Expression of bone morphogenetic protein and its receptors in osteosarcoma and malignant fibrous histiocytoma

BACKGROUND: Bone morphogenetic protein (BMP) activity has been found in cases of malignant fibrous histiocytoma (MFH) and osteosarcoma but only tumors in the latter category show evidence of ossification. The aim of this study was to try to understand this difference by examination of the distribution of BMP and its receptors (BMPR) for this bone inducing protein in these tumors. METHODS: Sections of 11 osteosarcoma and 10 MFH were analyzed immunohistochemically for BMP and BMPRs by use of the avidin-biotin peroxidase method. RESULTS: Nine out of 11 osteosarcoma cases (80.1%) showed positive staining for both BMP and BMPRs. Two cases of chondroblastic type osteosarcoma did not show any significant staining for BMP and BMPRs. In eight out of 10 MFH cases (80%) there was positive staining for BMP. No immunoreactivity for BMPRs was found in any case of MFH. CONCLUSIONS: MFH does not express BMPRs and this may be the reason why-MFH tumors do not ossify, even in the presence of BMP.     

The significance of bone and cartilage formation in malignant fibrous histiocytoma of soft tissue

Malignant fibrous histiocytoma of soft tissues (MFH), a mesenchymal tumor of varied morphologic patterns and cell types, sometimes contains bone and cartilage. Such bony and cartilaginous elements in a pleomorphic MFH may pose a difficult diagnostic challenge. An MFH with bone and cartilage can be distinguished from extraosseous osteogenic sarcoma and chondrosarcoma on the basis of qualitative and quantitative features of the osseous and chondroid elements. Five cases of soft tissue MFH containing bone and cartilage reported here showed: 1) that the bony and cartilaginous elements are mostly metaplastic and tend to be in the pseudo-capsule or fibrous septa of the tumor; and 2) that the bony elements may show a zoning pattern with peripheral maturation similar to myositis ossificans. In a single case, there was a small amount of "tumor osteoid." However, this was focal and lacked the diffuse and finely divided or ribbon-like pattern generally associated with osteogenic sarcoma. The probability that MFH with bone and cartilage is less aggressive than soft tissue osteogenic sarcoma or chondrosarcoma underscores the importance of accurate histologic diagnosis of these tumors.     

单纯髂骨切除术治疗髂骨原发恶性骨肿瘤

目的：探讨单纯髂骨切除术治疗髂骨原发恶性肿瘤的手术方法,分析其肿瘤学结果及骶髂关节连续性对患者肢体功能的意义。方法回顾分析1983年6月至2011年6月,诊断为髂骨原发恶性肿瘤并于我院骨肿瘤科行单纯髂骨切除术且资料完整的患者25例。分析该术式对髂骨恶性肿瘤的治疗效果及手术后骶髂关节连续性对患者肢体功能的影响。结果25例均于我院进行手术治疗,男19例,女6例。病例分布为软骨肉瘤13例,骨肉瘤6例,尤文肉瘤2例,梭形细胞肉瘤2例,未分化多形性肉瘤2例。随访14.2～127.9个月,平均41个月。截止随访期末,18例未发现肿瘤复发或转移。1例骨肉瘤患者于术后9个月出现肺转移,行胸腔镜肺部病灶切除,1例尤文肉瘤患者术后58个月出现肺部转移,行化疗,1例软骨肉瘤患者术后23个月出现局部复发,再次手术切除,此3例目前均存活。4例死亡,1例骨肉瘤患者术后10个月出现肺部转移,术后18个月死亡;1例软骨肉瘤患者术后12个月出现肺部转移,术后15个月死亡;1例术后29个月发现局部复发及肺部转移,术后39个月死亡;1例骨肉瘤患者术后26个月因肝功能衰竭死亡。15例可行MSTS评分系统进行评分,平均为27.5（24～30）分。其中10例骶髂关节连续性存在,MSTS评分平均为28.8分,5例骶髂关节连续性破坏,MSTS评分为25.0分。结论单纯髂骨切除术是治疗髂骨原发恶性肿瘤的有效术式,骶髂关节失去连续性对行走功能有一定影响。其功能可满足日常生活需要。     

Combination of gemcitabine and docetaxel in the treatment of children and young adults with refractory bone sarcoma

