Chen Wei, Yin Hongjian, Zhou Peng, Peng Muyun
Department of Cardiothoracic Surgery, People's Hospital of Zhangjiajie, Zhangjiajie, Hunan, China.
Department of Pathology, The Second Xiangya Hospital of Central South University, Changsha, 410011, Hunan, China.
J Cardiothorac Surg. 2024 Dec 19;19(1):647. doi: 10.1186/s13019-024-03184-x.
Müllerian cysts of the posterior mediastinum are rare, benign lesions typically discovered incidentally via routine medical exams.
We present a distinctive case of a 49-year-old asymptomatic woman, illustrating a rare Müllerian cyst located in the posterior mediastinum with serous papillary cystadenoma-like features, a novel finding in the medical literature. Identified during a routine health screening in December 2020, a 20 mm cystic lesion adjacent to the T4-5 vertebral body was detected through chest CT and MRI, initially suggesting a neurogenic tumor. Histopathological analysis revealed a Müllerian cyst characterized by an epithelium resembling serous papillary cystadenoma, with positive immunohistochemical markers for Paired Box 8 (PAX8), Estrogen Receptor (ER), and Progesterone Receptor (PR), indicative of Müllerian differentiation. The papillary growth pattern initially raised concerns about malignancy, yet the benign nature was confirmed by minimal cellular atypia, well-defined borders, absence of invasion, and a low proliferation index.
This case underscores the significance of detailed pathological analysis and immunohistochemical profiling in managing atypical Müllerian cysts, adding to the limited knowledge on Müllerian cysts. Future research should explore the mechanisms leading to Müllerian cysts with serous papillary cystadenoma-like features in the posterior mediastinum.
后纵隔苗勒管囊肿罕见，为良性病变，通常在常规医学检查中偶然发现。
我们报告一例独特的病例，患者为一名49岁无症状女性，其位于后纵隔的苗勒管囊肿具有浆液性乳头状囊腺瘤样特征，这在医学文献中是一项新发现。在2020年12月的一次常规健康筛查中，通过胸部CT和MRI检测到一个与T4 - 5椎体相邻的20毫米囊性病变，最初提示为神经源性肿瘤。组织病理学分析显示为苗勒管囊肿，其上皮类似于浆液性乳头状囊腺瘤，免疫组化标记物配对盒8（PAX8）、雌激素受体（ER）和孕激素受体（PR）呈阳性，表明为苗勒管分化。乳头状生长模式最初引发了对恶性肿瘤的担忧，但通过最小程度的细胞异型性、边界清晰、无浸润以及低增殖指数证实了其良性性质。
该病例强调了详细的病理分析和免疫组化分析在处理非典型苗勒管囊肿中的重要性，增加了关于苗勒管囊肿的有限知识。未来的研究应探索后纵隔出现具有浆液性乳头状囊腺瘤样特征的苗勒管囊肿的机制。
