Kumarasamy Sivaraman, Garg Kanwaljeet, Singh Pankaj Kumar, Kumar Amandeep, Sharma Rajeev, Kedia Shweta, Mishra Shashwat, Borkar Sachin, Sawarkar Dattaraj Parmanad, Verma Satish Kumar, Gupta Subhash, Satyarthee Gurudutta, Garg Ajay, Sharma Meher C, Kumar Rajinder, Singh Manmohan, Suri Ashish, Chandra Poodipedi Sarat, Kale Shashank Sharad
Department of Neurosurgery, All India Institute of Medical Sciences, CNC, Room No 720, New Delhi, India.
Department of Radiation Oncology, All India Institute of Medical Sciences, New Delhi, India.
Childs Nerv Syst. 2024 Dec 13;41(1):46. doi: 10.1007/s00381-024-06707-y.
Ewing's sarcoma (EWS) is a malignant round-cell tumor arising from the bone and soft tissue. It is a disease of children and young adults. EWS affecting the central nervous system (cranial and spinal column) is relatively rare with an annual incidence of approximately one case per million in the Western population. Due to their rarity, very few studies are available in the literature. We present our experience of managing 21 such cases, highlighting their clinical, and radiological findings, treatment strategy, and surgical outcomes in patients with primary EWS affecting the central nervous system.
We retrospectively collected hospital records of patients with primary EWS affecting the CNS (cranial and spinal column), who had been surgically treated in our Neuroscience Center between 2015 and 2023. Patients' demographics, presentation, radiological findings, treatment strategy including surgery and biopsy followed by adjuvant therapy, and outcome at discharge, and the latest follow-up were analyzed from our database.
There were sixteen male and five female patients with a mean age of 18.22 ± 12.73 years (ranging from 6 months to 59 years). The commonest presentation was headache and vomiting in cranial lesions (5/13 patients, 38.46%), and back pain in spinal lesions (4/8 patients, 50%). The site of lesions was cranial in thirteen patients (61.9%) and spinal column in eight patients (38.1%). The commonest site was the frontal region in the cranial group (6 patients, 46.15%) and the lumbar region in the spinal group (4 patients, 50%). All patients underwent surgical intervention [tumor resection (18) and biopsy (3)]. Tumor resection was achieved in 18 patients (85.71%). The extent of resection was gross-total excision in 9 patients (42.86%), near-total excision in 5 patients (23.8%), and tumor decompression in 4 patients (19%). Four patients underwent spinal instrumentation. Fifteen patients (71.42%) received multiagent chemo-radiotherapy according to institute protocol. Five patients (23.8%) with poor KPS expired within 6 months of surgery and could not receive adjuvant therapy. Two patients (9.52%) improved symptomatically, and nine patients (42.86%) remained asymptomatic at a mean follow-up of 25.1 ± 29 months. One patient (4.76%) after receiving 2 cycles of adjuvant therapy had initial improvement but expired later due to disease progression. Four patients after adjuvant chemo-radiotherapy (19%) expired due to systemic spread.
Primary EWS affecting the central nervous system is a rare variety. A detailed radiological assessment can aid in adequate planning for safe maximal resection. Timely tissue diagnosis is essential for initiating early treatment. Radical excision followed by adjuvant therapy offers a favorable outcome. Postoperative adjuvant chemo-radiotherapy aids in optimal disease control and surgical outcome. With the current treatment strategy, good surgical outcomes can be achieved. However, metastasis is not uncommon and should be evaluated regularly.
尤因肉瘤（EWS）是一种起源于骨骼和软组织的恶性圆形细胞肿瘤。它是儿童和年轻人易患的疾病。EWS累及中枢神经系统（颅骨和脊柱）相对罕见，在西方人群中每年发病率约为百万分之一。由于其罕见性，文献中相关研究很少。我们介绍了21例此类病例的治疗经验，重点阐述了原发性EWS累及中枢神经系统患者的临床、影像学表现、治疗策略及手术结果。
我们回顾性收集了2015年至2023年期间在我们神经科学中心接受手术治疗的原发性EWS累及中枢神经系统（颅骨和脊柱）患者的医院记录。从我们的数据库中分析患者的人口统计学资料、临床表现、影像学表现、治疗策略（包括手术和活检，随后进行辅助治疗）、出院时及最新随访时的结果。
16例男性和5例女性患者，平均年龄18.22±12.73岁（范围从6个月至59岁）。最常见的表现是颅骨病变患者出现头痛和呕吐（5/13例患者，38.46%），脊柱病变患者出现背痛（4/8例患者，50%）。病变部位13例患者为颅骨（61.9%），8例患者为脊柱（38.1%）。颅骨组最常见部位是额部（6例患者，46.15%），脊柱组最常见部位是腰部（4例患者，50%）。所有患者均接受了手术干预[肿瘤切除（18例）和活检（3例）]。18例患者（85.71%）实现了肿瘤切除。切除范围为9例患者（42.86%）行全切除，5例患者（23.8%）行近全切除，4例患者（19%）行肿瘤减压。4例患者接受了脊柱内固定。15例患者（71.42%）根据机构方案接受了多药化疗放疗。5例KPS评分低的患者在术后6个月内死亡，未能接受辅助治疗。2例患者（9.52%）症状改善，9例患者（42.86%）在平均25.1±29个月的随访中无症状。1例患者（4.76%）在接受2个周期辅助治疗后最初病情改善，但后来因疾病进展死亡。4例接受辅助化疗放疗的患者（19%）因全身扩散死亡。
原发性EWS累及中枢神经系统是一种罕见类型。详细的影像学评估有助于为安全的最大程度切除进行充分规划。及时的组织诊断对于尽早开始治疗至关重要。根治性切除后辅助治疗可带来良好的结果。术后辅助化疗放疗有助于实现最佳的疾病控制和手术结果。采用当前的治疗策略可取得良好的手术效果。然而，转移并不罕见，应定期进行评估。