BACKGROUND: The combination of gemcitabine and docetaxel has demonstrated promise in sarcomas diagnosed in adults. In the current study, the toxicity and efficacy of this combination were evaluated in pediatric sarcomas. METHODS: A retrospective case review of 22 patients with recurrent or refractory bone or soft-tissue sarcomas who received gemcitabine (at a dose of 675 mg/m(2) intravenously on Days 1 and 8) and docetaxel (at a dose of 75-100 mg/m(2) intravenously on Day 8) was undertaken. RESULTS: The patients (ages 8-23 years) received a total of 109 courses of chemotherapy (median, 4 courses; range, 1-13 courses). Seventeen patients had osteosarcoma, 2 patients had Ewing sarcoma family of tumors (ESFT), 1 patient had a malignant fibrous histiocytoma (MFH), 1 patient had a chondrosarcoma, and 1 patient had an undifferentiated sarcoma. Of the 14 patients evaluable for response, the patient with an MFH achieved a complete response (CR), 3 patients with osteosarcoma achieved a partial response (PR), and 2 patients (1 with ESFT and 1 with osteosarcoma) had stable disease (SD). The overall objective response (CR + PR) rate was 29%. Median duration of response (CR + PR + SD) was 4.8 months (range, 1.6-13 months). The toxicity was manageable and consisted primarily of thrombocytopenia and neutropenia. CONCLUSIONS: In the current study, gemcitabine in combination with docetaxel was found to be well tolerated and demonstrated antitumor activity in children and adolescents with recurrent or refractory osteosarcoma and MFH. Further evaluation of this drug combination is warranted in these patients.     

经手术或穿刺活检后骨盆恶性骨肿瘤患者病理分析及病灶特点分析

目的 分析经手术或穿刺活检后病理确诊为骨盆恶性骨肿瘤患者的病理类型以及病灶特点.方法 收集136例经病理确诊为骨盆恶性骨肿瘤患者的临床资料.根据WHO 2013版骨肿瘤分型标准以及骨盆分区对136例患者的病灶特点以及病理类型进行分类分析总结.结果 本组患者浆细胞瘤发病率最高(30.1%),其次为非霍奇金淋巴瘤(21.3%)、软骨肉瘤(16.2%)、骨肉瘤(9.6%);骨肉瘤好发于青少年以及儿童,平均年龄为(24.6±14.9)岁,好发年龄段位为2~28岁,男性发病率高于女性(男女发病比例为2.25:1);尤文肉瘤也是青少年好发的骨盆恶性骨肿瘤之一,年龄为2~39岁,相比骨肉瘤年龄跨度更大.136例患者中41例患者单区发病,Ⅲ区发病例数最多为20例,发病率最高(48.8%),其次为Ⅰ区、Ⅳ区、Ⅱ区,单区发病中软骨肉瘤病例数为16例,发病率最高(39.0%);53例患者为多区发病,Ⅰ+Ⅱ+Ⅳ区发病例数17例,发病率最高(32.1%),其次为Ⅰ+Ⅳ、Ⅰ+Ⅱ、Ⅱ+Ⅲ、Ⅰ+Ⅱ+Ⅲ+Ⅳ区,非霍奇金淋巴瘤发病例数为15例,发病率最高(28.3%).结论 根据经手术或穿刺病理活检分析得出骨盆恶性骨肿瘤中浆细胞瘤发病率最高,其次为非霍奇金淋巴瘤、软骨肉瘤、骨肉瘤;各个年龄段都有发病,总体男性发病率高于女性,其中骨肉瘤以及尤文肉瘤好发于青少年;骨盆恶性骨肿瘤患者单发病灶中以Ⅲ区相对好发,且软骨肉瘤在单区病灶中发病率最高;多区病灶中Ⅰ+Ⅱ+Ⅳ区相对好发,非霍奇金淋巴瘤发病率最高.     

