Intermediate results and functional evaluation of limb-salvage surgery for osteosarcoma: an intergroup study in Japan

This is a 2-7-year follow-up report by 21 institutes in Japan concerning limb-salvage surgery for osteosarcoma. Between 1980 and 1985, 248 patients with osteosarcoma were treated, and 105 patients had limb-salvage surgery. The percentage of cases treated by limb-salvage surgery increased with each year. The cumulative survival rate was 71% at 5 years in the limb-salvage group, and in the amputation group it was 46%; this indicates that cases for limb-salvage patients, tumor resection was classified this procedure. In limb-salvage patients, tumor resection was classified according to the surgical margin as intralesional excision, marginal excision, wide-with-marginal resection, wide resection, and radical resection. Wide resection was performed in most cases as the method of choice. Local recurrence was seen in 13 cases (12%). The overall functional evaluations by Enneking's system showed that the number of results rated excellent or good was relatively high in the early follow-up period, but this decreased later on, and cases rated fair or poor increased as the years passed. This was mainly due to postsurgical complications (44 cases). We identify a few problems in the functional evaluation system used now and suggest a reform of the system, so that physical and mental factors would be evaluated separately.     

四肢骨肉瘤保肢治疗的疗效观察

目的评价采用保肢和截肢术结合化疗对Ⅱ期四肢骨肉瘤患者的临床疗效。方法分析1996年1月～2007年7月山西医科大学第二医院骨科收治确诊的68例肢体骨肉瘤患者的临床资料,其中48例行保肢术治疗,20例行截肢术。术前、术后均进行化疗。63例患者获随访,随访时间6～63个月,平均38．5个月。将随访时间达3年的53例骨肉瘤患者分为保肢组和截肢组,以3年生存率、局部复发率以及术后的功能恢复评价为指标进行比较。结果在53例肢体骨肉瘤患者中,保肢组3年生存率为51．4％,局部复发率为8％,按照国际保肢协会推荐使用的肿瘤术后功能重建评价系统平均为26分。截肢组3年生存率为50％,局部复发率为6％,术后功能评分平均为12分。统计学检验比较保肢术和截肢术术后3年生存率无显著性差异（P〉0．05）。结论在术前、术后辅助化疗的基础上,保肢组的3年生存率、局部复发率与截肢组相似,并且保肢术后功能恢复满意,故采用保肢术治疗同期肢体骨肉瘤患者疗效优于截肢术。     

Predictors and Treatment Outcomes of Pediatric Osteosarcoma in Diverse Socioeconomic Backgrounds in Southeast Asia: A Retrospective Multicenter Study

Background: Pediatric osteosarcoma outcomes among developed and developing countries have not been previously compared. Countries in Southeast Asia (SEA) have a wide variety of socioeconomic statuses. A multi-institutional retrospective study was conducted to determine the prognostic factors and outcomes for pediatric osteosarcoma in SEA. Methods: Pediatric patients with osteosarcoma treated between 1998 and 2017 in 4 SEA pediatric oncology centers were studied. Countries were classified using the World Bank Atlas method. Kaplan–Meier method and Cox’s Proportion Hazard Model were applied to estimate survival outcomes and identify prognostic factors. Results: In all, 149 patients with osteosarcoma with a mean age of 12.48±3.66 years were enrolled. The localized to metastatic disease ratio was 1.5:1. The 5-year overall survival (OS) and event-free survival (EFS) were 53.8% and 42%, respectively. Prognostic factors associated with outcomes were country, stage of disease, MTX-containing regimens, and surgery type (p-value <0.05). In patients with localized disease, EFS was superior with limb-salvage surgery (62%) than amputation or rotationplasty (40%) (p-value 0.009). MTX-containing chemotherapies provided higher OS (45.3%) and EFS (37.9%) than non-MTX regimens (12.3% and 10.7%, respectively) among metastatic patients (p-value 0.004 and 0.005, respectively). Metastatic disease was an independent prognostic factor for death but not relapse outcome.  Conclusion: The disease outcomes in SEA were acceptable compared to developed countries. The stage of disease was the only independent prognostic factor. MTX-containing regimens and limb-salvage surgery should be considered where possible.     

