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[目的]低度恶性中心型成骨肉瘤是发生于骨髓腔的低度恶性成骨肉瘤,临床罕见。总结本中心收治的低度恶性中心型成骨肉瘤病例的诊治过程及经验。[方法]回顾本中心自1998年1月~2008年6月收治的13例低度恶性中心型成骨肉瘤。患者平均就诊年龄31岁。8例为初治病例,5例为外院手术后复发病例。后者中4例在外院行囊内手术,1例行边缘手术。Ⅰ期肿瘤11例,Ⅲ期肿瘤2例。Ⅰ期患者均首选肿瘤单纯广泛切除术。[结果]2例局部复发者术后病理分别为纤维肉瘤和恶性纤维组织细胞瘤。2例Ⅲ期患者转移灶术后病理均为低度恶性中心型成骨肉瘤。术后平均随访29个月。1例Ⅰ期肿瘤患者术后15个月出现肺转移,其接受综合治疗后已继续存活27个月。1例Ⅲ期患者术后26个月死亡,另1例术后随访6个月后失访。其余10例患者未发现局部复发、远处转移。保肢手术者,MSTS 93评分平均79%。[结论]低度恶性中心型成骨肉瘤患者多数只需要广泛切除、保肢治疗,无需化疗即可获得较长期的生存和较好的术后功能。手术切除边界不足依然是最终导致肿瘤复发的常见原因。长期随访是必要的。部分局部复发病灶可能表现为高度恶性肉瘤。少部分患者可能出现远处转移病灶,转移病灶病理可能依然表现为“高分化”。
[Objective] Low-grade malignant central osteosarcoma is a low-grade osteosarcoma in bone marrow which is rare in clinical practice. Summary of the center admitted to the low-grade malignant central osteosarcoma cases of diagnosis and treatment process and experience. [Method] Thirteen cases of low grade malignant central osteosarcoma were retrospectively treated in our center from January 1998 to June 2008. The average age of patients was 31 years old. 8 cases of newly diagnosed cases, 5 cases of recurrent cases of external hospital surgery. The latter 4 cases in the outer hospital cystic surgery, 1 case of marginal surgery. Stage Ⅰ tumor in 11 cases, stage Ⅲ tumor in 2 cases. Stage Ⅰ patients are the preferred choice of extensive radical resection of the tumor. [Results] The postoperative pathology of 2 cases of local recurrence were fibrosarcoma and malignant fibrous histiocytoma respectively. Two cases of stage Ⅲ patients with metastasis pathology were low-grade malignant central osteosarcoma. After an average follow-up of 29 months. One patient with stage I tumor developed pulmonary metastases 15 months after operation and survived for 27 months after receiving combined treatment. One patient with stage III died after 26 months and the other one was lost after 6 months of follow-up. The remaining 10 patients found no local recurrence, distant metastasis. Limb salvage operation, MSTS 93 average 79%. [Conclusion] The majority of patients with low-grade malignant central osteosarcoma need only extensive resection and limb salvage treatment, and can obtain long-term survival and good postoperative function without chemotherapy. Insufficient surgical removal of the border remains the most common cause of tumor recurrence. Long-term follow-up is necessary. Some local recurrent lesions may manifest as highly malignant sarcoma. A small number of patients may have distant metastasis lesions, metastasis lesions may still be manifested as “well-differentiated ”.