A curious cervical spine case: multiple, primary CNS leiomyosarcomas presenting with rapid growth in the immunocompromised patient.

IF 0.7 Q4 CLINICAL NEUROLOGY
Aleka Scoco, Kainaat Javed, Reza Yassari
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Abstract

Introduction: Primary CNS leiomyosarcomas are rare, dural-based intracranial or intravertebral tumors seen in immunocompromised patients and are associated with latent EBV infection. They may mimic a meningioma or schwannoma on imaging but their clinical presentation progresses much more rapidly. Often times, these tumors are hard to distinguish from secondary, metastatic leiomyosarcoma.

Case presentation: A 30-year-old female with congenital HIV presented to clinic with shoulder pain, paresthesias of the right upper extremity and gait instability. She was noted to have a contrast enhancing dural-based spinal canal lesion measuring 1.5 cm at the C1 vertebral level on MRI. Surgery was proposed but patient deferred. She represented to our Emergency Department 1 month later with right-sided hemiparesis and difficulty with ambulation. On repeat MRI, the lesion had grown to 2.6 cm. She was taken to the OR emergently for gross total tumor resection. The histopathology demonstrated a primary CNS leiomyosarcoma. MRI scan of the brain revealed an extra-axial right frontal lobe lesion measuring 1.8 cm which was also treated with subtotal surgical resection followed by proton beam radiotherapy.

Discussion: Primary CNS leiomyosarcomas should be considered in young immunocompromised patients presenting with dural-based spinal cord tumors. Histopathological studies including EBV testing can definitively make the diagnosis. These tumors have an aggressive nature and need to be treated with complete surgical resection to prevent severe neurological deterioration and adjuvant therapy to prevent recurrence.

Abstract Image

一个奇特的颈椎病例:免疫力低下患者中出现的快速生长的多发性原发性中枢神经系统白肌瘤。
导言:原发性中枢神经系统良性肉瘤是一种罕见的硬脑膜颅内或椎管内肿瘤,多见于免疫功能低下的患者,与潜伏的EB病毒感染有关。它们在影像学上可能与脑膜瘤或分裂瘤相似,但临床表现进展更快。很多时候,这些肿瘤很难与继发性、转移性骨髓瘤区分开来:一名患有先天性艾滋病的 30 岁女性患者因肩部疼痛、右上肢麻痹和步态不稳前来就诊。核磁共振检查发现,她的C1椎体位置有一个造影剂增强的硬脊膜椎管病变,大小为1.5厘米。医生建议进行手术治疗,但患者拒绝了。一个月后,她因右侧肢体偏瘫和行走困难到我院急诊科就诊。再次进行核磁共振检查时,病灶已增大到 2.6 厘米。她被紧急送往手术室进行肿瘤全切。组织病理学显示,这是一种原发性中枢神经系统良性肉瘤。脑部核磁共振成像扫描显示,右额叶轴外病变达1.8厘米,也进行了次全切手术,随后进行了质子束放疗:讨论:对于出现硬脊膜脊髓肿瘤的免疫功能低下的年轻患者,应考虑原发性中枢神经系统白肌瘤。组织病理学研究包括 EBV 检测可明确诊断。这些肿瘤具有侵袭性,需要进行彻底的手术切除治疗,以防止严重的神经功能衰退,并进行辅助治疗以防止复发。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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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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