Dorsal hemangioblastoma with holocord syringomyelia: case report

A. Arévalo-Sáenz, M. Pedrosa Sánchez
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Abstract

Introduction: Intramedullary hemangioblastomas are usually accompanied by syringomyelia. However, a holocord syringomyelia is rare. The most common cause of syringomyelia continues to be Chiari disease, and only 10 cases of hemangioblastomas with holocord syringomyelia reported so far. Case report: We present a case of a 35-year-old patient with a two-month history of cervicobrachialgia at the C7-C8 root level, previously preceded by pain at the D1-D2 level. Cervico-dorso-lumbar MRI revealed a medullar tumor with hyper-uptake mural nodule at the conus medullaris level accompanied by an extensive syringomyelic cavity from C5 to L1 compatible with medullary hemangioblastoma. The patient underwent surgery for tumor resection with subsequent resolution of her painful symptoms. Conclusion: It is important to note that the surgery is aimed at treating the origin of this syringomyelia and not the syringomyelia itself. Although the majority of patients with holocord syringomyelia have Chiari as its cause, the possibility of focal spinal intramedullary tumors as being responsible for syringomyelia should not be forgotten.
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背侧血管母细胞瘤伴完全性脊髓空洞1例
髓内血管母细胞瘤通常伴有脊髓空洞。然而,完全性脊髓空洞是罕见的。引起脊髓空洞最常见的原因仍然是基亚里氏病,迄今为止仅报道了10例血管母细胞瘤伴完全性脊髓空洞。病例报告:我们报告了一例35岁的患者,有两个月的C7-C8根水平颈臂痛病史,之前有D1-D2水平疼痛。颈背腰椎MRI显示髓质肿瘤伴髓圆锥高摄取壁结节,伴从C5到L1广泛的脊髓空洞,与髓质血管母细胞瘤相符。患者接受手术切除肿瘤,随后疼痛症状得到缓解。结论:重要的是要注意,手术的目的是治疗这种脊髓空洞的起源,而不是脊髓空洞本身。虽然大多数完全性脊髓空洞症患者的病因为Chiari,但不应忘记局灶性脊髓髓内肿瘤导致脊髓空洞的可能性。
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