Asymptomatic lipofibroadenoma in a 17-year-old male: a case report and literature review of a rare entity.

Michael A den Bakker, Marijn A Vermeulen, Cornelis P van de Ven, Simone A J Ter Horst, Lennart Kester, Ronald R de Krijger
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引用次数: 3

Abstract

Background: 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.

Case description: 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.

Conclusions: 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.

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17岁男性无症状脂肪纤维腺瘤:一例罕见病例报告及文献复习。
背景:最常见的胸腺肿瘤,胸腺瘤,起源于胸腺上皮,通常是低级别肿瘤。坦率地说,恶性肿瘤,胸腺癌更罕见。极为罕见的胸腺肿瘤含有间充质组织成分,如纤维结缔组织和/或成熟脂肪。脂纤维腺瘤是一种非常罕见的胸腺上皮-间充质混合性肿瘤,属于胸腺上皮性肿瘤。LFA除了含有成熟的脂肪细胞成分外,还含有可变的上皮和间充质组织成分。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)的胸腺上皮肿瘤类别。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LFA病例。病例描述:LFA是在一名先前健康的17岁男性疑似肺炎调查后偶然发现的。影像学发现前纵隔有肿块,随后手术切除。我们对切除的肿瘤进行了广泛的研究,包括首次对这种罕见的肿瘤进行了完整的分子分析,并收集了所有关于LFA的文献,以提供一个全面的概述。该LFA的组织学与先前描述的病例相似。没有发现基因突变或重排。患者顺利康复,随访13个月后病情保持良好。结论:本文报告了第13例LFA病例。根据现有文献,LFA可能被认为是一种良性的复合胸腺肿瘤,尽管合并了一种额外的常规胸腺瘤成分可能需要更密切的随访。
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