Type III pleuropulmonary blastoma in a 7-month-old female baby with impending respiratory failure: a case report

Abstract

IntroductionPleuropulmonary blastoma is a very rare, aggressive, embryonal pulmonary neoplasm which mostly affects children under the age of 5. According to the histopathological features, three subtypes of pleuropulmonary blastoma have been recognized: type I (purely cystic), type II (grossly visible cystic and solid elements) and type III (purely solid). Characteristics of type I and type II blastoma allow an earlier diagnosis compared with type III. Here we present a case report of an unusual presentation of type III pleuropulmonary blastoma.Case presentationWe describe the case of a 7-month-old female baby of Italian mother and Kurdish father who was diagnosed with type III pleuropulmonary blastoma, which entirely occupied her right hemithorax.ConclusionsThe reported case is an unusual presentation because type III pleuropulmonary blastoma typically occurs in older children. The complete re-expansion of her residual, previously totally compressed, right lung observed immediately after the resection of the lesion suggests an atypical rapid growth of this embryonal tumor in the late phase of gestation or after delivery. This case report suggests that, in addition to other childhood tumors, type III pleuropulmonary blastoma should be included in the differential diagnosis of solid nonhomogeneous thoracic large masses, compressing the mediastinal and chest wall structures in infants. This is an original case report of interest for several specialities such us pediatrics, radiology, surgery and oncology.

Clinical analysis of early death in children with pleuropulmonary blastoma in a single center in China

“…The ability to achieve radical resection and the absence of distant metastasis are the most important prognostic factors related to treatment outcome. 13,14 Gianni et al's, 15 from their clinical data on children with PPB over a six-year period in Europe, believed that the most important prognostic factor was the degree of tumor resection at diagnosis. In addition, the application of a doxorubicin-based regime was associated with better overall prognosis.…”

Type II Pleuropulmonary Blastoma in a 4 Month Old Infant with Negative Dicer1 Mutation on Next Generation Sequencing

“…The formation of PPB tumors can begin with multiple air-filled cysts that involve primitive mesenchymal cells (type I PPB). In subsequent stages, mesenchymal cells overgrow the delicate septa to produce a sarcomatous neoplasm that is either cystic and solid or exclusively solid ( 3 , 4 ). Type I PPB is characteristically multicystic.…”