den Bakker Michael A, Vermeulen Marijn A, van de Ven Cornelis P, Ter Horst Simone A J, Kester Lennart, de Krijger Ronald R
Department of Pathology, Maasstad Hospital, Rotterdam, The Netherlands.
Department of Pathology, Erasmus MC, Rotterdam, The Netherlands.
Mediastinum. 2022 Dec 16;7:19. doi: 10.21037/med-22-32. eCollection 2023.
The most common thymic tumours, thymomas, are derived from thymic epithelium and are generally low-grade neoplasm. Frankly malignant tumours, thymic carcinomas are rarer still. Exceedingly rare thymic tumours contain a mesenchymal tissue component such as fibrous connective tissue and/or mature fat. Lipofibroadenoma (LFA) is a very rare mixed epithelial-mesenchymal thymic tumour, included in the category of thymic epithelial tumors. LFA in addition to a mature adipocytic component, contains variable epithelial and mesenchymal tissue components. Owing to the presence of an epithelial component in LFA, this entity, in contrast to thymolipoma, is included in the World Health Organization (WHO) category of thymic epithelial neoplasm. Currently only 12 LFA cases have been described. The 12 previously reported cases all behaved in a benign fashion, although four cases were associated with a conventional type of thymoma. We here present a new, 13th, case of LFA.
The LFA was discovered incidentally in a previously healthy 17-year-old male after investigations for suspected pneumonia. On imaging a mass was discovered in the anterior mediastinum which was subsequently surgically removed. The resected tumour was extensively investigated, including the first instance of full molecular analysis of this rare entity and all available literature on LFA was sourced to provide a comprehensive overview. The histology of this LFA was similar to previously described cases. No gene mutations or rearrangements were identified. The patient made an uneventful recovery and after 13 months of follow-up remained well.
An additional, 13th case of LFA is presented. Based on the available literature it appears that LFA may be considered a benign composite thymic tumour, although the combination of an additional conventional thymoma component may warrant closer follow-up.
最常见的胸腺肿瘤是胸腺瘤，起源于胸腺上皮，通常为低度恶性肿瘤。真正的恶性肿瘤，胸腺癌则更为罕见。极其罕见的胸腺肿瘤包含间叶组织成分，如纤维结缔组织和/或成熟脂肪。脂肪纤维腺瘤（LFA）是一种非常罕见的混合性上皮-间叶性胸腺肿瘤，属于胸腺上皮肿瘤类别。LFA除了有成熟的脂肪细胞成分外，还包含不同的上皮和间叶组织成分。由于LFA中存在上皮成分，与胸腺脂肪瘤不同，该实体被列入世界卫生组织（WHO）胸腺上皮性肿瘤类别。目前仅报道了12例LFA病例。先前报道的12例病例均表现为良性，尽管有4例与传统类型的胸腺瘤相关。我们在此报告第13例新的LFA病例。
该LFA是在一名17岁既往健康的男性因疑似肺炎进行检查时偶然发现的。影像学检查发现前纵隔有一肿块，随后进行了手术切除。对切除的肿瘤进行了广泛研究，包括对这一罕见实体的首次全分子分析，并查阅了所有关于LFA的现有文献以提供全面概述。该LFA的组织学与先前描述的病例相似。未发现基因突变或重排。患者恢复顺利，随访13个月后情况良好。
报告了第13例LFA病例。根据现有文献，LFA似乎可被视为一种良性复合性胸腺肿瘤，尽管合并额外的传统胸腺瘤成分可能需要密切随访。
