Pulmonary Blastoma: A Comprehensive Overview of A Rare Entity

Tsamis, Ioannis and Chachali, Stavroula-Porfyria and Gomatou, Georgia and Trontzas, Ioannis and Mitsogianni, Maria and Syrigos, Nikolaos and Vamvakaris, Ioannis and Kotteas, Elias (2021) Pulmonary Blastoma: A Comprehensive Overview of A Rare Entity. Advances in Respiratory Medicine, 89 (5). pp. 511-519. ISSN 2543-6031

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Abstract

Introduction: 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. Material and methods: 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”. Results: 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 Guideliness.

Item Type: Article
Subjects: Oalibrary Press > Medical Science
Depositing User: Managing Editor
Date Deposited: 25 Nov 2022 04:58
Last Modified: 16 Jul 2024 06:52
URI: http://asian.go4publish.com/id/eprint/401

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