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肝原发性单相滑膜肉瘤的右半肝切除术:病例报告及文献复习

Right hepatectomy for primary monophasic synovial sarcoma of the liver: case report and review of the literature.

作者信息

Fodiya Moctar Noufou, Lahnaoui Oumayma, Khmou Mouna, Majbar Mohammed Anass, Souadka Amine, Benkabbou Amine

机构信息

Department of Digestive Oncology Surgery, National Oncology Institute Sidi Mohamed Ben Abdellah, Ibn Sina University Hospital Center, Mohamed V University, Rabat, Morocco; Faculty of Health Sciences, Abdou Moumuni University, Niamey, Niger.

Department of Digestive Oncology Surgery, National Oncology Institute Sidi Mohamed Ben Abdellah, Ibn Sina University Hospital Center, Mohamed V University, Rabat, Morocco.

出版信息

Int J Surg Case Rep. 2025 Jun;131:111348. doi: 10.1016/j.ijscr.2025.111348. Epub 2025 Apr 23.

DOI:10.1016/j.ijscr.2025.111348
PMID:40286691
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12060479/
Abstract

INTRODUCTION

Synovial sarcoma is a soft-tissue sarcoma whose histological origin has long been poorly understood. It occurs mainly near the extremities of larges joints but can occur anywhere in the body. Synovial sarcoma of hepatic origin is extremely rare, with only 13 cases reported in the literature.

CASE PRESENTATION

We report the case of a 68-year-old female patient who underwent right hepatectomy for a single 12.5 cm mass arising in the S6-S7 segments and evolving for over three years, long considered to be hepatocellular carcinoma. An Anatomopathological examination of the operative specimen showed an undifferentiated tumoral process. The immunohistochemical complement demonstrated a monophasic synovial sarcoma.

CLINICAL DISCUSSION

Clinical and radiological signs are not specific, and diagnosis is based on histology, of which there are three types. Complete surgical excision remains the best therapeutic option for localized forms. It may or may not be combined with adjuvant chemotherapy in other forms, although the prognosis of the disease remains guarded.

CONCLUSION

Synovial sarcoma of the liver is a rare disease with a histological diagnosis. Treatment is not standardized, and surgical excision remains the best option for localized forms.

摘要

引言

滑膜肉瘤是一种软组织肉瘤,其组织学起源长期以来一直难以理解。它主要发生在大关节的四肢附近,但也可发生在身体的任何部位。肝源性滑膜肉瘤极为罕见,文献中仅报道了13例。

病例报告

我们报告一例68岁女性患者,因位于S6 - S7段的一个12.5厘米的单发肿块接受了右肝切除术,该肿块已发展三年多,长期以来一直被认为是肝细胞癌。手术标本的解剖病理学检查显示为未分化肿瘤过程。免疫组化结果证实为单相滑膜肉瘤。

临床讨论

临床和放射学征象不具有特异性,诊断基于组织学,组织学有三种类型。对于局限性滑膜肉瘤,完整的手术切除仍然是最佳治疗选择。对于其他类型,可能联合或不联合辅助化疗,尽管该疾病的预后仍然不容乐观。

结论

肝滑膜肉瘤是一种罕见的具有组织学诊断的疾病。治疗尚无标准化方案,手术切除对于局限性滑膜肉瘤仍然是最佳选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/37be/12060479/dc145a313de0/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/37be/12060479/9b449c74f472/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/37be/12060479/39aaf0e19648/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/37be/12060479/dc145a313de0/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/37be/12060479/9b449c74f472/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/37be/12060479/39aaf0e19648/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/37be/12060479/dc145a313de0/gr3.jpg

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