Primary Chemotherapy and Delayed Surgery (Neoadjuvant Chemotherapy) for Telangiectatic Osteogenic Sarcoma of the Extremities

Summary

Twelve patients with telangiectatic osteogenic sarcoma (TOS) of the extremities were treated with neoadjuvant chemotherapy, according to two different protocols.

Preoperatively the patients received high-dose methotrexate(HD-MTX)/cisplatinum(CPD) or HD-MTX/CPD/adriamycin(ADM). CPD was delivered intra-arteriously, the other drugs intravenously. Limb salvage surgery was performed in eight instances and four patients underwent amputation. Post operative chemotherapy was tailored according to the grade of necrosis determined by preoperative treatment on the primary tumor. In ten cases (83%) the grade of necrosis resulted higher than 95%.

The mean length of follow-up was 3.5 years with a range of 18 to 72 months. Ten patients (83%) reimained continuously disease-free, while two patients developed lung metastases and died of uncontrolled disease. No local recurrences were observed.

These results are better than those observed in 167 contemporary cases of conventional osteosarcoma treated with the same protocols. This study confirms that TOS is not always a lethal tumor as suggested by prior reports. Employing neoadjuvant chemotherapy a high percentage of patients with TOS can be cured and in most of them, limb sparing surgery is possible and safe.     

Neoadjuvant chemotherapy in malignant fibrous histiocytoma of bone and in osteosarcoma located in the extremities: Analogies and differences between the two tumors

Background: Malignant fibrous histiocytoma (MFH) is a rare bone tumor usually treated like osteosarcoma. Studies on analogies and differences between the two tumors have seldom been reported.Patients and methods: Between March 1982 and December 1994, 51 patients with high-grade MFH of bone and 390 with high-grade osteosarcoma were treated with the same regimen of neoadjuvant chemotherapy. All of the tumors in both groups were located in the limbs. Preoperative chemotherapy was performed according to three different, successively activated, regimens consisting of MTX/CDP intraarterially, MTX/CDP/ADM, and MTX/CDP/ADM//IFO.Results: The rate of limb salvage was the same in both the MFH (92%) and osteosarcoma (85%) patients. MFH showed a statistically significantly lower rate of good histologic response, 90% or more tumor necrosis (27% vs. 67%, P = 0.00001) for all three regimens. Despite this low chemosensitivity, the disease-free survivals of the two neoplasms were similar (67% vs. 65%).Conclusions: In terms of histologic response to primary chemotherapy, MFH has a lower chemosensitivity than osteosarcoma. Nevertheless, the two tumors have similar prognoses when treated with chemotherapy regimens based on MTX, CDP, ADM and IFO.     

Bone primary sarcomas undergone unplanned intralesional procedures - the possibility of limb salvage and their oncologic results

BACKGROUND: It is quite rare but some primary sarcomas of the bone can be misdiagnosed as benign and be treated using intralesional procedures. An unplanned surgical excision occurs when tumors are removed without the appropriate preoperative evaluation and consideration for the need to obtain tumor-free margins. Residual tumor tissue as a result of unplanned excision of soft tissue sarcoma is a risk factor for local recurrence. METHODS: Twenty-five patients, undergone unplanned intralesional procedures of lower extremities, were enrolled in this study. There were 22 (88%) cases of osteosarcomas, 2 (8%) MFH of bone and 1 (4%) adamantinoma. Twenty-two (88%) cases had a limb salvage and among them, 4 (18%) cases had local recurrences. Three cases (12%) had amputation without any local event. RESULTS: Lung metastasis developed in 6 (24%) cases. The CDF 5 year survival for 22 osteosarcoma cases was 65%(confidence interval: 52-82%). DISCUSSION: Despite the high recurrence rate, the CDF survival of osteosarcoma cases was comparable to the primary one. Limb salvage procedures are worthwhile in cases whose initial radiographic findings simulate benign lesions, showing favorable response to neoadjuvant chemotherapy. The relative contraindication to the limb salvage is cases with a history of a pathologic fracture and extensive operative fixation.     

