Childhood Pulmonary Blastoma: A Pleuropulmonary Variant of the Adult-Type Pulmonary Blastoma

Abstract

Two fatal childhood cases of the rare pulmonary blastoma are reported. One was associated with a congenital cystic adenomatoid malformation. Both neoplasms extended to involve visceral pleura and were entirely composed of blastemal and mesenchymal elements without recognizable neoplastic epithelial components. The mesenchymal component in both instances consisted of malignant rhabdomyoblasts, undifferentiated mesenchyme, and differentiated, apparently benign, cartilage. Review of the literature suggests that these features may be specific for the childhood forms of pulmonary blastoma. It is further suggested that pulmonary blastoma, malignant mesenchymoma of the lung, and primary pulmonary rhabdomyosarcoma may have a common pathogenetic origin.

Gains of Chromosome 8 Are Confined to Mesenchymal Components in Pleuropulmonary Blastoma

“…Recent schemas define it as a subset of [12,13] or as an entity distinct from [3,6,14 -16] pulmonary blastoma. Defining pulmonary blastoma as a lung tumor that mimics developing lung [5], the term "pulmonary blastoma" may encompass the following: (1) classic biphasic blastoma, composed of malignant glands and malignant stroma [5,17]; (2) well-differentiated fetal adenocarcinoma, comprised only [18,19] or predominantly [17] of malignant epithelium; and (3) PPB, consisting of malignant stroma with or without accompanying benign epithelium [3,5].…”

“…Despite past variation in interpreting the definition of pulmonary blastoma [5,12,13,[15][16][17]20], PPB appears to be emerging as a distinct entity of childhood with characteristic clinicopathologic features that distinguish it from pulmonary blastomas of adulthood [3,6,21]. Children with PPB, usually less than 5 years of age, most often present with persistent cough and respiratory difficulty accompanied occasionally by fever, pneumothorax, or empyema [3,4,15].…”

Sarcomatoid Carcinomas of the Lung: A Clinicopathologic Review

“…[51][52][53][54][55][56][57] This view has been fostered in part by confusion of PB with pleuropulmonary blastoma (PPB), which is confined to prepubescent and adolescent patients. [58][59][60][61][62][63][64] PB is a biphasic neoplasm, manifesting a mixture of tubular epithelial cell profiles and compact groups of nondescript bluntly spindled cells with a blastemalike configuration 30 ' 58 ' 61 ( Fig 9) resembling those seen in Wilms' rumors.…”

Rhabdomyosarcoma in a child with massive pleural effusion: Cytological diagnosis from pleural fluid

“…In addition, two distinctive neoplastic processes should be considered in the differential diagnosis of thoracopulmonary lesions in children: peripheral neuroepithelioma (Askin's tumor), 7 and pulmonary blastoma. 8 The former bears a histological similarity to neuroblastoma, in which Homer-Wright rosettes may be present microscopically. In the absence of typical rosette formations, ancillary techniques such as immunohistochemical stains may be used to distinguish peripheral neuroepithelioma from other malignancies.…”