脊髓罗赛-多夫曼病--2 例病例报告和文献综述。

IF 0.7 Q4 CLINICAL NEUROLOGY
Saumyajit Basu, Rohan Gala, Kushal Gohil
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引用次数: 0

摘要

简介罗赛-多夫曼病(RDD)是一种罕见的良性组织细胞疾病,很少累及脊柱。我们报告了两例脊柱 RDD 病例,并回顾了相关文献。本报告探讨了与脊柱 RDD 评估和治疗相关的各种诊断难题:病例 1:一名 32 岁男性,因腰痛和左大腿前侧疼痛就诊 8 个月。经检查,大腿内侧感觉减退,左膝抽搐消失。CT/MRI 扫描显示 L2/3 硬膜外病变,神经受压。正电子发射计算机断层扫描显示,肋骨、肱骨、股骨和椎骨有多处高代谢病变。他接受了硬膜外肿块全切术,并用椎弓根螺钉固定 L2-3,随后接受了化疗。病例 2:一名 42 岁的男性患者,因痉挛性截瘫伴尿失禁就诊 4 周。经检查,他的神经水平为 T6。核磁共振扫描显示,T6-7 后部有病变,压迫脊髓。他接受了 T6-7 椎板切除减压术。两个病例均经组织病理学确诊,并由血液肿瘤专家进一步处理。随访1年后,两人的神经功能均有所改善:讨论:迄今为止,脊柱 RDD 仍是一个巨大的诊断难题,它没有任何临床或放射学特征,与脊柱中的许多溶骨性病变相似。诊断完全依靠组织病理学和免疫学。病变完全手术切除是治疗的主要方法,预后较好,复发几率较低。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Spinal Rosai-Dorfman disease-a report of 2 cases and review of literature.

Spinal Rosai-Dorfman disease-a report of 2 cases and review of literature.

Introduction: Rosai-Dorfman Disease (RDD) is a rare benign histiocytic disease that infrequently affects the spine. We report two cases of spinal RDD and review the relevant literature. This report addresses the various diagnostic dilemmas related to the evaluation of Spinal RDD and its treatment.

Case presentation: Case 1: A 32-year-old male presented with low back pain and left anterior thigh for last 8 months. On examination, there was sensory diminution on inner aspects of the thigh with an absent left knee jerk. CT/MRI scans revealed an extradural lesion at L2/3 with neural compression. PET scan showed several hypermetabolic lesions in ribs, humerus, femur, and vertebrae. He underwent en bloc excision of the extradural mass with L2-3 pedicle screw-rod fixation and was later managed with chemotherapy. Case 2: A 42-year-old male presented with spastic paraparesis with urinary incontinence for the last 4 weeks. On examination, he had a neurological level of T6. MRI scan revealed a lesion in posterior elements of T6-7 compressing the spinal cord. He underwent T6-7 laminectomy with decompression. In both cases, the diagnosis was confirmed by histopathology and further managed by Hemato-oncologist. They both did well at 1-year follow-up with improvement in neurology.

Discussion: Spinal RDD to date remains a large diagnostic dilemma with no pathognomonic clinical or radiological features; mimicking many osteolytic lesions in the spine. The diagnosis is purely histopathological and immunological. The lesion's complete surgical excision is the mainstay of treatment with a better prognosis and decreased chances of recurrences.

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来源期刊
Spinal Cord Series and Cases
Spinal Cord Series and Cases Medicine-Neurology (clinical)
CiteScore
2.20
自引率
8.30%
发文量
92
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