Pulmonary blastoma: a case report and review of the literature

Abstract

BackgroundPulmonary blastomas are a rare aggressive neoplasm comprising 0.25-0.5% of all primary lung tumors and portend a poor prognosis. They display a biphasic histology with mesenchymal and epithelial components. Historically, the term pulmonary blastoma had included both pure fetal adenocarcinomas, pleuropulmonary blastomas as well as the classic biphasic blastomas. However recent World Health Organisation re-classifications separated well-differentiated fetal adenocarcinomas and pleuropulmonary blastomas from the biphasic tumours.Case presentationWe present a case of a systemically well 67-year-old Caucasian male who presented with haemoptysis. Investigations confirmed the presence of a large right-sided lung mass and biopsy identified non-small cell carcinoma. The resected tumour was markedly necrotic revealing a biphasic pattern. It was composed of malignant glandular tissue with sub-nuclear vacuoles, associated with a pleomorphic stromal malignant blastematous component, characteristic of classic biphasic pulmonary blastoma.ConclusionWe present a case of a classic biphasic pulmonary blastoma, a rare lung cancer occurring at an earlier age and portending to poorer prognosis than other more common lung cancers. Given the small number of cases and recent re-classification, interpreting the published epidemiology and clinical features of this disease is difficult. Many earlier reports may have included fetal adenocarcinomas (in particular high grade variant), which need to be considered when discussing treatment and prognosis with newly-diagnosed patients. Much could be gained from a central registry of individual experiences to improve our understanding of this rare lung cancer.

A retrospective analysis of the clinicopathological and molecular characteristics of pulmonary blastoma

“…Due to the low incidence rate and reclassification of the disease, it is difficult to interpret the epidemiology, clinical features, treatment, and prognosis of PB. A lot of earlier reports of PB have included fetal adenocarcinomas, so data are not in accordance to the new classification 10. Considering all these limits, even small number of cases or individual experiences of PB are important to improve our understanding of this rare lung cancer.…”

Endobronchial pulmonary blastoma – an unusual presentation of a rare lung malignancy and review of literature

“…WHO classifications (1999 and 2004) separated BPB from WDFA and PPB and are now grouped under sarcomatoid carcinomas [8]. Classical BPB typically occurs in adults with an average age of onset at 40 years with a slight male predisposition (2:1) [9]. There are no identifiable risk factors, but there appears to be a strong correlation with cigarette smoking [10].…”

A case of biphasic pulmonary blastoma presenting in a 55-year-old man

“…Histologically, WDFA contains both malignant glands and benign appearing mesenchymal tissue and PPB contains malignant glands of embryonal appearance and benign appearing epithelium. CBPB however contains glands and mesenchymal tissue that are both embryonal and malignant [6] .…”