Pleuro‐pulmonary blastoma: A case report and review of the literature

Abstract

Pleuro-pulmonary blastoma is a rare malignant lung tumor in children and distinct from ordinary pulmonary blastoma of adulthood. Combination chemotherapy plays an important role in the treatment of this tumor, but so far no attempt of high dose chemotherapy with subsequent bone marrow or blood stem cell transplantation has been published. The case is reported of a 2-year-old boy with pleuro-pulmonary blastoma who achieved partial remission with conventional chemotherapy and surgery. Subsequent administration of high dose melphalan, etoposide, and carboplatin, followed by autologous blood stem cell transplantation resulted in a 3-month disease-free interval but did not prevent eventual local recurrence, leading to death within a few weeks. Clinical presentation, pathology, management, and prognosis are discussed and the literature reviewed.

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. 65 Conversely, PPB lacks epithelial differentiation and may demonstrate evidence of divergent mesenchymal differentiation into myogenous or chondroosseous tissues 61 ( Fig 10).…”

Pulmonary Blastoma in a Child: Report of a Case

“…3,5-9 The etiology and predisposing factors of PB remain unknown; however, several case reports suggest that PB arises from cystic lung disease. 4,[9][10][11] The occurrence of PB appears to result in a heritable predisposition to childhood neoplastic diseases, including rhabdomyosarcoma, cystic nephroma, and synovial sarcoma in 25% of cases, 12 although our patient had no significant family history.…”

“…The diagnosis is usually indicated by a chest X-ray which shows a unilateral solid and cystic lesion, or sometimes a partial or complete opacification of the hemithorax with mediastinal deviation to the contralateral side and minimal effusion on the hemithorax side. 3,4,13,14 CT and MRI reveal extensive involvement of a solid and/or cystic tumor. In our patient, the clinical examination, chest Xray, CT, and MRI findings were similar to the features 3.…”

“…4 However, it is very difficult to make an early diagnosis of a malignant lung tumor. The diagnosis is usually indicated by a chest X-ray which shows a unilateral solid and cystic lesion, or sometimes a partial or complete opacification of the hemithorax with mediastinal deviation to the contralateral side and minimal effusion on the hemithorax side.…”

“…[1][2][3][4] PB of children was first described by Manivel et al in 1988, and differs from the classic adult-type PB in its variability of anatomic location, primitive embryonic-like blastoma and stroma, absence of a carcinomatous component, and propensity for sarcomatous differentiation. 3,4 Although radiotherapy and chemotherapy have been attempted without any reproducible success, the usual treatment is surgical resection. Despite aggressive treatment, PB is still associated with a poor prognosis.…”