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采用蛤壳式入路切除的重达3500克的巨大纵隔脂肪肉瘤:一例报告并文献复习

A giant mediastinal liposarcoma weighing 3500g resected with clam shell approach, a case report with review of literature.

作者信息

Sugiura Yasoo, Hashizume Toshinori, Fujimoto Hiroyuki, Nemoto Etsuo

机构信息

Department of General Thoracic Surgery, National Hospital Organization, Kanagawa National Hospital, 666-1 Ochiai Hadano, Kanagawa 257-8585, Japan.

Department of General Thoracic Surgery, National Hospital Organization, Kanagawa National Hospital, 666-1 Ochiai Hadano, Kanagawa 257-8585, Japan.

出版信息

Int J Surg Case Rep. 2017;41:292-295. doi: 10.1016/j.ijscr.2017.10.055. Epub 2017 Nov 7.

Abstract

INTRODUCTION

Liposarcoma is rare in the mediastinum and is less than 1% of all mediastinal tumors. In the present report, we demonstrated our case and summarized the principal treatment of the mediastinal liposarcoma with literature review.

PRESENTATION OF CASE

A 50-year-old man presented at our hospital with complain of dyspnea. Chest radiography showed remarkable cardiomegaly. Computed tomography revealed an anterior mediastinal tumor from the level of the cephalic vein to the diaphragm of bilateral thoracic cavity with fat component. Using clam shell approach, complete en bloc resection of the tumor was performed. The weight of the tumor was 3500g. The pathological findings were 0that size of adipocyte and lipoblast were different, and the nuclei of atypical stromal cell were misshapen. Immune-histologic examination was negative for MDM2 and cyclin-dependent kinase 4. The diagnosis was liposarcoma, well-differentiated type. He could discharge 10days after surgery. Without adjuvant therapy, disease free survival for three years has passed.

DISCUSSION

From 1990-2016 in Japan, 60 cases of the mediastinal liposarcoma were reported. In analysis of the 61 cases including the present case, adjuvant therapy was performed in 14 cases, subsequently, and recurrence was recognized in 5 cases. Adjuvant therapy did not significantly suppress the recurrence.

CONCLUSION

Mediastinal liposarcoma weighing 3500g could be resected using calm shell approach, and no recurrence interval for 3 years has been achieved without adjuvant therapy. Complete resection is the only means to achieve the favorable outcome in mediastinal liposarcoma.

摘要

引言

脂肪肉瘤在纵隔中较为罕见,占所有纵隔肿瘤的比例不到1%。在本报告中,我们展示了我们的病例,并通过文献回顾总结了纵隔脂肪肉瘤的主要治疗方法。

病例介绍

一名50岁男性因呼吸困难到我院就诊。胸部X线检查显示明显的心脏扩大。计算机断层扫描显示前纵隔有一个肿瘤,从双侧胸腔头臂静脉水平至膈肌,含有脂肪成分。采用蚌式切口,完整整块切除肿瘤。肿瘤重量为3500克。病理结果显示脂肪细胞和成脂细胞大小不同,非典型间质细胞核畸形。免疫组织学检查MDM2和细胞周期蛋白依赖性激酶4均为阴性。诊断为高分化型脂肪肉瘤。术后10天他可以出院。未经辅助治疗,已无病生存三年。

讨论

1990年至2016年在日本,共报道了60例纵隔脂肪肉瘤。在分析包括本病例在内的61例病例时,有14例进行了辅助治疗,随后有5例复发。辅助治疗并未显著抑制复发。

结论

重达3500克的纵隔脂肪肉瘤可采用蚌式切口切除,未经辅助治疗已实现三年无复发期。完整切除是纵隔脂肪肉瘤取得良好预后的唯一方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/44f0/5683751/ae44f3449d2f/gr1.jpg

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