成骨性骨肿瘤中手术和辅助治疗对生存率的作用。

The role of surgery and adjuvants to survival in Pagetic osteosarcoma.

机构信息

Department of Orthopedics, Istituto Ortopedico Rizzoli and University of Bologna, Via Pupilli 1, Bologna, Emilia Romagna, 40136, Italy.

出版信息

Clin Orthop Relat Res. 2010 Nov;468(11):2962-8. doi: 10.1007/s11999-010-1473-7.

DOI:10.1007/s11999-010-1473-7

PMID:20652460

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC2947701/

Abstract

BACKGROUND

Osteosarcoma is a rare complication of Paget's disease with a very poor prognosis. Treatment is controversial: the older age of the patients affected by Paget's disease may limit the use of chemotherapy and axial involvement may limit the practicality of surgery.

QUESTIONS/PURPOSES: The purposes of this study are (1) to report the survival in patients treated for osteosarcoma in Paget's disease; (2) to identify correlations between type of treatment and survival comparing our data with those in the literature; (3) to determine if the extent of Paget's disease and risk of malignant transformation are associated; (4) to assess if prognosis is related with site; and (5) to identify the variations of histologic subtypes of these osteosarcomas.

METHODS

We retrospectively reviewed the medical records of 26 patients treated between 1961 and 2006 who had bone sarcoma arising from a site of Paget's disease. Twenty two of the 26 patients had surgery. In six surgery only was performed; three had surgery, adjuvant chemotherapy, and radiotherapy; one surgery and radiotherapy; 12 underwent surgery and chemotherapy, adjuvant in 10 patients and neoadjuvant in two; two had only radiotherapy and two had only chemotherapy. We performed survival analyses between various combinations of treatment.

RESULTS

At last followup four patients had no evidence of disease (NED) at a minimum followup of 42.6 months (mean, 139 months; range, 42.6-257.4 months) and 22 died with disease (DWD) at a minimum time of 1 month (mean, 20.2 months; range, 1-84 months). One of the six patients (11%) treated with surgery only had NED at 10 years; the other five died from disease at a mean of 30 months. Three of 12 patients (25%) treated with surgery and chemotherapy are NED at a mean followup of 12 years; nine died of disease at a mean of 24 months. All patients treated without surgery died at a mean of 7.5 months (range, 1-13.7 months).

CONCLUSIONS

Despite improvements in surgery and medical treatments the prognosis remains poor in patients with Paget's sarcoma.

摘要

背景

骨肉瘤是 Pagets 病的一种罕见并发症，预后极差。治疗存在争议：受 Pagets 病影响的患者年龄较大，可能限制化疗的应用，而轴性受累可能限制手术的实用性。

问题/目的：本研究的目的是：(1)报告 Pagets 病患者治疗骨肉瘤的生存率；(2)通过与文献数据进行比较，确定治疗方法与生存率之间的相关性；(3)确定 Pagets 病的范围和恶变风险是否相关；(4)评估预后与部位的关系；(5)确定这些骨肉瘤的组织学亚型的变化。

方法

我们回顾性分析了 1961 年至 2006 年间接受治疗的 26 例源于 Pagets 病部位的骨肉瘤患者的病历。26 例患者中有 22 例接受了手术治疗。6 例仅行手术治疗，3 例手术联合辅助化疗和放疗，1 例手术联合放疗，12 例手术联合化疗，其中 10 例为辅助化疗，2 例为新辅助化疗，2 例仅行放疗，2 例仅行化疗。我们对各种治疗组合进行了生存分析。

结果

末次随访时，4 例患者无疾病证据（NED），随访时间至少为 42.6 个月（平均 139 个月；范围 42.6-257.4 个月），22 例患者死亡（DWD），随访时间至少为 1 个月（平均 20.2 个月；范围 1-84 个月）。6 例仅接受手术治疗的患者中，1 例（11%）在 10 年内 NED，其余 5 例在 30 个月内死于疾病。12 例接受手术联合化疗的患者中，3 例（25%）NED，平均随访 12 年；9 例在 24 个月内死于疾病。所有未行手术治疗的患者平均在 7.5 个月（范围 1-13.7 个月）死亡。