Role of surgery in local treatment of Ewing's sarcoma of the extremities in patients undergoing adjuvant and neoadjuvant chemotherapy

Although more and more patients with Ewing's sarcoma of bone (ESB) are being treated by surgery, the relative role of surgery and radiotherapy in the local treatment of this tumor has yet to be determined. Because the outcome of ESB may differ according to the anatomical site of the tumor, results reported in the literature, which generally refer to series with tumors located in all sites, may be selection biased. Therefore, we have retrospectively evaluated patients with ESB exclusively in the extremity and locally treated by surgery or radiotherapy. Two hundred and sixty-eight patients treated at Rizzoli 1979-1996 for non-metastatic ES of the extremities were assessed. Chemotherapy was administered according to four sequentially activated protocols. One hundred and thirty-six patients were treated by surgery, 70 by surgery and radiotherapy, and 60 patients by radiotherapy. Two patients underwent only chemotherapy. The follow-up range was 5-23 years (mean 13 years). One hundred and fifty-two patients remained continuously free of disease, 108 relapsed, 2 died of chemotherapy toxicity and 6 developed a second malignancy. The 5-year event-free survival (EFS) and overall survival (OS) were respectively 62 and 69%. Although patients of all groups were matched for possible risk factors, the rates of 5-year EFS and local control were significantly lower in patients treated with radiotherapy compared to patients treated by surgery or surgery and radiotherapy (48% vs 66%, p=0.002; 80% vs 94%, p=0.0001). Furthermore, in group 3 there were 6 secondary malignancies. Our results indicate that surgery should always be considered in the local treatment of ES of the extremities. Postoperative radiation therapy must be added in case of inadequate surgical margins.     

The oncological outcomes of isolated limb perfusion and neo-adjuvant radiotherapy in soft tissue sarcoma patients - A nationwide multicenter study

IntroductionPatients with locally extensive high-grade extremity soft tissue sarcomas (eSTS) are often presented in multidisciplinary teams to decide between ablative surgery (amputation) or limb-salvage surgery supplemented with either neo-adjuvant radiotherapy (RT) or induction isolated limb perfusion (ILP).In The Netherlands, ILP typically aims to reduce the size of tumors that would otherwise be considered irresectable, whereas neo-adjuvant RT aims mainly at improving local control and reducing morbidity of required marginal margins.This study presents a 15-year nationwide cohort to describe the oncological outcomes of both pre-operative treatment strategies.MethodsAll consecutive patients with locally extensive primary high-grade eSTS surgically treated between 2000 and 2015 at five tertiary sarcoma centers that received neo-adjuvant ILP or RT were included. 169 patients met the inclusion criteria (89 ILP, 80 RT). Median follow-up was 7.3 years.ResultsLimb salvage was achieved in 84% of cases in the ILP group (80% for patients with amputation indication) and 96% of cases in the RT group. 5-Year overall survival was 47% in the ILP group, 69% in the RT group. 5-Year local recurrence rate was 14% in the ILP group, 10% in the RT group. Distant metastasis rate was 55% in the ILP group, 36% in the RT group.ConclusionWe find oncological outcomes and limb salvage rates in line with existing literature for both treatment modalities. Whether the tumor was locally advanced with an indication for induction therapy to prevent amputation or morbid surgery appeared to be the main determinant in choosing between neo-adjuvant ILP or RT.     

92例初治尤文氏肉瘤家族肿瘤综合治疗疗效和生存分析

背景和目的：尤文氏肉瘤家族肿瘤（Ewing’ssarcomafamilyoftumor,ESFF）恶性度高、进展快,其最佳治疗方法目前仍在探讨中。本研究旨在分析ESFF的临床特点和探讨其治疗方法。方法：回顾性分析1995年1月至2008年4月中山大学肿瘤防治中心收治的92例初治ESFF。结果：骨尤文氏肉瘤（Ewing’ssarcomaofbone,ETB）23例,骨外尤文氏肉瘤（extraosseousEwing’Ssarcoma,EOE）21例,外周性原始神经外胚叶瘤（peripheralprimitiveBeuroectodermaltumor,PNET）43例,Askin瘤5例。中位随访时间31．5个月（10～137个月）。局限期综合治疗38例,单一治疗19例,两组3年生存（overallsurvival,OS）率分别为63％、20％,3年无事件生存（events．freesurvival,EFS）率分别为46％、18％,两组问生存差异具有统计学意义（P均〈0．001）。局限期综合治疗患者在全身化疗的基础上加用手术加或不加放疗组远期生存均优于化疗＋放疗组（x2=7．591、9．212,P=0．006、0．002）。CAV／IE交替方案对局限期接受综合治疗患者延长了无事件生存期,但其总生存时间差异无统计学意义（X2=6．950、3．530,P=0．008、0．06）。多因素生存分析显示治疗模式以及疗效是独立的预后因素。结论：综合治疗能明显改善局限期ESFT患者疗效和生存．手术加化疗加或不加放疗的治疗模式在疗效和生存方面优于化疗加放疗治疗模式。治疗模式和近期疗效是独立的预后因素。     

