单纯广泛切除肿瘤治疗低度恶性中心型成骨肉瘤——13例低度恶性中心型成骨肉瘤的诊治经验及文献回顾

Low grade central osteosarcoma treated by wide resection-experience in 13 consecutive patients from one center

[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]To retrospectively study all the low grade central osteosarcoma（LGCO） cases treated in our center in about a past decade.[Method]Thirteen cases with LGCO treated from January 1998 to June 2008 were studied.The mean age at diagnosis was 31-year-old.The anatomical location of the lesions was similar to that of a conventional osteosarcoma.Pain and mass were common complaints with a mean duration of 16 months before diagnosis.Eight cases accepted the first operation in our center,while the other 5 cases had undergone operations in other hospitals with an intralesional resection in four and a marginal resection in one.Eleven patients had an Enneking stage I disease and two had a stage III disease.The first choice for patients with stage I disease was wide resection.[Result]In two patients with local recurrence,the postoperative pathological diagnoses were fibrosarcoma and malignant fibrous histocytoma respectively.The metastatic lesions were found to be a LGCO in both patients with stage III disease.The average follow up duration was 29 months.Lung metastasis occurred in one stage I patient 15 months after operation.Gama-knife was used to get rid of the lung lesions and then systemic chemotherapy was given.Now he was still alive with disease 27 months after the first operation.One of the 2 patients with stage III disease died of the disease 26 months after operation and the other one was failed to follow up 6 months after operation.The other 10 patients were alive without disease at the last follow-up.For those who received limb salvage operations,the mean MSTS 93 score was 79%.[Conclusion]For most patients with LGCO,wide resection of tumor is preferred.Good oncologic and functional results could be expected after a limb salvage operation without chemotherapy.Insufficient resection of tumor would lead to recurrence.Long time follow up is compulsory.High grade sarcomas would be found in some of the local recurrent lesions.Metastasis would occur in few patients and the metastatic lesions may still be a LGCO.