Tan Yi Liang, Ong Wilson, Tan Jiong Hao, Kumar Naresh, Hallinan James Thomas Patrick Decourcy
Department of Diagnostic Imaging, National University Hospital, 5 Lower Kent Ridge Rd, Singapore 119074, Singapore.
University Spine Centre, Department of Orthopaedic Surgery, National University Health System, 1E, Lower Kent Ridge Road, Singapore 119228, Singapore.
J Clin Med. 2023 Aug 29;12(17):5632. doi: 10.3390/jcm12175632.
Epithelioid sarcoma is a rare malignant mesenchymal tumor that represents less than 1% of soft-tissue sarcomas. Despite its slow growth, the overall prognosis is poor with a high rate of local recurrence, lymph-node spread, and hematogenous metastasis. Primary epithelioid sarcoma arising from the spine is extremely rare, with limited data in the literature. We review the existing literature regarding spinal epithelioid sarcoma and report a case of epithelioid sarcoma arising from the spinal cord. A 54 year old male presented with a 1-month history of progressive left upper-limb weakness and numbness. Magnetic resonance imaging (MRI) of the spine showed an enhancing intramedullary mass at the level of T1 also involving the left T1 nerve root. Systemic radiological examination revealed no other lesion at presentation. Surgical excision of the mass was performed, and histology was consistent with epithelioid sarcoma of the spine. Despite adjuvant radiotherapy, there was aggressive local recurrence and development of intracranial metastatic spread. The patient died of the disease within 5 months from presentation. To the best of our knowledge, spinal epithelioid sarcoma arising from the spinal cord has not yet been reported. We review the challenges in diagnosis, surgical treatment, and oncologic outcome of this case.
上皮样肉瘤是一种罕见的恶性间充质肿瘤，占软组织肉瘤的比例不到1%。尽管其生长缓慢，但总体预后较差，局部复发、淋巴结转移和血行转移率较高。起源于脊柱的原发性上皮样肉瘤极为罕见，文献中的数据有限。我们回顾了关于脊柱上皮样肉瘤的现有文献，并报告了一例起源于脊髓的上皮样肉瘤病例。一名54岁男性，有1个月进行性左上肢无力和麻木的病史。脊柱磁共振成像（MRI）显示T1水平有一个强化的髓内肿块，也累及左侧T1神经根。全身放射学检查在初诊时未发现其他病变。对肿块进行了手术切除，组织学检查结果与脊柱上皮样肉瘤一致。尽管进行了辅助放疗，但仍有侵袭性的局部复发和颅内转移扩散。患者在就诊后5个月内死于该病。据我们所知，起源于脊髓的脊柱上皮样肉瘤尚未见报道。我们回顾了该病例在诊断、手术治疗和肿瘤学结局方面的挑战。