Pattern of relapse in patients with osteosarcoma of the extremities treated with neoadjuvant chemotherapy

570 patients with osteosarcoma of the extremities were treated with five different protocols of neoadjuvant chemotherapy at Rizzoli Institute between 1983 and 1995. Surgery consisted of limb salvage in 83% rotation plasty in 5% and amputation in 12%. The 5-year event-free survival (EFS) was 60% which varied according to the protocol followed, ranging from 47.6% to 66.4%. 234 patients relapsed. The pattern of relapse was analysed. The mean relapse time was 23.8 months (range: 2-96). The first site of systemic relapse was the lung in 88% (32% of these had less than three pulmonary metastases and 68% three or more), bone in 9%, lung and bone in 2% and other sites in 3%. The relapse time and the number of pulmonary metastases were strictly correlated with the efficacy of the protocol of chemotherapy used. Patients treated with the three protocols that gave a 5-year EFS of more than 60% relapsed later and had fewer pulmonary lesions than patients treated with the two protocols that gave a 5-year EFS of 47.6% and 52.5%. The rate of local recurrence was relatively low (6%). This was not correlated with the protocol or the type of surgery used: limb salvage (6.4%), rotation plasty or amputation (4.1%). However, the rate of local recurrence was very high (21.9%) in the few patients (7%) that had less than wide surgical margins. We conclude that for patients with osteosarcoma of the extremities treated with neoadjuvant chemotherapy: (a) the pattern of systemic relapse changes according to the efficacy of the protocol of chemotherapy used. This should be always considered when evaluating the preliminary results of new studies as well as in defining the time of follow-up; (b) limb salvage procedures are safe and do not jeopardise the outcome of the patient, provided that wide surgical margins are achieved.     

Extraskeletal Ewing Sarcomas in Late Adolescence and Adults: A Study of 37 Patients

Background: Extraskeletal Ewing sarcoma (EES)/primitive neuroectodermal tumours (PNET) are raresoft tissue sarcomas. Prognostic factors and optimal therapy are still unconfirmed. Materials and Methods:We performed a retrospective analysis on patients to explore the clinic characteristics and prognostic factorsof this rare disease. A total of 37 patients older than 15 years referred to our institute from Jan., 2002 to Jan.,2012 were reviewed. The characteristics, treatment and outcome were collected and analyzed. Results: Themedian age was 28 years (range 15-65); the median size of primary tumours was 8.2 cm (range 2–19). Sixteenpatients (43%) had metastatic disease at the initial presentation. Wide surgical margins were achieved in 14 cases(38%). Anthracycline or platinum-based chemotherapy was performed on 29 patients (74%). Radiotherapy wasdelivered in 13 (35%). At a median follow-up visit of 24 months (range 2–81), the media event-free survival (EFS)and overall survival (OS) were 15.8 and 30.2 months, respectively. The 3-year EFS and OS rates were 24% and43%, respectively. Metastases at presentation and wide surgical margins were significantly associated with OSand EFS. Tumour size was significantly associated with OS but not EFS. There were no significant differencesbetween anthracycline and platinum based chemotherapy regarding EFS and OS. Conclusions: EES/PNET is amalignant tumour with high recurrence and frequent distant metastasis. Multimodality therapy featuring widesurgical margins, aggressive chemotherapy and adjuvant local radiotherapy is necessary for this rare disease.Platinum-based chemotherapy can be used as an adjuvant therapy.     

