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一例实现长期生存的原发性纵隔平滑肌肉瘤病例。

A Case of Primary Mediastinal Leiomyosarcoma in Which Long-Term Survival Was Achieved.

作者信息

Iijima Yoshihito, Akiyama Hirohiko, Nakajima Yuki, Kinoshita Hiroyasu, Hirata Tomomi

机构信息

Division of Thoracic Surgery, Saitama Cancer Center, Saitama, Saitama Japan.

出版信息

Ann Thorac Cardiovasc Surg. 2020 Apr 20;26(2):95-99. doi: 10.5761/atcs.cr.18-00041. Epub 2018 Mar 30.

Abstract

INTRODUCTION

Primary leiomyosarcomas (LMS) of the mediastinum are extremely rare malignant mesenchymal tumors developing from soft tissues or great vessels. We present a case of a primary leiomyosarcoma of the middle mediastinum in which long-term survival was achieved.

CASE REPORT

A 77-year-old man presented to us for examination with an extrapleural sign in his upper mediastinum on chest X-ray. Computed tomography (CT) revealed a well-circumscribed mass in the middle mediastinum. Thoracoscopic resection of the mediastinal tumor and immunohistological findings, which were positive for smooth muscle actin (SMA), HHF-35, vimentin, and desmin confirmed primary leiomyosarcoma. It recurred twice with solid right pulmonary metastases, which were resected. He was followed-up for a total of 9 years and 6 months from the first surgery with no signs of recurrence after his last surgery.

CONCLUSION

Surgical resection of both the primary tumor and pulmonary metastases remains the mainstay of treatment of primary leiomyosarcomas.

摘要

引言

纵隔原发性平滑肌肉瘤(LMS)是极为罕见的起源于软组织或大血管的恶性间叶组织肿瘤。我们报告一例中纵隔原发性平滑肌肉瘤患者,该患者实现了长期生存。

病例报告

一名77岁男性因胸部X线显示上纵隔胸膜外征象前来我院检查。计算机断层扫描(CT)显示中纵隔有一个边界清晰的肿块。经胸腔镜切除纵隔肿瘤,免疫组织学检查结果显示平滑肌肌动蛋白(SMA)、HHF-35、波形蛋白和结蛋白呈阳性,确诊为原发性平滑肌肉瘤。该肿瘤复发两次,伴有实性右肺转移,均行手术切除。自首次手术起,他总共接受了9年6个月的随访,最后一次手术后未出现复发迹象。

结论

手术切除原发性肿瘤和肺转移灶仍然是原发性平滑肌肉瘤的主要治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a788/7184031/660b3ee66921/atcs-26-095-g001.jpg

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