15例骨和软组织内间叶性软骨肉瘤临床病理分析

目的 探讨原发间叶性软骨肉瘤的临床特点、诊断和鉴别诊断依据。方法 复习本院15例骨及软组织原发问叶性软骨肉瘤的临床资料和病理学特征（包括对HE染色、免疫组化染色切片的观察分析），对14例行密切随访。结果 10年内15例间叶性软骨肉瘤占同期软骨肉瘤总数的9．4％，占小细胞恶性肿瘤的0．3％，占全部骨肿瘤的7．4％。患者年龄14～70岁，平均42岁。86％的患者存11-40岁之间，无明显性别差异。其中骨内发病13例，软组织内原发肿瘤2例。2例有外伤史。诊断初期多中心性发病者5例，患者多主诉局部疼痛伴肿胀。影像学特征与普通型软骨肉瘤相似，表现为伴有点状钙化的大片溶骨性破坏。组织学表现通常具有双相性，表现为间变的未分化间叶性小细胞与分化较好的分叶状肿瘤性软骨及软骨样基质并存。两种组织之间界限较清晰，移行区可见肿瘤细胞成软骨现象。免疫组织化学显示小细胞区CD99阳性，CollagenII在小细胞区和软骨区均阳性，部分病例CgA、Ki-67、P53有阳性表达，Syn在少数病例有阳性表达。本组患者最长在确诊后7年内死亡，最短在9个月内死亡，其中2例70岁以上的患者均在2年内死于肿瘤转移。结论 间叶性软骨肉瘤少见且预后差。虽然具有典型组织学特征，其诊断和鉴别诊断仍很困难。特别是在穿刺标本中，未穿到软骨性成分时与其他小细胞恶性肿瘤鉴别困难，未穿到小细胞成分时与其他类型软骨肉瘤鉴别困难。诊断中除仔细观察HE和免疫组化染色切片外，还需密切结合临床资料和影像学特征。     

骨囊肿、骨巨细胞瘤和骨肉瘤的计算机图象定量研究

目的:研究和解决骨巨细胞瘤的传统组织学分级与生物学行为不甚相符的问题.方法:利用自动图象分析仪对13例骨囊肿,51例骨巨细胞瘤(GCT)和15例骨肉瘤,共计79例标本进行了8项参数的形态定量研究.结果:在8项参数中,核X_6(核圆度)、X_7(核椭率)在不同肿瘤或分级的两两比较中全部无显著性差异;其余6项参数GCTⅠ、Ⅱ级均无显著性差异,而 GCTⅠ级与骨囊肿、Ⅲ级骨肉瘤各组间均有显著性差异,GCTⅡ级与骨囊肿、Ⅲ级骨肉瘤各组织间也有显著性差异.结论:经计算机图象定量研究,将8项参数经多元逐步判别分析,结果优选出有较多诊断价值的3项参数见、X_1、X_5、X_8,并依次建立判别函数方程(回归方程).     

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.     

胫骨远端恶性肿瘤瘤段切除大段异体骨移植术后临床效果分析

目的探讨大段异体骨移植在胫骨远端骨肿瘤保肢手术后的临床效果。方法自2004年6月至2009年6月我院骨肿瘤科共收治了15例进行大段异体骨移植的胫骨远端恶性骨肿瘤患者。其中骨肉瘤3例,软骨肉瘤4例,恶性纤维组织细胞瘤4例,恶性骨巨细胞瘤2例,造釉细胞瘤2例。其中15例患者均进行了瘤段切除大段异体骨移植,并进行了髓内钉内固定和踝关节融合。术后每月复查平片。患肢功能应用MSTS评分系统评价。结果15例患者均获得随访,随访时间12—72个月。至最近1次随访,所有患者异体骨与自体骨均得到骨性愈合。主要并发症：骨不连及排异反应,各1例。骨不连经再次手术植骨后愈合,排异反应患者经切口换药2个月后切口愈合。15例患者随访过程中无1例出现病理性骨折及感染,肿瘤复发等情况。平均MSTS评分为85％。结论我们认为大段同种异体骨修复胫骨远端肿瘤术后骨缺损是～种切实可行的有效方法。     

Twenty-year outcome of prevalence,incidence, mortality and survival rate in patients with malignant bone tumors