Primary chemotherapy and delayed surgery for malignant fibrous histiocytoma of bone in the extremity

Between March 1983 and September 1988, 22 patients with non-metastatic malignant fibrous histiocytoma MFH of bone of the extremities were treated with two regimens of neo-adjuvant chemotherapy successively activated. Preoperatively, the patients received moderate doses of methotrexate and cisplatinum-Regimen 1- or high dose methotrexate, cisplatinum and adriamycin-Regimen 2. Cisplatinum was delivered intraarteriously, the other drugs intravenously. Limb salvage surgery was performed in 20 patients, and 2 patients were amputated. The surgical margins were adequate (radical or wide) in 18 cases and inadequate (marginal) in 4. The histologic response to chemotherapy was good (90% or more tumor necrosis) in 8 patients. In both regimens postoperative chemotherapy was tailored according to the grade of necrosis determined by preoperative treatment on the primary tumor. At an average follow-up of 40 months (15-70), 15 patients (68%) remained continuously disease-free and 7 relapsed with metastases. No local recurrences were observed. Regimen 2 was slightly more effective than Regimen 1 in terms of good histologic response (5/10 vs 1/12) and continuous disease-free survival (8/10 vs 7/127). The results demonstrate that, as in osteosarcoma, in non-metastatic malignant fibrous histiocytoma of bone in the extremities a high percentage of patients can be cured with neoadjuvant chemotherapy and that in most of them limb sparing surgery is possible and safe.     

Neoadjuvant Chemotherapy for High Grade Osteosarcoma of the Extremities: Long-Term Results for Patients Treated According to the Rizzoli IOR/OS-3b Protocol

Abstract

The results of the Rizzoli IOR/OS-3b neoadjuvant protocol for the treatment of osteosarcoma of the extremity are reported. Preoperative chemotherapy consisted of two cycles of high-dose methotrexate (HDMTX i.v.), followed by a combination of cisplatin (CDP i.a.)/ doxorubicin (ADM i.v.). Postoperatively all patients, regardless of the histologic response, received 3 more cycles of MTX, CDP/ADM alternated with 3 cycles of ifosfamide. In the study performed between January and December 1992 43 patients were enrolled and limb salvage was performed in 39 of them (91%). The histologic response to chemotherapy was good (90% or more tumor necrosis) in 24 patients (56%) and poor (less than 90% tumor necrosis) in 19 (44%). With a minimum follow-up of 7 years, 23 pts (53%) remained continuously free of disease, 19 relapsed and one died due to unrelated cause. In spite of the high number of limb salvages performed, only 2 local recurrences were registered. The 7-year event-free survival and overall survival were, respectively, 53% and 68%. The hematopoietic and extrahematopoietic toxicity experienced by the patients during the entire treatment was relatively mild.

These long-term results confirm that, with neoadjuvant chemotherapy, it is possible to cure more than 60% of patients with osteosarcoma of the extremities, avoiding amputation in most of them. These results, however, are no better than those achieved in our previous study IOR/OS-3a, in which only poor responder patients received ifosfamide during the postoperative treatment.     

骨肉瘤新辅助化疗,保肢手术疗效分析

报告骨肉瘤新辅助化疗，保肢手术的初步成绩。收治５６例四肢骨肉瘤。其中３５例接受了不同的化疗方案，在此基础上，２２例接受了肿瘤广泛边缘切除，假体置换手术。结果显示：化疗组的３年和５年生存率分别达５４％和４０％，优于非化疗组；新辅助化疗疗效优于辅助化疗；保肢术和根治性手术比较，生存率无明显差别。作者认为：在有效新辅助化疗基础上保肢手术是四肢骨肉瘤较为理想的治疗方法。     

Chemotherapy for nonmetastatic osteogenic sarcoma: the Memorial Sloan-Kettering experience.