Navigation-assisted pelvic resections and reconstructions for periacetabular chondrosarcomas

ObjectivesSurvival in patients with chondrosarcomas has not improved over 40 years. Although emerging evidence has documented the efficacy of navigation-assisted surgery, the prognostic significance in chondrosarcomas remains unknown. We aimed to assess the clinical benefit of navigation-assisted surgery for pelvic chondrosarcomas involving the peri-acetabulum.MethodsWe studied 50 patients who underwent limb-sparing surgery for periacetabular chondrosarcomas performed with navigation (n = 13) without it (n = 37) at a referral musculoskeletal oncology centre between 2000 and 2015.ResultsThe intralesional resection rates in the navigated and non-navigated groups were 8% (n = 1) and 19% (n = 7), respectively; all bone resection margins were clear in the navigated group. The 5-year cumulative incidence of local recurrence was 23% and 56% in the navigated and non-navigated groups, respectively (p = 0.035). There were no intra-operative complications related to use of navigation. There was a trend toward better functional outcomes in the navigated group (mean MSTS score, 67%) than the non-navigated group (mean MSTS score, 60%; p = 0.412). At a mean follow-up of 63 months, the 5-year disease-specific survival was 76% and 53% in the navigated and non-navigated group, respectively (p = 0.085), whilst the 5-year progression-free survival was 62% and 28% in the navigated and non-navigated group, respectively (p = 0.032).ConclusionThis study confirmed improved local control and progression-free survival with the use of computer navigation in patients with limb-salvage surgery for periacetabular chondrosarcomas, although the advancement in other treatment modalities is required for improvement of disease-specific survival.     

Outcome after disarticulation of the hip for sarcomas.

AIMS: To review the oncological and functional outcome in 80 patients who underwent disarticulation of the hip as part of their treatment. METHODS: Eighty patients had disarticulation, of whom 46 had a bone sarcoma and 34 a soft tissue sarcoma. In 42 patients the operation was done as the first definitive surgical procedure for that patient. In 38 patients the disarticulation followed local recurrence after unsuccessful limb salvage, three of these patients had palliative amputations already having metastatic disease. All patients had adjuvant therapy when appropriate. RESULTS: The overall survival of the patients following the amputation was 56% at 1 year, 39% at 2 years, 27% at 5 years and 21% at 10 years. The 5-year survival of patients having the amputation as a primary procedure was 32%, for those with local recurrence it was 25% whilst for those with a palliative amputation it was nil. Local recurrence developed in 10 patients following the amputation, and was related to close margins of excision; all of these patients subsequently died. Function was on the whole poor, with only one surviving patient regularly using an artificial limb. CONCLUSION: Disarticulation of the hip remains a disabling procedure usually carried out for high grade sarcomas with extensive involvement of bone and soft tissues in the thigh. Long term survival is possible if wide margins of excision can be achieved.     

Prognosis of osteosarcoma treated by limb-salvage surgery: the ten-year intergroup study in Japan.

We report the prognoses for 107 patients treated by limb-salvage surgery and 147 treated by amputation or disarticulation, during the period 1980-1985, at 22 university hospitals, cancer centers and national hospitals in Japan. In this multi-institute non-randomized study, the five-year cumulative survival rate was 70% for the former group and 49% for the latter. The prognosis was more favorable among patients who responded to preoperative chemotherapy than among those who did not. Limb-salvage surgery was feasible for most of the surgical stage IIB patients with small extraosseous tumor extensions who had responded to preoperative chemotherapy. Local tumor recurrences were seen in 15 (14%) of the 107 patients in the limb-salvage surgery group, nine of whom died of the metastasis. While limb-salvage surgery is being increasingly used in Japan, present indications show the procedure not to reduce survival rate for osteosarcoma patients. The survival rate of patients with wide-with-marginal margins was similar to that of patients with wide margins. Taking into account the long-term survival of osteosarcoma patients, limb-salvage surgery can be recommended when the osteosarcoma responds to preoperative chemotherapy.     

