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霍奇金淋巴瘤伴发脊髓压迫:诊断和初始治疗的挑战。

Hodgkin Lymphoma Presenting With Spinal Cord Compression: Challenges for Diagnosis and Initial Management.

机构信息

Department of Paediatric Haematology and Oncology, 2153Cambridge University Hospitals NHS Foundation Trust, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.

Department of Radiology, 2153Cambridge University Hospitals NHS Foundation Trust, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.

出版信息

Pediatr Dev Pathol. 2022 Mar-Apr;25(2):168-173. doi: 10.1177/10935266211033269. Epub 2021 Aug 24.

Abstract

Hodgkin lymphoma (HL) can present with extra-nodal disease, but spinal cord compression is exceptionally rare. We describe a 15-year-old presenting with hip/back pain with normal initial examination. Persistent pain and raised inflammatory markers prompted further investigation with MRI, which revealed an epidural mass causing spinal cord compression. On examination, there was no palpable lymphadenopathy or cauda equina syndrome, but absent lower limb reflexes were noted. Following multidisciplinary discussion, it was determined that cauda equina syndrome was imminent and therefore surgical debulking was undertaken, both to prevent this complication and establish a diagnosis. At surgery, the tumor was highly vascular. Frozen section confirmed lesional material. Following surgery, and given the frozen section findings, a short course of steroids was commenced to reduce any peri-surgical edema. Unfortunately, histopathology was ultimately non-diagnostic, due to failure of immunohistochemistry on technically challenging material. Consequently, ultrasound-guided excision biopsy of a (non-palpable) cervical lymph node was performed five days later; histopathology showed typical effacement of the normal architecture and a conspicuous population of CD15/CD30-positive larger pale cells present, confirming nodular sclerosis classic HL, despite recent steroids. We review the available literature for HL presenting with spinal cord compression and describe the challenges for diagnosis and initial management in such cases.

摘要

霍奇金淋巴瘤(HL)可表现为结外疾病,但脊髓压迫极为罕见。我们描述了一位 15 岁的患者,最初表现为髋/背痛,检查正常。持续性疼痛和炎症标志物升高促使进一步进行 MRI 检查,结果显示硬膜外肿块导致脊髓压迫。检查时,未触及可触及的淋巴结肿大或马尾综合征,但下肢反射消失。经过多学科讨论,确定马尾综合征即将发生,因此进行了手术减压,以防止这种并发症并明确诊断。手术时,肿瘤高度血管化。冷冻切片证实了病变材料。手术后,鉴于冷冻切片的结果,开始短期使用类固醇以减少任何围手术期水肿。不幸的是,由于在技术上具有挑战性的材料上免疫组织化学失败,最终组织病理学诊断不明确。因此,五天后对(不可触及的)颈淋巴结进行了超声引导下切除活检;组织病理学显示典型的正常结构破坏和大量 CD15/CD30 阳性的大苍白细胞,尽管最近使用了类固醇,但仍证实为结节性硬化型经典 HL。我们回顾了 HL 伴发脊髓压迫的现有文献,并描述了此类病例的诊断和初始治疗的挑战。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1956/9109237/2c81533e5ea7/10.1177_10935266211033269-fig1.jpg

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