PURPOSE: Adjuvant chemotherapy improves disease-free survival (DFS) for patients with osteogenic sarcoma (OS). We reviewed our experience with OS to determine prognostic factors, the role of preoperative chemotherapy and subsequent histologic response, and the role of salvage chemotherapy after poor initial response. METHODS: From 1975 to 1984, we saw 279 patients with previously untreated OS without metastasis. All patients received intensive chemotherapy and underwent surgical resection of primary tumor. Chemotherapy included high-dose methotrexate; Adriamycin (doxorubicin; Adria Laboratories, Columbus, OH); and bleomycin, cyclophosphamide, and dactinomycin (BCD). Selected patients also received cisplatin. RESULTS: DFS was not affected by use of preoperative chemotherapy versus immediate surgery, by use of limb-sparing surgery versus amputation, age, sex, or dose intensity of chemotherapy. DFS did correlate with serum lactate dehydrogenase (LDH), alkaline phosphatase, primary tumor site, race, and histologic response to preoperative chemotherapy. There was no difference in DFS for patients with a poor histologic response who did or did not receive cisplatin, although patients who did receive cisplatin had a longer time to relapse. The 5-year DFS was 76% for patients aged less than or equal to 21 years who had extremity primary tumor and were treated with the T10 protocol. CONCLUSIONS: Intensive chemotherapy can achieve DFS for a high proportion of patients with OS. Although it is a powerful predictor of DFS, histologic response to preoperative chemotherapy cannot be assessed at diagnosis. We have not shown an ability to salvage patients with an unfavorable response. We need to increase the proportion of patients with a favorable response, identify the patients who will have an unfavorable response, and develop novel treatments to salvage poor responders.     

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.     

新辅助化疗加保肢手术治疗肢体骨肉瘤

目的评价新辅助化疗加保肢手术治疗肢体骨肉瘤的临床疗效。方法选择1994年6月至2002年6月新辅助化疗加保肢手术治疗肢体骨肉瘤17例。达到广泛切除者11例，仅达边缘切除者6例；15例接受过术前化疗的病例切除标本均做多病理切片的坏死率评估。结果17例中无瘤生存超过5年的共有9例(52．9％)，其中截肢术2例(17．6％)，肢体功能评分结果，优7例、良2例、可3例、差5例，优良率为69．9％。结论新辅助化疗加保肢手术是治疗肢体骨肉瘤患者理想的治疗方法，术前有效化疗是保肢术成功的关键。     

Primary chemotherapy and delayed surgery (neoadjuvant chemotherapy) for telangiectatic osteogenic sarcoma of the extremities

Twelve patients with telangiectatic osteogenic sarcoma (TOS) of the extremities were treated with neoadjuvant chemotherapy, according to two different protocols. Preoperatively the patients received high-dose methotrexate(HD-MTX)/cisplatinum(CPD) or HD-MTX/CPD/adriamycin(ADM). CPD was delivered intra-arterially, the other drugs intravenously. Limb salvage surgery was performed in eight instances and four patients underwent amputation. Post operative chemotherapy was tailored according to the grade of necrosis determined by preoperative treatment on the primary tumor. In ten cases (83%) the grade of necrosis resulted higher than 95%. The mean length of follow-up was 3.5 years with a range of 18 to 72 months. Ten patients (83%) remained continuously disease-free, while two patients developed lung metastases and died of uncontrolled disease. No local recurrences were observed. These results are better than those observed in 167 contemporary cases of conventional osteosarcoma treated with the same protocols. This study confirms that TOS is not always a lethal tumor as suggested by prior reports. Employing neoadjuvant chemotherapy a high percentage of patients with TOS can be cured and in most of them, limb sparing surgery is possible and safe.     

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.     

Adjuvant and neoadjuvant chemotherapy for osteosarcoma of the extremities: 27 year experience at Rizzoli Institute, Italy

Around 1148 patients with non-metastatic osteosarcoma of the extremity were treated in a single institution between 1972 and 1999 with 4 different protocol of adjuvant and 7 different protocols of neoadjuvant chemotherapy. The rate of limb salvage increased from 20% to 71%. The 5-year event-free survival (EFS) and overall survival (OS) were 57% and 66%, respectively. The 10-year EFS and OS were 52% and 57%, respectively, and the results significantly correlated with serum alkaline phosphatase levels; the type of chemotherapy (adjuvant vs neoadjuvant); and with histologic response to pre-operative treatment. Aggressive chemotherapy and surgery could cure about the 60% of patients with osteosarcoma of the extremity. However, since local or systemic relapses, myocardiopathies and a second malignancy are possible even 5 or more years since the beginning of treatment, a long-term follow-up is recommended.     