Recurrent retroperitoneal sarcomas: Clinical outcomes of surgical treatment and prognostic factors

Background and purposeLocoregional recurrence after resection of primary retroperitoneal sarcoma (RPS) is a challenging therapeutic issue. The objective of this study was to identify clinicopathological factors predictive of overall survival (OS) and disease specific survival (DSS) after reoperation for recurrent RPS.Patients and methodsWe retrospectively collected data from the medical records of 800 patients who underwent resection for sarcoma at our Institution, from 1983 to 2015. Among these patients, 120 were treated for retroperitoneal sarcoma and 55 had a locoregional recurrence (LR). Four of them did not undergo surgery and thus were excluded from this study leaving 51 cases available for data analysis. Univariate and multivariate survival analyses were performed to identify prognostic factors.ResultsMedian overall survival was 33 months. The 1-year, 3-year and 5-year OS rates were 75.5%, 47.1% and 31.6% respectively. Multivariate Cox regression analysis suggested that extension of surgery (P = 0.026), surgical margin status (P = 0.015) and histological grade of recurrent tumor (P = 0.047) were independent prognostic factors for OS. Median DSS was 48 months. The 1-year, 3-year and 5-year DSS rates were 79.2%, 53.1% and 40.9%, respectively. At multivariate analysis, predictors of DSS were extension of surgery (P = 0.004), margin status (P = 0.011), histological grade of recurrent tumor (P = 0.008), and disease free interval (DFI) (P = 0.020). As regards histological subtype of recurrent RPS, at univariate analysis, well-differentiated liposarcoma (WDLS) was associated with better OS and DSS (P = 0.052 and P = 0.016 respectively) compared to dedifferentiated liposarcoma (DDLS).ConclusionsAccording to our findings, surgery is more beneficial in patients with low-grade sarcoma, WDLS and long DFI. The achievement of clear resection margins, rather than performing a multivisceral resection, appears to be a key factor to improve OS and DSS.     

European intergroup studies (MMT4-89 and MMT4-91) on childhood metastatic rhabdomyosarcoma: final results and analysis of prognostic factors.

PURPOSE: Final results are presented from two consecutive European studies for patients with metastatic rhabdomyosarcoma (RMS) to identify prognostic variables and determine the value of high-dose chemotherapy (HDCT) in complete remission. PATIENTS AND METHODS: A total of 174 patients aged 3 months to 18 years participated. From 1989 to 1991, patients received four cycles of intensive multiagent chemotherapy. From 1991 to 1995, patients achieving complete remission received consolidation with HDCT. All received local therapy (surgery, radiation therapy) according to response. RESULTS: At a median follow-up of 8 years, 5-year overall survival (OS) and event-free survival (EFS) for the whole group were 24% and 20%, respectively. No statistical difference was found between HDCT and standard chemotherapy (5-year OS, 36% v 27%; EFS 29% v 23%). Univariate analysis identified primary tumor in parameningeal, extremity, or other sites; age younger than 1 year and older than 10 years; bone or bone marrow metastases; multiple metastases; and multiple sites of metastases as unfavorable prognostic factors for OS and EFS. Multivariate analysis identified unfavorable site, bone or bone marrow involvement, and unfavorable age as independently unfavorable factors. Two subgroups were identified. Those with fewer than two unfavorable factors had 5-year EFS and OS of 40% and 47%, respectively. Patients with > or = two unfavorable factors had 5-year EFS and OS of 7.5% and 9%, respectively. CONCLUSION: A minority of patients with metastatic RMS have better survival than overall results for this population suggest. Those in the highest risk group have such poor survival that they are candidates for first-line novel therapies. There is no evidence that consolidation with HDCT improves outcome.     

Neoadjuvant Chemotherapy for Osseous Malignant Fibrous Histiocytoma of the Extremity: Results in 18 Cases and Comparison with 112 Contemporary Osteosarcoma Patients Treated with the Same Chemotherapy Regimen

Abstract

Eigthteen patients with high grade malignant fibrous histiocytoma (MFH) of bone and 112 patients with high grade osteosarcoma (OS) of the extremity were treated with neoadjuvant chemotherapy comprised of methotrexate, cisplatinum, doxorubicin and ifosfamide. For the 18 patients with MFH, surgery involved amputation in 2 cases and limb salvage in 16 (89%); the 112 osteosarcoma patients had amputation in 8 cases and limb salvage procedure in 104 cases (93%). The rate of good histologic response to preoperative chemotherapy (90% or more tumor necrosis) was significantly higher in patients with osteosarcoma than in patients with MFH (74% vs 28%; p<0.003). However, at a median follow-up of 38 months (range 25-61), the 3-year event-free survival (EFS) did not differ in the two groups (MFH 77.8%, OS 70.5%; p=ns). In patients with MFH, no local recurrences were registered, whereas in the osteosarcoma group there were 6 local relapses (5.%).

