Addesso Luke, Koenig David, Huq Nadia
Medical College of Wisconsin, Milwaukee, WI.
Des Moines University College of Osteopathic Medicine, Des Moines, IA.
Ochsner J. 2018 Winter;18(4):402-405. doi: 10.31486/toj.18.0039.
Ewing sarcoma, a rare cause of cord compression, is predominantly of osseous origin but can also originate in soft tissues. Soft-tissue manifestations account for <15% of all Ewing sarcoma tumors, and even fewer cases of Ewing sarcoma originating in the epidural space have been documented.
A 19-year-old female presented to the emergency department for worsening low-back pain during the previous 6 months and numbness and weakness in her legs during the prior 2 weeks. Magnetic resonance imaging revealed an epidural mass at the L4-L5 level. Intravenous steroids were started for a presumed diagnosis of lymphoma. Orthopedic surgery consultants deferred computed tomography-guided biopsy of the mass out of concern for tumor seeding. Compression symptoms worsened to include foot drop and saddle anesthesia, prompting urgent radiation therapy. After the patient showed poor response to appropriate treatment for lymphoma, other malignant and infectious causes were considered. Biopsy was performed on day 3 of the patient's hospital stay, and by day 7, preliminary cytology results revealed Ewing sarcoma. Subsequent laminectomy and tumor resection produced immediate relief of pain, along with a gradual return of strength and sensation. The mass was found to be of soft-tissue origin and was classified as an extraosseous Ewing sarcoma. The patient was referred to a pediatric oncologist to complete the appropriate chemotherapy after diagnosis.
This case demonstrates how an uncommon manifestation of a rare disease can mimic a classic presentation of cord compression. Our aim is to bring awareness to this disease and to emphasize the importance of timely biopsy of any mass.
尤因肉瘤是导致脊髓压迫的罕见病因，主要起源于骨组织，但也可起源于软组织。软组织表现占所有尤因肉瘤肿瘤的比例不到15%，而起源于硬膜外间隙的尤因肉瘤病例记录更少。
一名19岁女性因前6个月来下背部疼痛加重以及前2周腿部麻木和无力就诊于急诊科。磁共振成像显示L4 - L5水平有一个硬膜外肿块。因推测诊断为淋巴瘤，开始静脉注射类固醇。骨科手术会诊医生出于对肿瘤播散的担忧，推迟了对肿块进行计算机断层扫描引导下的活检。压迫症状加重，包括足下垂和鞍区感觉缺失，促使进行紧急放射治疗。在患者对淋巴瘤的适当治疗反应不佳后，考虑了其他恶性和感染性病因。在患者住院第3天进行了活检，到第7天，初步细胞学结果显示为尤因肉瘤。随后的椎板切除术和肿瘤切除术立即缓解了疼痛，同时力量和感觉逐渐恢复。发现肿块起源于软组织，被归类为骨外尤因肉瘤。患者被转诊给儿科肿瘤学家，以便在诊断后完成适当的化疗。
本病例展示了一种罕见疾病的罕见表现如何模仿脊髓压迫的典型表现。我们的目的是提高对这种疾病的认识，并强调对任何肿块及时进行活检的重要性。
