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腰椎神经根恶性孤立性纤维瘤酷似神经鞘瘤:一例报告。

Malignant solitary fibrous tumor of the lumbar spinal root mimicking schwannoma: a case report.

机构信息

Department of Orthopaedic Surgery, Graduate School of Medicine, Gifu University, 1-1 Yanagido, Gifu 501-1194, Japan.

Department of Orthopaedic Surgery, Graduate School of Medicine, Gifu University, 1-1 Yanagido, Gifu 501-1194, Japan.

出版信息

Spine J. 2014 Jan;14(1):e17-20. doi: 10.1016/j.spinee.2013.07.463. Epub 2013 Oct 9.

Abstract

BACKGROUND CONTEXT

Malignant solitary fibrous tumors (SFTs) arising from the spinal cord are extremely rare and poorly understood mesenchymal neoplasms. To date, only one malignant SFT located in the spinal canal of the sacrum has been described, but none arising from the lumbar nerve root have been reported. Although most SFTs with benign histological features can be treated by complete surgical excision alone, malignant SFTs may require adjuvant therapy. However, systemic chemotherapy and radiotherapy have not been shown effective in patients with malignant SFTs.

PURPOSE

To describe a patient with a malignant SFT arising from the lumbar nerve root.

STUDY DESIGN

A case report and review of literature.

METHODS

We describe the clinical course of the patient and the radiological and pathological findings of the tumor. The effect of systemic chemotherapy was evaluated and the relevant literature was reviewed. This work has no disclosure of funding and was approved by the Institutional Review Board of Gifu University.

RESULTS

The tumor had been resected previously at another hospital, but it recurred and showed multiple metastatic lesions on both lungs within 3 months. Although the patient received systemic chemotherapy, both primary and metastatic lesions were found to be stable disease according to Response Evaluation Criteria in Solid Tumors. The patient died due to cachexia 6 months after her first visit.

CONCLUSION

This patient presented with a highly unusual tumor. Even if a tumor is a dumbbell-shaped mass, similar to a neural tumor, SFT should be considered in the differential diagnosis.

摘要

背景

脊髓内恶性孤立性纤维瘤(SFT)极为罕见,其发病机制尚不清楚。迄今为止,仅描述了 1 例发生在骶骨椎管内的恶性 SFT,而未报道发生在腰椎神经根的恶性 SFT。虽然大多数具有良性组织学特征的 SFT 仅通过完全手术切除即可治疗,但恶性 SFT 可能需要辅助治疗。然而,全身化疗和放疗对恶性 SFT 患者并无疗效。

目的

描述 1 例发生在腰椎神经根的恶性 SFT 患者。

研究设计

病例报告及文献复习。

方法

我们描述了患者的临床病程、肿瘤的影像学和病理学发现。评估了全身化疗的效果,并复习了相关文献。这项工作没有披露资金来源,并得到岐阜大学机构审查委员会的批准。

结果

肿瘤曾在另一家医院切除,但在 3 个月内再次复发,并在双肺出现多处转移病灶。尽管患者接受了全身化疗,但根据实体瘤反应评估标准,原发和转移病灶均为疾病稳定。患者在首次就诊后 6 个月因恶病质死亡。

结论

本例患者表现出一种非常罕见的肿瘤。即使肿瘤呈哑铃状肿块,类似于神经肿瘤,在鉴别诊断中也应考虑 SFT。

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