METHODS: This case report documents a rare instance of an isolated long-segment spinal Rosai-Dorfman disease (C4-D6) along with the review of relevant literature. A 33-year male presented with progressive quadriparesis and urinary retention. A magnetic resonance scan (MRI) revealed a long segment epidural lesion from C4-D6 levels that led to the displacement of the cord. A core biopsy of the spinal tumor revealed characteristic histiocytic emperipolesis and confirmational immunocytohistochemistry markers, confirming the diagnosis. Surgical resection and laminoplasty were performed.
UNASSIGNED: The following histopathology and immunocytohistochemistry findings showed the presence of histiocytes positive for S100 and CD68 positive. Therefore, it was diagnosed to be a case of Rosai-Dorfman disease. The patient had a smooth postoperative recovery and displayed marked motor improvement in the ensuing days. This is a rare case that posed an intriguing challenge to approach.
CONCLUSIONS: To our knowledge, we have encountered one of the most prolonged segmental lesions in isolated spinal Rosai-Dorfman diseases, where surgical management (surgical resection and laminoplasty) has proven to bring about remarkable improvement.
方法:该病例报告了一例罕见的孤立性脊柱长节段Rosai-Dorfman病(C4-D6),并复习了相关文献。一名33岁的男性出现进行性四肢瘫痪和尿潴留。磁共振扫描(MRI)显示C4-D6水平的长段硬膜外病变导致脊髓移位。脊柱肿瘤的核心活检显示了特征性组织细胞增生和确证的免疫细胞组织化学标志物,确认诊断。进行手术切除和椎板成形术。
未经证实:以下组织病理学和免疫细胞组织化学结果显示S100阳性和CD68阳性的组织细胞的存在。因此,被诊断为Rosai-Dorfman病.患者术后恢复顺利,随后几天表现出明显的运动改善。这是一个罕见的案例,对方法提出了有趣的挑战。
结论:据我们所知,我们在孤立的脊柱Rosai-Dorfman病中遇到了最长时间的节段性病变之一,其中手术管理(手术切除和椎管成形术)已被证明带来了显着的改善。