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胸椎原发性孤立性淀粉样瘤:一例报告并文献复习

Primary solitary amyloidoma of thoracic spine: a case report and review of the literature.

作者信息

Unal A, Sütlap P-N, Kýyýk M

机构信息

Department of Neurology, Faculty of Medicine, Zonguldak Karaclmas University, Kozlu, 67700, Zonguldak, Turkey.

出版信息

Clin Neurol Neurosurg. 2003 Jul;105(3):167-9. doi: 10.1016/s0303-8467(02)00141-5.

Abstract

Primary solitary amyloidoma of spine is a rare subset of amyloidosis in which the amyloid deposition is focal and not secondary to a systemic process or plasma cell discrasia. Only seventeen cases of spine involvement have been reported. We report a 71-year-old man with 3 months history of ataxia and frequent falls. On the 4th month paraparesis developed. Magnetic resonance imaging of the dorsal spine revealed a lesion involving the T-9 vertebral level with significant spinal cord compression. Decompressive laminectomy with posterior instrumentation and fusion from T8 to T10 was performed. Histopathology revealed abundant amyloid deposits. No evidence of myeloproliferative disease or systemic amyloidosis was found. The patient showed marked neurological improvement with residual mild spastic gate 6 months after surgery. When occurring primarily in a localized anatomic area, the prognosis of amyloidoma is excellent, and cure can be expected by local resection. It is important to know that diagnosis requires a high index of suspicion and, ultimately, adequate tissue biopsy for histopathological studies.

摘要

脊柱原发性孤立性淀粉样瘤是淀粉样变性的一种罕见类型,其中淀粉样沉积是局灶性的,并非继发于全身性疾病或浆细胞异常增生。仅报告过17例脊柱受累病例。我们报告一名71岁男性,有3个月共济失调和频繁跌倒病史。第4个月出现双下肢轻瘫。胸段脊柱磁共振成像显示一个累及T9椎体水平的病变,伴有明显脊髓受压。行减压性椎板切除术并进行T8至T10的后路内固定及融合术。组织病理学显示有大量淀粉样沉积物。未发现骨髓增殖性疾病或全身性淀粉样变性的证据。术后6个月,患者神经功能有明显改善,残留轻度痉挛步态。当主要发生在局部解剖区域时,淀粉样瘤的预后良好,通过局部切除有望治愈。重要的是要知道,诊断需要高度怀疑,并最终进行足够的组织活检以进行组织病理学研究。

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