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原发性腹膜后滑膜肉瘤的治疗:一例病例报告及文献综述

Management of primary retroperitoneal synovial sarcoma: A case report and review of literature.

作者信息

Mastoraki Aikaterini, Schizas Dimitrios, Papanikolaou Ioannis S, Bagias George, Machairas Nikolaos, Agrogiannis George, Liakakos Theodore, Arkadopoulos Nikolaos

机构信息

4 Department of Surgery, National and Kapodistrian University of Athens, Attikon University Hospital, Chaidari, Athens 12462, Greece.

1 Department of Surgery, National and Kapodistrian University of Athens, Laikon Hospital, Athens 11527, Greece.

出版信息

World J Gastrointest Surg. 2019 Jan 27;11(1):27-33. doi: 10.4240/wjgs.v11.i1.27.

Abstract

BACKGROUND

Synovial sarcoma (SS) is a rare type of soft tissue sarcoma that is usually developed from areas where synovial tissue exists, especially at the extremities. Nevertheless, several cases of retroperitoneal SS (RSS) have been described. We herein report a case of RSS presented in our institution.

CASE SUMMARY

A 69-year-old female patient was admitted with a large, palpable, firm mass in the right abdominal space SS. Computerized tomography scan depicted a concentric, sharply marinated retro-peritoneal lesion which was displacing the right kidney and the lower edge of the liver. Subsequently, the patient underwent surgical excision of the mass with additional right nephrectomy and resection of the right adrenal gland and a part of the diaphragm. The final histological diagnosis of the tumour was grade II monophasic RSS.

CONCLUSION

RSS is encountered in the biphasic type, the monophasic fibrous, and the monophasic epithelial category as well. Relevant clinical manifestations are not always documented at early stages. Therefore, the final diagnosis is posed after complete histological examination taking into consideration the results of immunochemistry and genetic analysis. Therapeutic approach happens often late when metastases at the lungs and the liver are apparent. Thus, 5-year survival rates remain low.

摘要

背景

滑膜肉瘤(SS)是一种罕见的软组织肉瘤,通常发生于存在滑膜组织的部位,尤其是四肢。然而,也有几例腹膜后滑膜肉瘤(RSS)的病例报道。我们在此报告我院收治的一例RSS病例。

病例摘要

一名69岁女性患者因右侧腹腔内可触及的巨大实性肿块入院。计算机断层扫描显示一个同心的、边界清晰的腹膜后病变,该病变使右肾和肝脏下缘移位。随后,患者接受了肿块的手术切除,并额外进行了右肾切除术、右肾上腺切除术及部分膈肌切除术。肿瘤的最终组织学诊断为II级单相性RSS。

结论

RSS可见于双相型、单相纤维型和单相上皮型。早期相关临床表现并不总是有记录。因此,最终诊断需在全面的组织学检查后,结合免疫化学和基因分析结果做出。当肺部和肝脏出现转移时,治疗往往已经延迟。因此,5年生存率仍然很低。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d513/6354068/c914eb67bad3/WJGS-11-27-g001.jpg

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