Department of Internal Medicine, Guthrie Robert Packer Hospital, Sayre, Pennsylvania, USA.
Department of Internal Medicine, Penn State College of Medicine, Hershey, Pennsylvania, USA.
Cancer Rep (Hoboken). 2022 Nov;5(11):e1725. doi: 10.1002/cnr2.1725. Epub 2022 Oct 3.
Ewing sarcoma (ES), the second most common malignant bone tumor after osteosarcoma in the second decade, occurs in 0.9% of cases as the primary non-sacral form.
A 20-years-old male presented with acute paraparesis of bilateral lower limb and numbness following initial back pain for the last 6 months. Magnetic resonance imaging (MRI) of the lumbar spine revealed a 4 cm enhancing soft tissue mass at the L4/L5 vertebra extending into the spinal canal with compression of the thecal sac. The computed tomography (CT) of the chest, abdomen, and pelvis revealed aggressive lytic lesions in the L4 spinous process with soft tissue extension into the spinal canal with no other site of distant metastasis. He was treated with IV steroids (Injection dexamethasone 10 mg IV followed by 4 mg tablet dexamethasone q6h; subsequently tapered off). A core needle biopsy showed a small, round blue cell neoplasm, (suggestive of a primitive neuroectodermal) stained positive for CD99 and vimentin stain. The diagnosis of ES lumbar spine was made which was treated with surgical resection with an appropriate margin measuring 8 × 4.5 × 2.5 cm with decompression and L4/5 laminectomies, which had a negative margin in the surgical pathology report. Concomitant local radiotherapy and chemotherapy [cycles of vincristine 2 mg/m , adriamycin/doxorubicin 75 mg/m , cyclophosphamide 1200 mg/m (VDC) with mesna rescue alternating with cycles of ifosfamide 1800 mg/m and etoposide 100 mg/m (IE)] was started. The motor strength was regained gradually with preserved spine biomechanics and oncological control with no recurrence in 2-year follow-ups.
The presentation of lumbar ES can vary from local pain and swelling to acute paraparesis. Timely diagnosis and treatment with multimodal therapy, namely, steroids for acute spinal cord compression and surgery with chemoradiotherapy for ES can improve spinal biomechanics and oncological control.
尤因肉瘤(Ewing sarcoma,ES)是继骨肉瘤之后第二十年纪第二常见的恶性骨肿瘤,其原发非骶骨形式的发病率为 0.9%。
一名 20 岁男性,最初表现为背痛 6 个月后出现双侧下肢急性截瘫和麻木。腰椎磁共振成像(MRI)显示 L4/L5 椎骨处有 4cm 增强的软组织肿块,延伸至椎管并压迫脊膜囊。胸部、腹部和骨盆的 CT 显示 L4 棘突处侵袭性溶骨性病变,伴有软组织向椎管内延伸,无其他远处转移部位。他接受了 IV 类固醇(静脉注射地塞米松 10mg,随后口服地塞米松 4mg,q6h;随后逐渐减量)治疗。骨髓活检显示为小圆蓝细胞肿瘤,(提示原始神经外胚层)CD99 和波形蛋白染色阳性。诊断为腰椎 ES,行手术切除,切缘适当,大小为 8×4.5×2.5cm,行减压和 L4/5 椎板切除术,手术病理报告显示切缘阴性。同时进行局部放疗和化疗[长春新碱 2mg/m2,阿霉素/多柔比星 75mg/m2,环磷酰胺 1200mg/m2(VDC)并用美司钠解救,与异环磷酰胺 1800mg/m2 和依托泊苷 100mg/m2(IE)交替的周期]。随着脊柱生物力学和肿瘤控制的恢复,运动力量逐渐恢复,且在 2 年随访中无复发。
腰椎 ES 的表现可从局部疼痛和肿胀到急性截瘫不等。及时诊断和多模式治疗,即类固醇治疗急性脊髓压迫和手术联合放化疗治疗 ES,可改善脊柱生物力学和肿瘤控制。