以脊髓压迫为首发症状的髓系肉瘤5例临床分析

Diagnosis and treatment of spinal myeloid sarcoma with acute spinal cord compression as an initial symptom: a report of 5 cases

目的 探讨以脊髓压迫为首发症状脊柱髓系肉瘤患者的临床表现、影像学特征及治疗方法.方法 采用回顾性横断面研究方法.纳入2014年1月—2017年12月长征医院骨肿瘤科收治的经骨髓穿刺及病理证实的脊柱髓系肉瘤患者5例,其中男3例、女2例,年龄15~54岁;肿瘤位于胸椎3例、腰椎2例.4例采取开放性外科手术治疗,出院后根据骨髓穿刺及病理结果,予以化疗及血液系统肿瘤规范化治疗;另1例行内科保守治疗(消炎镇痛、营养支持、化疗等).分析患者治疗前后各项观察项目.结果 5例患者均有腰背部疼痛,伴下肢无力3例、双下肢不全瘫1例;X线检查均未见异常,CT和MRI表现为椎体骨质破坏或占位性病变.骨髓穿刺及术后肿瘤病理结果显示为急性粒细胞白血病4例,慢性粒细胞白血病1例;5例患者均经治疗后症状有所缓解. 5例患者均获随访:1例术后行去甲氧柔红霉素+阿糖胞苷方案化疗5个疗程,并行异基因造血干细胞移植治疗,目前已随访至术后28个月,患者一般状态良好、无肿瘤复发;另外4例经化疗后出现复发,均死于感染,生存期5~26.5个月.结论 以脊髓压迫为首发症状的髓系肉瘤病例临床罕见,其影像学表现缺乏特异性,易出现误诊,骨髓穿刺及病理检查可确诊;当出现脊髓压迫表现时,建议早期行肿瘤切除神经减压术,术后辅以异基因造血干细胞移植和全身化疗.

Objective To explore the clinicopathological features,imaging features and treatment of spinal cord myeloma in patients with spinal cord compression as the first symptom.Methods The retrospective cross-sectional study was conducted which the clinical data of five patients with spinal myeloid sarcoma confirmed by bone marrow aspiration and pathology from January 2014 to December 2017 in Changzheng Hospital.There were 3 males and 2 females,aged from 15 to 54 years old.the tumors were located in 3 cases of thoracic vertebrae and 2 cases of lumbar vertebrae.Four cases were treated with open surgery.After discharge,they were treated with chemotherapy and hematological tumors according to bone marrow puncture and pathological results.Another patient underwent conservative treatment(anti-inflammatory analgesia,nutritional support,chemotherapy,etc.).The observation items were analysed.Results All the 5 patients had a low back pain,three of them had a lower limbs weakness,and one of them was accompanied by paralysis of both lower limbs.X-ray examination showed no abnormal findings.CT and MRI showed bone destruction or soft tissue shadow.Bone marrow aspiration and postoperative pathological examination showed that five cases were leukemia including four acute myeloid leukemia(AML)and one chronic myeloid leukemia(CML).All patients’preoperative symptoms were relieved after treatment.All patients were followed up.One patient underwent IA regimen chemotherapy for five courses,and was treated with allogeneic hematopoietic stem cell transplantation.It had been followed up for 28 months after surgery and still in good condition without tumor recurrence.The other four patients relapsed after chemotherapy,all died of infection,and the survival period was from 5 to 26.5 months.Conclusions Spinal cord compression caused by myeloid sarcoma as an initial symptom is rare.The imaging manifestations of the myeloid sarcoma are lack of specificity and and it is easy to be misdiagnosed.Bone marrow aspiration and pathological examination can confirm the diagnosis.When the symptoms of spinal cord compression occur,it is recommended to perform early tumor decompression.The allogeneic hematopoietic stem cell transplantation and systemic chemotherapy should be performed after surgery.