新辅助化疗结合保肢手术治疗肢体骨肉瘤

目的：评价新辅助化疗结合保肢手术治疗肢体骨肉瘤的临床疗效。方法：选择1995年1月-2003年1月新辅助化疗结合保肢手术治疗的肢体骨肉瘤31例。广泛切除22例，边缘切除9例。所有病例切除标本均做多病理切片的坏死率评估结果：随访2～8年，31例中无瘤生存超过5年19例（61.3％），肿瘤局部复发4例（12.9％）。肢体功能评估优良率为67．7％：结论：新辅助化疗结合保肢手术是治疗肢体骨肉瘤理想的治疗方法，术前有效化疗是保肢术成功的关键。     

Changing Pattern of Relapse in Osteosarcoma of the Extremities Treated with Adjuvant and Neoadjuvant Chemotherapy

Abstract

In 551 patients with osteosarcoma of the extremities treated between 1980 and 1991 in our Institution with surgery only (35 cases), surgery combined with adjuvant chemotherapy (147 cases) or neoadjuvant chemotherapy (369 cases) the relapse patterns were analyzed. Adjuvant chemotherapy was performed according to 2 different protocols and neoadjuvant chemotherapy according to 3 different protocols successively activated.

In the 252 patients who relapsed, the interval between initial treatment and first relapse was significantly longer in the group treated with adjuvant and neoadjuvant chemotherapy (18.1 and 21.3 mo) than in the group treated with surgery only (5.4 mo). For patients treated with neoadjuvant chemotherapy, a longer interval was seen in the most effective regimen of neoadjuvant chemotherapy (25 mo). No significant differences were seen among the 3 groups, according to the site of first metastasis, although in patients treated with the most effective neoadjuvant regimen there was a higher incidence of bone metastasis.

In patients who relapsed with pulmonary metastases the average number of nodules seen by standard X-rays, as well as CT scans, was significantly higher in patients treated with surgery only (3.6) than in patients treated with adjuvant or neoadjuvant chemotherapy (2.5 and 2.6 nodules).

We conclude that these changes in metastatic pattern in patients treated with adjuvant and neoadjuvant chemotherapy are important, because they may encourage the use of salvage therapy with thoracotomy in a larger number of patients.

Prolongation of time relapsed after more effective regimens of adjuvant and neoadjuvant chemotherapy should be considered when evaluating the preliminary results of new chemotherapy protocols.     

新辅助化疗联合保肢手术治疗骨肉瘤的近期疗效观察

目的探讨新辅助化疗联合保肢手术治疗骨肉瘤的临床疗效。方法 40例四肢骨肉瘤患者随机分为试验组和对照组,试验组患者采用新辅助化疗联合保肢手术治疗,对照组患者采用截肢手术治疗。两组患者手术后均给予化疗,同时对比分析两组患者的临床疗效。结果试验组和对照组分别有3例和11例患者出现复发和(或)转移(P<0.05)。两组患者的术后1年和2年生存率差异无统计学意义,但试验组患者的术后3年生存率高于对照组(P<0.05)。两组患者的临床疗效优良率比较,试验组优于对照组(P<0.05)。试验组患者肢体功能评分为(23.5±4.6)分,而对照组为(11.8±3.1)分,两组比较,差异有统计学意义(P<0.05)。结论新辅助化疗联合保肢手术治疗骨肉瘤能够保证患者的生存率,其局部复发率和转移率与截肢手术一致,同时保存了患者肢体,提高了患者的生活质量,值得临床推广。     

新辅助化疗结合保肢手术治疗骨肉瘤的临床观察

目的 探讨新辅助化疗结合保肢手术治疗骨肉瘤的临床疗效.方法 对23例四肢骨肉瘤患者实施保肢手术,并分别于术前、术后进行辅助化疗.患者出院后加强随访,并对恢复情况进行功能评定.结果 随访期内患者无瘤生存率为69.57%(16/23),术后功能评定优9例、良7例,优良率为69.57%(16/23).结论 新辅助化疗结合保肢手术治疗骨肉瘤患者疗效较好,值得临床推广应用.     