Malignant bone tumors are a group of rare malignant tumors and our study aimed to update the recent epidemiologic estimates based on the Surveillance, Epidemiology and End Results database. Patients diagnosed with malignant bone tumors from 2000 to 2019 were included and their characteristics were retrospectively described. The limited-duration prevalence, annual age-adjusted incidence and mortality were calculated, and the annual percentage changes were analyzed to quantify the rate change. Finally, observed survival and relative survival rate were illustrated. Subgroup analysis across tumor type, age, gender, tumor Grade, primary tumor site and stage was also performed. As for results, a total of 11 655 eligible patients with malignant bone tumor were selected. Osteosarcoma was the most common tumor type, followed by chondrosarcoma, Ewing sarcoma and chordoma. The estimated limited-duration prevalence of malignant bone tumors increased from 2000 (0.00069%) to 2018 (0.00749%). Steady age-adjusted incidence was observed in all patients during the study period while the highest rate occurred in osteosarcoma. Mortality rates differed in subgroups while elder patients (older than 64 years) presented the highest mortality rate compared to other age groups. In all bone tumors, the 10-year observed survival and relative survival rates were 58.0% and 61.9%, respectively. Chondrosarcoma patients had the best survival outcome, followed by osteosarcoma, Ewing sarcoma, chordoma and other bone tumors. In conclusion, different epidemiologic performance in incidence and mortality was observed across tumor type as well as other demographic and clinicopathological variables, which provide potential suggestion for further adjustment of medical resource.     

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.     

Value of immunohistochemical detection of noncollagenous proteins of bone for the diagnosis of bone tumours

The expression of osteonectin, osteopontin, bone sialoprotein, and osteocalcin was evaluated by immunohistochemistry in 57 cases of osteoid-forming and non-osteoid-forming bone tumours using specific polyclonal antibodies and the avidin-biotin peroxidase complex method. A positive immunostaining was found in all of the osteoid-forming rumours (osteoblastoma and osteosarcoma), both in the cells and in the extracellular matrix. Among non-osteoid-forming tumours, immunoreactivity to noncollagenous proteins was present in the cells but not in the matrix of chondrosarcoma, malignant fibrous histiocytoma, and fibrosarcoma, as well as in the mononuclear component of giant-cell rumours. Contrary to small-cell osteosarcoma, Ewing's sarcoma was always negative for all of the noncollagenous proteins considered. These results suggest that the immunohistochemical evaluation of noncollagenous proteins of bone may be a useful tool for the differential diagnosis of bone neoplasms, particularly among the heterogeneous group of small round cell tumours.     

葡萄糖转运蛋白-1在不同骨肿瘤中的表达

目的研究葡萄糖转运蛋白-1（GLUT-1）在正常骨组织及不同骨肿瘤标本中的表达情况。方法收集我院临床骨肉瘤标本30例，骨巨细胞瘤标本15例，骨样骨瘤标本10例及正常骨组织标本10例，采用免疫组化染色检测GLUT-1的分布。体外培养骨肉瘤细胞系MG63、临床骨肉瘤细胞、临床骨巨细胞瘤细胞及成骨细胞，RT-PCR检测GLUT-1基因的表达。结果免疫组化显示，骨肉瘤标本GLUT-1染色强阳性占总例数的53％；骨巨细胞瘤切片GLUT-1染色，阳性占总例数的60％；骨样骨瘤标本多为阴性，阴性占总例数的80％；正常骨组织中未见GLUT-1阳性表达。骨肉瘤明显高于与其余3组标本（χ^2=1．622，P=0．009；χ^2=34．667，P〈0．001，χ^2=40．000，P〈0．001）。RT-PCR显示，GLUT-1基因表达存在于MG63细胞、临床骨肉瘤细胞及骨巨细胞瘤中，而正常成骨细胞内并无GLUT-1表达。结论骨肿瘤中存在GLUT-1表达，且GLUT—1与骨肿瘤的恶性程度有较密切的关系。GLUT-1有可能作为判断骨肿瘤恶性程度参考指标及骨肿瘤治疗的新靶点。     

Bone and soft-tissue tumors

There have been great advances in recent years in the treatment of malignant bone and soft-tissue tumors thanks to advanced diagnostic techniques, improved chemotherapy and improved surgery involving extensive resection of the area involved, with bone and joint replacement. Here, we present details of the pathological findings, treatment and prognosis of cases of malignant bone and soft-tissue tumors, such as osteosarcoma, bone MFH, soft-tissue MFH, rhabdomyosarcoma and synovial sarcoma.     

