Department of Orthopaedic Oncology, Spinal Tumor Center, Shanghai Changzheng Hospital, Second Military Medical University, Shanghai, 200003, China.
Department of Orthopaedic Oncology, Spinal Tumor Center, Shanghai Changzheng Hospital, Second Military Medical University, Shanghai, 200003, China; Department of Orthopaedics, The People's Hospital of Taizhou City, Taizhou, Jiangsu Province, 225300, China.
Clin Neurol Neurosurg. 2020 May;192:105729. doi: 10.1016/j.clineuro.2020.105729. Epub 2020 Feb 6.
Rhabdomyosarcoma (RMS) involving the spine is rare. The aim of the present study is to explore the clinicopathological features, surgical treatments and outcomes of this rare disease.
Eleven patients with spinal RMS who received surgery in our institution between 2012 and 2018 were retrospectively investigated. The literature on spinal RMS was also reviewed.
Our study consisted of 7 cases of primary RMS and 4 cases of metastatic RMS. Seven primary and one metastatic spinal RMS received radical resection, the remaining three metastatic patients received palliative resection. Eight patients died with a median survival time of 8 months. The mean value of Ki-67 positivity was 48.2 %. Literature review revealed a total of 22 previously reported cases. 54.5 % of the pooled cases of the 33 patients were under the age of 18. Of the 20 patients with primary spinal RMS, 9 cases were diagnosed as embryonal, while 6 of the 13 metastatic patients were diagnosed as alveolar. Multiple modalities, including surgery and concurrent adjuvant therapy were performed in 19 patients. The median overall survival (OS) for 28 patients with detailed follow-up information was 10 months. Radical resection offered a significant longer median OS than non-radical resection (18 vs. 6 months, p = 0.027).
Spinal RMS mainly affects young patients. The embryonal form and alveolar form is the most frequent subtype for primary and metastatic spinal RMS respectively. Spinal RMS is highly aggressive with dismal prognosis. Multimodality therapies are the mainstay of treatment. Radical resection is strongly recommended in eligible patients.
脊柱横纹肌肉瘤（RMS）较为罕见。本研究旨在探讨该罕见疾病的临床病理特征、手术治疗及预后。
回顾性分析 2012 年至 2018 年期间在我院接受手术治疗的 11 例脊柱 RMS 患者的临床资料。同时对脊柱 RMS 的相关文献进行复习。
本研究中 7 例为原发性 RMS，4 例为转移性 RMS。7 例原发性和 1 例转移性 RMS 患者接受了根治性切除，其余 3 例转移性患者接受了姑息性切除。8 例患者死亡，中位生存时间为 8 个月。Ki-67 阳性率的平均值为 48.2%。文献复习共纳入 33 例患者的 22 例既往报道病例。33 例患者中 54.5%的患者年龄小于 18 岁。20 例原发性脊柱 RMS 患者中，9 例诊断为胚胎性，13 例转移性患者中 6 例诊断为肺泡性。19 例患者采用了包括手术和辅助治疗在内的多种治疗方式。28 例有详细随访资料的患者的中位总生存期（OS）为 10 个月。根治性切除患者的中位 OS 明显长于非根治性切除患者（18 个月 vs. 6 个月，p=0.027）。
脊柱 RMS 主要发生于年轻患者。原发性和转移性脊柱 RMS 分别以胚胎性和肺泡性为最常见的亚型。脊柱 RMS 侵袭性强，预后差。多学科治疗是主要治疗方法。在合适的患者中，强烈推荐根治性切除。