膝关节周围恶性骨肿瘤的保肢治疗（附48例随访报告）

目的总结膝关节周围恶性骨肿瘤的保肢治疗经验。方法回顾分析1999年2月～2007年2月48例接受保肢治疗的膝关节周围恶性骨肿瘤患者的临床资料。男30例,女18例。平均年龄27.3岁（11～67岁）。病理证实：高恶性肿瘤32例（A组）,包括骨肉瘤23例,恶性纤维组织细胞瘤5例,尤文肉瘤2例,恶性淋巴瘤2例;低度恶性肿瘤16例（B组）,包括侵袭性骨母细胞瘤1例,骨巨细胞瘤15例。手术方式包括：瘤段切除假体置换术或灭活再植术、异体骨移植术、异体骨复合假体移植术、病灶刮除充填术保肢。A组术前、术后给予化疗。保留肢体功能按Enneking肌肉骨骼肿瘤外科治疗重建后功能评估标准评估。结果平均随访3.2年（0.5～8年）。A组中因局部肿瘤复发、感染等并发症截肢11例（34.4%）,死亡13例,存活19例,3年存活率59.3%（19/32）。B组中因局部肿瘤复发和感染截肢2例（12.5%,2/16）,死亡1例。肢体功能优良率A组71%,B组81%。结论膝关节周围恶性骨肿瘤保肢应掌握个体化原则,假体置换术及异体骨复合假体移植术保肢功能最佳,高恶性肿瘤患者若无法承受强力的辅助化疗则不宜行保肢术。     

Long-term outcome for patients with nonmetastatic osteosarcoma of the extremity treated at the istituto ortopedico rizzoli according to the istituto ortopedico rizzoli/osteosarcoma-2 protocol: an updated report.

PURPOSE: To provide an estimate of long-term prognosis for patients with osteosarcoma of the extremity treated in a single institution with neoadjuvant chemotherapy and observed for at least 10 years. PATIENTS AND METHODS: Patients with nonmetastatic osteosarcoma of the extremity were preoperatively treated with high-dose methotrexate, cisplatin, and doxorubicin (ADM). Postoperatively, good responders (90% or more tumor necrosis) received the same three drugs used before surgery, whereas poor responders (less than 90% tumor necrosis) received ifosfamide and etoposide in addition to those three drugs. RESULTS: For the 164 patients who entered the study between September 1986 and December 1989, surgery was a limb salvage in 136 cases (82%) and a good histologic response was observed in 117 patients (71%). At a follow-up ranging from 10 to 13 years (median, 11.5 years), 101 patients (61%) remained continuously free of disease, 61 relapsed, and two died of ADM-induced cardiotoxicity. There were no differences in prognosis between good and poor responding patients. ADM-induced cardiotoxicity (six patients), male infertility (10 of the 12 assessable patients), and second malignancies (seven patients) were the major complications of chemotherapy. Despite the large number of limb salvages performed, only four local recurrences (2.4%) were registered. CONCLUSION: With an aggressive neoadjuvant chemotherapy, it is possible to cure more than 60% of patients with nonmetastatic osteosarcoma of the extremity and amputation may be avoided in more than 80% of them. Because local or systemic relapses, myocardiopathies, and second malignancies are possible even 5 years or more after the beginning of treatment, a long-term follow-up is recommended for these patients.     

Limb salvage by neoadjuvant isolated perfusion with TNFalpha and melphalan for non-resectable soft tissue sarcoma of the extremities.

