Abstract
Pulmonary blastoma is a rare malignancy, accounting for less than 0.5% of primary lung tumors. It belongs to the group of pulmonary sarcomatoid carcinomas, and it is typically characterized by a biphasic pattern of an epithelial and a mesenchymal component. Only a few hundred cases have been reported worldwide. The aim of this study is to review and critically assess the literature regarding pulmonary blastoma. A narrative literature review of PubMed database from the inception of the database up to January 2021, limited to the English language, was conducted, using combinations of the following keywords: "pulmonary blastoma", "biphasic pulmonary blastoma", "sarcomatoid carcinoma". Pulmonary blastoma is composed of an epithelial and a mesenchymal malignant component. Regarding pathogenesis, the origin of the biphasic cell population remains elusive. Characteristic immunohistochemical stains are supportive of diagnosis.Clinically, the symptomatology is non-specific, while 40% of the cases are asymptomatic. It is diagnosed at a younger agecompared to other types of lung cancer, and it is often non-metastatic at diagnosis allowing for surgical treatment. Data on management and survival are scarce and mainly come from isolated cases. Advances on targeted therapy may provide novel treatment options. Given the rarity of the cases, multicenter collaboration is needed in order to establish therapeutic guidelines.
Highlights
Pulmonary blastoma is a rare malignancy, accounting for less than 0.5% of primary lung tumors
Pleuropulmonary blastoma predominantly presents in children and represents the most common primary pediatric pulmonary malignancy [3]
Classic biphasic pulmonary blastoma is typically characterized by a biphasic pattern consisting of a primitive mesenchymal stroma along with an epithelial component of fetal adenocarcinoma, while well-differentiated fetal adenocarcinoma is a monophasic tumor, presenting with immature adenocarcinoma as histologic characteristics [2]
Summary
Pulmonary blastoma is a rare malignancy, accounting for less than 0.5% of primary lung tumors. It belongs to the group of pulmonary sarcomatoid carcinomas, and it is typically characterized by a biphasic pattern of an epithelial and a mesenchymal component. The symptomatology is non-specific, while 40% of the cases are asymptomatic It is diagnosed at a younger age compared to other types of lung cancer, and it is often non-metastatic at diagnosis allowing for surgical treatment. Classic biphasic pulmonary blastoma is typically characterized by a biphasic pattern consisting of a primitive mesenchymal stroma along with an epithelial component of fetal adenocarcinoma, while well-differentiated fetal adenocarcinoma is a monophasic tumor, presenting with immature adenocarcinoma as histologic characteristics [2]. Pulmonary blastoma is separately categorized as a type of sarcomatoid carcinoma of the lung
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