 8-year-old child with congenital heart disease and cerebral palsy may demonstrate a severe variant of systemic connective tissue dysplasia and gene mutation.