Berner Kjetil, Johannesen Tom Børge, Bruland Øyvind S
Department of Oncology, Oslo University Hospital, Norwegian Radium Hospital, 0424 Oslo, Norway ; The Norwegian Cancer Registry, 0304 Oslo, Norway.
The Norwegian Cancer Registry, 0304 Oslo, Norway.
Sarcoma. 2015;2015:917679. doi: 10.1155/2015/917679. Epub 2015 Aug 30.
Purpose. To describe epidemiological, clinical characteristics and treatment outcomes of low-grade osteosarcoma (LGOS), including dedifferentiated osteosarcoma (DLGOS). Method. We analysed a nationwide cohort comprised of patients with histologically verified LGOS and DLGOS between 1975 and 2009, based on registry sources supplemented with clinical records from hospitals involved in sarcoma management. Results. Fifty-four patients were identified, 12 of whom had DLGOS. The annual incidence for all patients was 0.3 per million, with the peak incidence in the third decade of the life. Fifteen patients experienced local relapses during follow-up and ten developed metastatic diseases, including three at primary diagnosis. Patients with DLGOS dominated the metastatic relapse group. The five-year sarcoma-specific survival rate was 91%, with no documented improvement over time. Free margin following surgical resection of the primary tumour had a positive impact on survival. As expected, both local relapse and metastasis during follow-up were associated with an unfavourable outcome. Radiotherapy predicted poor survival due to the selection of high-risk patients in need of such treatment. Neither higher age nor axial tumour localisation was adverse prognostic factors. Conclusion. LGOS has an excellent prognosis when surgically resected with a free margin; however, LGOS has the potential to dedifferentiate and metastasize with a poor outcome.
目的。描述低级别骨肉瘤（LGOS）的流行病学、临床特征及治疗结果，包括去分化骨肉瘤（DLGOS）。方法。我们分析了一个全国性队列，该队列由1975年至2009年间组织学确诊的LGOS和DLGOS患者组成，数据来源于登记处，并补充了参与肉瘤管理的医院的临床记录。结果。共识别出54例患者，其中12例为DLGOS。所有患者的年发病率为百万分之0.3，发病高峰在生命的第三个十年。15例患者在随访期间出现局部复发，10例发生转移性疾病，其中3例在初次诊断时即出现转移。DLGOS患者在转移性复发组中占主导地位。五年肉瘤特异性生存率为91%，随时间推移无明显改善。原发肿瘤手术切除后的切缘阴性对生存有积极影响。正如预期的那样，随访期间的局部复发和转移均与不良预后相关。放疗预示着较差的生存率，因为接受此类治疗的是高危患者。较高年龄和轴向肿瘤定位均不是不良预后因素。结论。LGOS手术切除且切缘阴性时预后良好；然而，LGOS有去分化和转移的可能，预后较差。