Sorafenib in patients with progressed and refractory bone tumors

Patients with metastatic, progressive, or recurrent bone tumors have a dismal outcome. Sorafenib has been proposed as an effective salvage regimen for some malignancies. Thus, we sought to evaluate this approach for young patients with relapsed or refractory bone tumors. Twelve patients with refractory bone tumors (two with Ewing sarcoma, two with chondrosarcoma, and eight with osteosarcoma) received salvage treatment with sorafenib. All patients had standard tumor imaging and laboratory evaluation. All toxicities were documented. At the time of the beginning of sorafenib treatment median age among 12 patients was 18 years (range 4.1–27.9 years), eight were male, and eight had osteosarcoma. All received sorafenib because of relapse. Seven patients were treated parallel to other standard chemotherapy. Overall response rate was 75%. Median time to sorafenib time to progression for patients with osteosarcoma was 4 months (range 1.8–7.9 months). Four patients (33%) are alive, in that two with no evidence of disease with a median follow-up of 41 months (range 26.5–60.9 months). The estimated 5 year overall survival (OS) for the whole group was 64.49%. There were no serious toxicities. Sorafenib is well-tolerated in young patients with bone tumors, and particularly could be an option for patients with metastatic disease and refractory osteosarcoma. Sorafenib only allows to extend OS and different procedures are needed to achieve permanent remission. This regimen deserves further investigation in the upfront management of patients with high-risk bone tumors.     

Extracorporeal irradiation for malignant bone tumors

PURPOSE: Extracorporeal irradiation (ECI) has been used selectively in the management of primary malignant bone tumors since 1996. We report our techniques for ECI and the short-term oncologic and orthopedic outcomes. METHODS AND MATERIALS: Sixteen patients with primary malignant bone tumors were treated with ECI from 1996 to 2000. The median age was 14 years. The histologic diagnoses were Ewing's sarcoma (11), osteosarcoma (4) and chondrosarcoma (1). The treated sites were femur (7), tibia (4), humerus (2), ilium (2), and sacrum (1). Following induction chemotherapy in Ewing's sarcomas and osteosarcoma, en bloc resection of the tumor and tumor-bearing bone was performed. A single dose of 50 Gy was delivered to the bone extracorporeally using either a linear accelerator (9 cases) or a blood product irradiator (7 cases). The orthopedic outcome was recorded using a standard functional scale. RESULTS: At a median follow-up of 19.5 months, there were no cases of local recurrence or graft failure. One patient required amputation due to chronic osteomyelitis. For the 10 patients with follow-up greater than 18 months, the functional outcomes were graded good to excellent. CONCLUSION: The short-term oncologic and orthopedic results are encouraging and suggest that ECI provides a good alternative for reconstruction in limb conservative surgery in selected patients. This technique should only be used in a multidisciplinary setting, where careful follow-up is available to assess the long-term outcomes.     

Primary bone and joint tumor--statistical analysis of 571 cases

This paper analyses 571 cases of primary bone and joint tumors diagnosed by histopathology, excluding the tumor-like lesions. They were divided into two groups: the benign (412 cases, 72.15%) and the malignant (159 cases, 27.85%). The susceptible ages were between 15-29 years and the susceptible location of these tumors were femur and tibia often on the right side. There were more males than the females. The frequencies in the two groups are as follows: In the benign group, osteoma had the highest incidence and then, with decreasing frequencies: osteochondroma, chondroma, synovioma, giant cell tumor, ossifying fibroma, osteoid osteoma, chondromyxoid fibroma. In the malignant group, the highest incidence was in the osteosarcoma, and then synoviosarcoma, chondrosarcoma, malignant giant cell tumor, Ewing's tumor, fibrosarcoma, osteomyeloma. The sequence of the incidence was basically similar to that reported abroad and at home. The pathogenesis of several kinds of bone tumor are discussed.     