The effectiveness of neoadjuvant chemotherapy in the treatment of osteosarcoma has been assessed during the last 15 years. The results of the present study seem to indicate that, in spite of a usually poor histologic response to preoperative treatment, neoadjuvant chemotherapy is very effective also in MFH of bone.     

Carboplatin and Doxorubicin in Treatment of PediatricOsteosarcoma: A 9-year Single Institute Experience in theNorthern Region of Thailand

Background: Osteosarcoma is the most common primary bone tumor in childhood and adolescence.Carboplatin, a platinum-derived agent, is used as neoadjuvant chemotherapy for pediatric osteosarcoma becauseof its anti-tumor activity and had low toxicity as compared to cisplatin. Objective: To determine demographic data,prognostic factors and outcome of childhood osteosarcoma treated with a carboplatin-based chemotherapeuticprotocol at Chiang Mai University. Method: A retrospective analysis was conducted on 34 osteosarcoma patientsaged less than 18 years and treated between 2003 and 2011. Results: Overall limb-salvage and amputation rateswere 23.5% and 70.6%, respectively. With the mean follow-up time of 29.5 months (1.5-108.9), the Kaplan-Meieranalysis for 3-year disease-free survival (DFS) and 3-year overall survival (OS) were 20.2±7.7% and 47.1±9.5%respectively. Patients who had initial pulmonary metastasis were at significantly greater risk for developingrecurrence (p=0.02, OR=7; 1.2-40.1) and had a tendency to have lower 3-year OS compared to those withoutinitial pulmonary metastasis (28.1±13%, 63.1±12.3%, respectively, p=0.202). On univariate analysis, age at diagnosis >14 years and patients who were declined surgery were significantly associated with lower 3-year OS (p=0.008 and <0.05, respectively). However, age at diagnosis, sex, tumor size and histological subtypes were not found to significantly affect recurrence or survival. Conclusions: In our study, the survival rate was far lower than those reported from developed countries. These might indicate the ineffectiveness of carboplatin in combination with doxorubicin as frontline treatment of pediatric osteosarcoma, especially in those with initial pulmonary metastasis.Refinement in risk and treatment stratification and dose intensification for pediatric osteosarcoma constitutes a future challenge to improve outcomes, especially in metastatic patients who may need a more intensive regimen.     

Prognostic variables for the selection of patients with operable soft tissue sarcomas to be considered in adjuvant chemotherapy trials.

From 1975 to 1988, 144 patients naive of treatment, with non-metastatic soft tissue sarcoma were treated at Fondation Bergonié by surgery, followed by radiotherapy and without chemotherapy. An analysis of prognostic variables was done on this population to determine patients for whom an adjuvant chemotherapy would be relevant. Prognostic variables in overall survival (OS), metastasis-free survival (MFS), disease-free and local free recurrence survivals were analysed by univariate and multivariate analysis. In multivariate analysis using Cox's model, only tumour depth and tumour grade were significant with the MFS end point, while tumour depth, tumour grade and tumour site were significant when considering OS. A predictive stratification for patients is proposed: a favourable prognostic group with grade 1 tumour or superficial, grade 2 tumour (5-year OS: 97.8%; 5-year MFS: 100%); an intermediate prognostic group with deep, grade 2 tumour or superficial, grade 3 tumour (5-year OS: 58.8%; 5-year MFS: 48.1%); and finally a poor prognostic group with deep, grade 3 tumour (5-year OS: 31.7%; 5-year MFS: 34.1%). Patients in the intermediate and poor prognostic groups who present a high metastatic risk are to be considered for adjuvant chemotherapy trials.     

