巨大中纵隔平滑肌肉瘤1例。

Stéphane Collaud, Clemens Aigner
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引用次数: 1

摘要

原发性纵隔平滑肌肉瘤是一种极为罕见的软组织肿瘤,占所有原发性纵隔肉瘤的不到15%。中纵隔肿瘤非常罕见,健康人的患病率为0.1%。通常,纵隔平滑肌肉瘤起源于并累及纵隔结构如食道、心脏或大血管。我们在此报告一位70岁的女性病患,发生巨大的中纵隔平滑肌肉瘤,但未累及任何周围结构。主要相关症状为咳嗽和呼吸困难加重。影像学检查显示一个11厘米的巨大中纵隔肿瘤位于隆突下间隙并压迫食道。超声引导下经支气管针吸细胞病理学检查诊断为平滑肌肉瘤。通过第五肋间隙右后外侧开胸术完全切除肿瘤。术中肿瘤未累及周围结构。根据肉瘤肿瘤委员会的建议,患者接受了辅助放化疗(达卡巴嗪和阿霉素5个周期,随后60 Gy)。最后一次随访,术后10个月影像学未见复发迹象。这个罕见的病例证实了巨大的中纵隔平滑肌肉瘤可能不累及周围的纵隔结构,并且可以完全安全地切除而不需要切除邻近的结构。这里回顾了关于这个主题的相关文献。
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A case report of a giant middle mediastinal leiomyosarcoma.

Primary mediastinal leiomyosarcomas are extremely rare soft tissue tumors, accounting for less than 15% of all primary mediastinal sarcomas. Middle mediastinal tumors are very rare, with a prevalence of 0.1% in healthy individuals. Usually, mediastinal leiomyosarcoma originates and involves mediastinal structures such as oesophagus, heart or great vessels. Here we report the rare case of a giant middle mediastinal leiomyosarcoma without involvement of any surrounding structures in a 70 years old female. Main related symptoms were cough and increasing dyspnea. Imaging work-up showed an 11-cm giant middle mediastinal tumor located in the subcarinal space and compressing the oesophagus. Cytopathologic examination of endobronchial ultrasound-guided transbronchial needle aspiration diagnosed leiomyosarcoma. The tumor was completely removed through a right posterolateral thoracotomy in the fifth intercostal space. None of the surrounding structures were involved by the tumor intraoperatively. The patient underwent adjuvant chemoradiation as advised by the sarcoma tumor board (5 cycles of dacarbazine and doxorubicin followed by 60 Gy). At last follow-up, no evidence of recurrence was seen on imaging ten months after surgery. This rare case confirms that giant middle mediastinal leiomyosarcoma may not involve surrounding mediastinal structure and that resection can be completely and safely done without the need for resection of neighboring structures. The relevant literature on the subject is reviewed here.

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