AIMS: Patients with non-resectable soft tissue sarcomas of the extremities do not live longer if they are treated by amputation or disarticulation. In order to avoid major amputations, we tested isolated limb perfusion (ILP) with tumour necrosis factor alpha (TNF)+melphalan+/-interferon-gamma (IFN) as a pre-operative, neoadjuvant limb salvage treatment. METHODS: Twenty-two patients were included (six men and 16 women; three upper limb and 19 lower limb tumours). The AJCC stage was IIA in four patients, III in seven and IV in 11. Thirteen cases were recurrent or progressive after previous therapy; five tumours had a diameter >/=20 cm, and four were multiple or regionally metastatic. There were six malignant fibrous histiocytomas, five liposarcomas, four malignant peripheral nerve sheath tumours, three rhabdomyosarcomas, two leiomyosarcomas, one recurrent extraskeletal osteosarcoma and one angiosarcoma. RESULTS: Twenty-four ILPs were performed in the 22 patients, and 18 (82%) experienced an objective response: this was complete in four (18%) and partial in 14 (64%). Three patients had a minimal or no response and the tumour progressed in one case. All patients had fever for 24 hours but only one developed a reversible grade 3 distributive shock syndrome with no sequelae. There was no grade 4 toxicity. Seventeen patients (77%) underwent limb-sparing resection of the tumour remnants after a median time of 3.4 months: 10 resections were intracompartmental and seven extracompartmental. Surgery included flaps or skin grafts in five patients, arterial replacement in two and knee arthrodesis in one. Adjuvant chemotherapy was given to eight patients and radiotherapy to six. In one patient amputation was necessary after a second ILP. Secondary amputations were performed for recurrence in two patients, resulting in an overall limb salvage rate of 19/22 (86%). After a median follow-up of 18.7 months, 10 recurrences were recorded: seven were both local and systemic and three were only local. The median disease free and overall survival times have been >12.5 and 18.7 months respectively: this is similar to the outcome after primary amputations for similar cases. CONCLUSION: ILP with TNF and chemotherapy is an efficient limb sparing neoadjuvant therapy for a priori non-resectable limb soft tissue sarcomas.     

新辅助化疗结合新轴心式肿瘤特制型假体置换治疗膝关节周围骨肉瘤

目的 探讨新辅助化疗结合新轴心式肿瘤型假体置换在膝关节周围骨肉瘤保肢治疗中的临床效果.方法 采用新辅助化疗结合新轴心式肿瘤型假体置换保肢治疗膝关节周围骨肉瘤26例,其中男性15例,女性11例,年龄7～56岁,平均25.3岁.肿瘤部位股骨远端18例,胫骨近端8例.根据Enneking分期：I A期4例,I B期4例,II A期6例,II B期12例.采用化疗药物多柔比星、顺铂、甲氨蝶呤和异环磷酰胺进行新辅助化疗.术前化疗2～3个疗程,2-3周后进行保肢手术,术后继续化疗6个疗程.术前、术后每个化疗疗程间隔2-3周.所有病例切除标本均行病理检查并进行坏死率评估,以调整术后化疗方案.胫骨近端肿瘤切除后行腓肠肌内侧头肌瓣移位修复软组织缺损并与髌韧带缝合重建伸膝装置.术后根据Enneking评分标准评定疗效.结果 26例患者均获得随访,随访时间为8-56个月,平均28个月.经过术前化疗后患者疼痛迅速缓解,通过查体、X线片、CT或磁共振检查发现瘤体不同程度缩小、瘤体硬化、边缘清楚及有部分活动度.肿瘤坏死率测定：＞90%共18例,＜90%共6例.3例出现肺部转移,并因肺转移合并呼吸循环衰竭死亡,3例局部复发,行股骨下段截肢,20例无复发或远处转移.3年生存率为88.0%,复发率为11.5%,最终保肢率88.5%.26例患者术后根据Enneking评分标准评定疗效优19例,良4例,差3例,优良率88.5%.结论 新辅助化疗结合新轴心式肿瘤型膝关节假体置换是膝关节周围骨肉瘤保肢治疗的一种有效方法,能减少复发、远处转移及提高临床治疗效果.