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成人纵隔淋巴管肌瘤病:病例报告。

Mediastinal lymphangiomyoma in an adult: Report of a case.

机构信息

Department of Thoracic Surgery, Akita Red Cross Hospital, 222-1 Kamikitate, Akita, 010-1495, Japan.

出版信息

Surg Today. 2010 Apr;40(4):365-8. doi: 10.1007/s00595-009-4074-x. Epub 2010 Mar 26.

Abstract

Lymphangiomyoma is a rare benign hamartoma of lymphatic origin. A 70-year-old woman presented with a 6-month history of a cough and dyspnea on exertion. Computed tomography and magnetic resonance imaging of the chest showed a 10 x 9 x 4 cm multiloculated tumor in the anterior mediastinum. We resected the tumor successfully, preserving all vital structures, even though the tumor margin was partially indistinct. The tumor was diagnosed as lymphangiomyoma based on the pathological and immunohistological findings. Hamartomatous lymphangiomyoma is not expressed by markers for secondary lymphangiomyoma of lymphangioleiomyoma, including human-melanoma-black-45 and progesterone receptor. The terminology and relevant literature on lymphangiomyoma are reviewed following this case report.

摘要

淋巴管平滑肌瘤病是一种罕见的良性淋巴源错构瘤。一位 70 岁女性因咳嗽和活动后呼吸困难就诊,病史 6 个月。胸部计算机断层扫描和磁共振成像显示前纵隔有一个 10 x 9 x 4 cm 的多房性肿瘤。尽管肿瘤边缘部分不清晰,我们仍成功地切除了肿瘤,保留了所有重要结构。根据病理和免疫组化检查结果,诊断为淋巴管平滑肌瘤病。错构性淋巴管平滑肌瘤病不表达淋巴管平滑肌瘤病的继发性淋巴管平滑肌瘤的标志物,包括人黑色素瘤黑 45 和孕激素受体。在本病例报告之后,对淋巴管平滑肌瘤的术语和相关文献进行了回顾。

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