Oncology Unit, Third Department of Medicine, Sotiria General Hospital, National and Kapodistrian University of Athens, Messogion, Athens, Greece.
4th Department of Internal Medicine, National and Kapodistrian University of Athens, Medical School, Athens, Greece.
Adv Respir Med. 2021;89(5):511-519. doi: 10.5603/ARM.a2021.0085.
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.
肺母细胞瘤是一种罕见的恶性肿瘤，占原发性肺肿瘤的比例不到 0.5%。它属于肺肉瘤样癌群，其特征通常为上皮和间叶成分的双相模式。全世界仅报道了几百例病例。本研究旨在回顾和批判性评估有关肺母细胞瘤的文献。
对截至 2021 年 1 月的 PubMed 数据库进行了叙述性文献回顾，检索语言为英语，使用了以下关键词的组合：“肺母细胞瘤”、“双相肺母细胞瘤”、“肉瘤样癌”。
肺母细胞瘤由上皮和间叶恶性成分组成。关于发病机制，双相细胞群的起源仍不清楚。特征性免疫组织化学染色有助于诊断。临床上，症状是非特异性的，而 40%的病例无症状。与其他类型的肺癌相比，它的发病年龄更小，而且在诊断时通常没有转移，这使其可以进行手术治疗。关于管理和生存的数据稀缺，主要来自于孤立的病例。靶向治疗的进展可能为新的治疗方法提供选择。鉴于病例罕见，需要多中心合作以制定治疗指南。
