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整块全脊椎切除术治疗原发性脊柱平滑肌肉瘤:泰国首例病例报告及文献综述

Primary leiomyosarcoma of the spine treated with total en bloc spondylectomy: the first case in Thailand-a case report and literature review.

作者信息

Pluemvitayaporn Tinnakorn, Piyaskulkaew Chaiwat, Kunakornsawat Sombat, Pruttikul Pritsanai

机构信息

Spine Unit, Institute of Orthopaedics, Lerdsin Hospital, Bangkok, Thailand.

出版信息

J Spine Surg. 2017 Dec;3(4):707-714. doi: 10.21037/jss.2017.10.04.

Abstract

Leiomyosarcoma is a rare type of malignant soft tissue tumor and also one of the most aggressive soft tissue sarcomas. It commonly occurs in uterus, abdominal viscera, retroperitoneal space and soft tissue of the extremities. Primary osseous leiomyosarcoma is a rare condition. Furthermore, primary leiomyosarcoma of the spine is extremely rare. Only few cases have been reported. However, the treatment and outcomes remains controversial. Therefore, the objective of this case report is to illustrate the management of this extremely rare disease by using total en bloc spondylectomy (TES) procedure, which is one of a suitable option for surgical resection of the spinal tumors. In this study, we presented an unusual case of a 61-year-old female, who was diagnosed as primary leiomyosarcoma of the twelfth thoracic spine with liver metastasis, treated with total en bloc spondylectomy of the twelfth thoracic vertebra followed by chemotherapy. On last follow-up, 6 months after the surgery, the clinical outcome remained in good condition and no signs of local recurrence. In conclusion, primary leiomyosarcoma of the spine is an extremely rare and difficult to diagnosis. Immunohistochemistry studies are very important for confirmation of the diagnosis. Standard treatment remains controversial. However, surgical resection is still treatment of choice. One of the most effective surgical options is TES, which give the better outcome and minimize local recurrence, if indicate.

摘要

平滑肌肉瘤是一种罕见的恶性软组织肿瘤,也是最具侵袭性的软组织肉瘤之一。它通常发生于子宫、腹腔脏器、腹膜后间隙及四肢软组织。原发性骨平滑肌肉瘤较为罕见。此外,原发性脊柱平滑肌肉瘤极为罕见,仅有少数病例报道。然而,其治疗方法及预后仍存在争议。因此,本病例报告的目的是通过整块全脊椎切除术(TES)来说明这种极其罕见疾病的治疗,TES是脊柱肿瘤手术切除的合适选择之一。在本研究中,我们报告了一例不同寻常的病例,一名61岁女性被诊断为第十二胸椎原发性平滑肌肉瘤伴肝转移,接受了第十二胸椎整块全脊椎切除术后进行化疗。在术后6个月的最后一次随访中,临床结果良好,无局部复发迹象。总之,原发性脊柱平滑肌肉瘤极为罕见且难以诊断。免疫组织化学研究对确诊非常重要。标准治疗仍存在争议。然而,手术切除仍是首选治疗方法。最有效的手术选择之一是TES,如果有指征,它能带来更好的结果并将局部复发降至最低。

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