2140例骨肿瘤及瘤样病变统计分析

目的 探讨骨肿瘤及瘤样病变在我区的发病情况。方法 统计我院1963～2000年经病理检查证实的2140例骨肿瘤及瘤样病变进行统计分析。结果 原发性良性肿瘤583例(27．24％)，骨巨细胞瘤480例(22．43％)，原发恶性骨肿瘤590例(27．57％)，转移瘤33l例(15．47％)，瘤样病变156例(7．29％)。原发良、恶性肿瘤之比为0．99：1，男女之比为1．53：1，好发年龄为11～30岁，好发部位为股骨(30．18％)，其次为胫骨(20．40％)，良性骨肿瘤以骨软骨瘤(36．71％)、骨瘤(14．92％)多见。恶性骨肿瘤以骨肉瘤(53．56％)，软骨肉瘤89例(15．08％)多见。瘤样病变则以骨囊肿(66．03％)、骨纤维异样增殖症(28．21％)多见。结论 本组结果与国内文献对比有较多相似之处，但良、恶性骨肿瘤之比有较大不同，这可能与广西少数民族生活习惯及骨巨细胞瘤另分组统计有关。     

Malignancy in giant cell tumor of bone

BACKGROUND: The term malignant giant cell tumor embraces multiple entities and therefore can be confusing. The goals of the current study were to define the clinicopathologic and histologic features of malignancy in giant cell tumors and to clarify the terminology. METHODS: The authors reviewed all cases from the Rizzoli Institute (Bologna, Italy) of primary (PMGCT) and secondary (SMGCT) malignancy in giant cell tumors. PMGCT is a high-grade sarcoma that arises side by side with benign giant cell tumors. SMGCT is a high-grade sarcoma that occurs at the sites of previously treated giant cell tumors of bone. RESULTS: The authors report 5 PMGCTs and 12 SMGCTs; half of the SMGCTs were postradiation sarcomas. Patient age ranged from 20 to 68 years (median, 62 years) for PMGCT and from 30 to 77 years (median, 40 years) for SMGCT. The average latent period between diagnosis of giant cell tumor and diagnosis of SMGCT was 9 years (range, 3-15 years) for patients with postradiation SMGCT and 19 years (range, 7-28 years) for patients with SMGCT resulting from spontaneous transformation. The histologic classification of high-grade sarcomas in the PMGCT group was osteosarcoma in four cases and malignant fibrous histiocytoma in one case. In the SMGCT group, the histologic classification was osteosarcoma in nine cases, fibrosarcoma in two cases, and malignant fibrous histiocytoma in one case. The outcomes associated with all malignancies in giant cell tumors were poor, with the worst outcome associated with postradiation SMGCT. CONCLUSIONS: Malignancies in giant cell tumors of bone always are high-grade sarcomas with a poor prognosis. These lesions must be distinguished from benign giant cell tumors of bone. SMGCT usually is easy to diagnose upon malignant clinicoradiographic presentation. In contrast, PMGCT often mimics giant cell tumors both clinically and radiographically. In addition, upon histologic examination, PMGCT shows areas of conventional giant cell tumor, which can lead to difficulties in making the correct diagnosis.     

肿瘤假体置换在肱骨近端恶性骨肿瘤治疗中的应用

目的：探讨肿瘤假体置换在肱骨近端恶性骨肿瘤中应用的临床疗效。方法回顾分析2000年7月至2010年1月,我院治疗的32例肱骨近端恶性骨肿瘤患者,其中骨肉瘤10例,尤文氏肉瘤6例,骨巨细胞瘤7例,转移癌5例,软骨肉瘤4例。肩关节功能重建的方法是采用定制的人工肩关节肿瘤假体,术后根据Enneking骨骼肌肉肿瘤术后肢体功能评定标准进行疗效评价。结果术后随访3.1～5.3年（平均4.2年）,32例中2例死亡,2例复发,1例发生假体周围慢性感染,2例发生肩关节半脱位;所有患者肘、腕关节和手的功能基本正常,肩关节外展60°～90°（平均75°）,前屈35°～55°（平均45°）,后伸40°～60°（平均50°）,内收15°～20°（平均17.5°）。根据Enneking的骨骼肌肉肿瘤术后功能评分系统对32例进行评估,最低11分2例,最高28分2例;其中24～30分14例,18～23分9例,12～17分7例,12分以下2例,优良率为71.9%。结论人工肩关节假体置换在治疗肱骨近端恶性肿瘤,不仅能保留患肢完整的外观形态,而且能在很大程度上保留上肢的功能,是一种值得肯定的治疗方法。