Local recurrence and local control of non-metastatic osteosarcoma of the extremities: a 27-year experience in a single institution

BACKGROUND: Indications and contraindications for limb salvage versus amputation for local treatment of osteosarcoma of the extremity are still controversial. PATIENTS AND METHODS: Patients (1,126) with non-metastatic osteosarcoma of the extremity, treated in a single institution between 1972 and 1999 with different protocols of adjuvant and neoadjuvant chemotherapy were evaluated to establish factors that could influence local recurrence (LR) and outcome. RESULTS: The 5-year event-free survival and overall survival were 55% and 66%. At a follow-up ranging between 5.5 and 32.5 years (mean18.6 years) of the 1,126 evaluated patients, 607 (54%) remained continuously disease-free and 519 relapsed. LR developed in 61 patients (5.4%) after a median time of 2.3 years (0.2-17). For this group of patients the 5-year post-relapse event-free survival and overall survival from the last relapse were, respectively, 11.4% and 16.4%. At the multivariate analyses only surgical margins and histologic response to preoperative treatment resulted to be independent prognostic factors for LR. CONCLUSION: Considering the risk of LR after surgery with inadequate surgical margins and poor prognosis of LR in osteosarcoma, limb salvage procedures should be performed only when adequate margins surgical margins can be achieved. In case of inadequate margins, an immediate amputation should be considered.     

软组织肉瘤动脉灌注新辅助化疗后的保肢治疗

目的探讨术前动脉内灌注化疗对软组织肉瘤的疗效及在保肢治疗中的意义。方法28例肢体软组织肉瘤,其中恶性纤维组织细胞瘤18例,滑膜肉瘤3例,脂肪肉瘤6例,原始神经外胚层肿瘤（PNET）1例。术前均给予选择性肿瘤供血动脉灌注化疗2-3周期,化疗药物为阿霉素／顺铂和异环磷酰胺。化疗后均行保肢手术治疗。结果28例术前化疗后均有疼痛缓解、皮温降低、肿胀减轻、瘤体不同程度缩小、边缘变清楚及关节活动度增加。化疗后均行肿瘤广泛切除术,术中获得良好的外科切除界限。随访6-46个月,平均25个月。2例死亡,1例局部复发行截肢术。术后12个月后采用MSTS93评分系统评分为24-29分,平均为27分。28例3年生存率为92.9％,初次保肢率100％,最终保肢率89.3％。结论肢体软组织肉瘤术前动脉灌注新辅助化疗是一种有效的保肢治疗方法。     

72例标危型髓母细胞瘤放疗剂量对生存的影响

目的 回顾分析标危型髓母细胞瘤采用全脑全脊髓放疗剂量≤24 Gy和>24 Gy对预后的影响。方法 标危型髓母细胞瘤定义为年龄>3岁、未发生转移、肿瘤全切或近全切(残留≤1.5 cm³)。2003—2013年共入组72例初治儿童、青少年标危型髓母细胞瘤患者。患者术后接受全脑全脊髓+局部瘤床放疗和8个疗程辅助化疗,化疗方案为顺铂、司莫司汀或卡莫司汀联合长春新碱。按放疗剂量≤24 Gy和>24 Gy分为A、B组(20、52例),比较两组患者复发率和生存率。Kaplan-Meier法计算复发率和生存率并Logrank法检验组间差异。结果 A组接受全脑全脊髓放疗19.2~24.0 Gy,B组接受全脑全脊髓放疗24.1~30.6 Gy。放疗后66例(92%)患者完成全部辅助化疗。共11例患者复发。随访满3年患者48例,其中复发11例,死亡7例。全组3年EFS率为83%,3年OS率为86%。A组和B组患者3年EFS率分别为84%和83%(P=0.609), 3年OS率分别为85%和87%(P=0.963)。结论 标危型髓母细胞瘤经规范综合治疗效果较好,其中全脑全脊髓放疗剂量减少至19.2~24.0 Gy未影响疗效。     

基于儿童肾母细胞瘤-2016诊疗规范的单中心疗效分析(附38例)

目的:应用儿童肾母细胞瘤(Wilms tumor,WT)-2016诊疗规范对单中心WT患儿的临床疗效进行分析.方法:回顾性分析2017年02月至2020年03月,采用CCCG-WT-2016方案治疗肾母细胞瘤患儿的临床资料,分析其临床特点及治疗转归,并采用Kaplan-Meier法分析3年无事件生存(event-fre...