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Genetic characterization of bone and soft tissue tumors. 骨和软组织肿瘤的遗传特征。
Pub Date : 2004-04-01 DOI: 10.1080/00016470410001708290
N Mandahl, F Mertens, I Panagopoulos, S Knuutila
21 During the last 20 years, genetic analyses of bone and soft tissue tumors (BSTT) have revealed that most histopathologic entities investigated in sufficient detail are characterized by acquired, clonal chromosome aberrations. Many of these rearrangements are nonrandom and several of them are strongly associated with specific histo pathologic tumor subtypes. Cytogenetic studies using chromosome banding techniques have been an important first mapping step that has guided further efforts to identify mutations at the gene level by fluorescence in situ hybridization (FISH), reverse-transcriptase polymerase chain reaction (RT-PCR) and other molecular techniques. Chromosomal comparative genomic hybridization (CGH), and more recently CGH microarray techniques, have revealed many specific ampli cons in various sarcomas. The strong association between chromosome or gene rearrangements and tumor subtype has more and more been exploited to aid the diagnostic work. Less conclusive data are available as regards the role of genetic aberrations for disease progression, but there are reports indicating that some genetic changes may provide prognostically important information. Even less is known about the origin of chromosome mutations and the relation between genetic aberrations and abnormal cellular processes associated with tumorigenesis.
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引用次数: 9
The Scandinavian Sarcoma Group--background, organization and the SSG Register--the first 25 years. 斯堪的纳维亚肉瘤组织-背景,组织和SSG注册-前25年。
Pub Date : 2004-04-01 DOI: 10.1080/00016470410001708250
T A Alvegård, H Bauer, C Blomqvist, A Rydholm, S Smeland
� � � � � � � � � � � � � � � � Musculoskeletal sarcomas call for multidisciplinary management by a “tumor team” of specialized orthopedic surgeons, radiologists, pathologists, tumor biologists (e.g. molecular and cytogenetics, DNA cytometry), cytologists, radiotherapists, and oncologists (Figure 1). Only a few such teams existed in Scandinavia during the 1970s. With the inception of the Scandinavian Sarcoma Group (SSG) in 1979, several new teams were started, each with regional responsibility for centralized treatment of sarcoma patients. Together, Denmark, Finland, Iceland, Norway and Sweden have a population of 25 million. These countries have similar social structures, with modern medical services covering all inhabitants and an effective registration of all cancer patients. The similarity of the
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引用次数: 14
Diagnosis and tumor response in osteosarcoma and Ewing's sarcoma. 骨肉瘤和尤文氏肉瘤的诊断和肿瘤反应。
Pub Date : 2004-04-01 DOI: 10.1080/00016470410001708350
T Böhling, P Bacchini, F Bertoni, B Bjerkehagen, H Domanski, L G Kindblom, J Meis-Kindblom, J Wejde
Both osteosarcoma and Ewingʼs sarcoma are highly malignant tumors, usually affecting children and adolescents. Before chemotherapy was introduced in the treatment of these tumors the prognosis was poor, but by intensified pre- and postoperative chemotherapy regimens the prognosis has improved dramatically. It soon became obvious that the tumor response to preoperative chemotherapy also served as a prognostic marker and today the postoperative chemotherapy treatment modality is based on the response rate. Those with a good tumor response receive a less intensive postoperative treatment than those whose tumor response has been poor (Picci 1997 et al., Bacci 2000 et al.). The Scandinavian sarcoma group (SSG), has since 25 years carried out several studies on both osteosarcoma and Ewingʼs sarcoma, some of them in collaboration with the Italian sarcoma group (ISG). The protocols for osteosarcoma SSG II, SSG VIII, ISG/SSG I, ISG/SSG II and SSG XIV, as well as the protocols for Ewingʼs sarcoma SSG IV, SSG IX, ISG/SSG III and ISG/SSG IV are described elsewhere (http://www.ssg-org.net/). In these protocols different methods of assessing the chemotherapy response have been used. Here, we will present and discuss the different methods.
{"title":"Diagnosis and tumor response in osteosarcoma and Ewing's sarcoma.","authors":"T Böhling, P Bacchini, F Bertoni, B Bjerkehagen, H Domanski, L G Kindblom, J Meis-Kindblom, J Wejde","doi":"10.1080/00016470410001708350","DOIUrl":"https://doi.org/10.1080/00016470410001708350","url":null,"abstract":"Both osteosarcoma and Ewingʼs sarcoma are highly malignant tumors, usually affecting children and adolescents. Before chemotherapy was introduced in the treatment of these tumors the prognosis was poor, but by intensified pre- and postoperative chemotherapy regimens the prognosis has improved dramatically. It soon became obvious that the tumor response to preoperative chemotherapy also served as a prognostic marker and today the postoperative chemotherapy treatment modality is based on the response rate. Those with a good tumor response receive a less intensive postoperative treatment than those whose tumor response has been poor (Picci 1997 et al., Bacci 2000 et al.). The Scandinavian sarcoma group (SSG), has since 25 years carried out several studies on both osteosarcoma and Ewingʼs sarcoma, some of them in collaboration with the Italian sarcoma group (ISG). The protocols for osteosarcoma SSG II, SSG VIII, ISG/SSG I, ISG/SSG II and SSG XIV, as well as the protocols for Ewingʼs sarcoma SSG IV, SSG IX, ISG/SSG III and ISG/SSG IV are described elsewhere (http://www.ssg-org.net/). In these protocols different methods of assessing the chemotherapy response have been used. Here, we will present and discuss the different methods.","PeriodicalId":75404,"journal":{"name":"Acta orthopaedica Scandinavica. Supplementum","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2004-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/00016470410001708350","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"24559786","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 4
Combined radiology and cytology in the diagnosis of bone lesions--a review of 399 cases. 放射学与细胞学联合诊断骨病变——附399例报告
Pub Date : 2004-04-01 DOI: 10.1080/00016470410001708320
V Söderlund
Most patients with symptoms from the locomotor system undergo radiological examination, among which a minority is found to have a lesion arousing the suspicion of neoplasia. The diagnosis of primary bone tumors is generally perceived as difficult. A main reason is the low incidence making it difficult to gain wide diagnostic experience. Basically, this can only be attained at referral centers. However, even for specialists the diagnosis of bone lesions may pose considerable difficulties because of highly variable macroand microfeatures (Figure 1). Hence, it is important to collect data from different diagnostic modalities while considering also age, clinical history and clinical findings. Most importantly, the diagnostic approach should entail both a macroand a microcharacterization. Over the last years, core needle biopsy and fine needle aspiration have gradually gained acceptance as a diagnostic alternative to open biopsy to circumvent associated inconveniences and risks (Hajdu 1971, Tehranzadeh et al. 1983, El-Khoury Combined radiology and cytology in the diagnosis of bone lesions—a review of 399 cases
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引用次数: 9
The Scandinavian Sarcoma Group 25 years' experience. 斯堪的纳维亚肉瘤集团25年的经验。
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引用次数: 0
Gastrointestinal stromal tumors--a review. 胃肠道间质瘤——综述。
Pub Date : 2004-04-01 DOI: 10.1080/00016470410001708340
H Joensuu, L G Kindblom

Gastrointestinal stromal tumors (GISTs) may be defined as intraabdominal nonepithelial (mesenchymal) tumors that express the KIT protein or have an activating mutation in a class III receptor tyrosine kinase gene (KIT or PDGFRA). GISTs are diagnosed at a frequency of about 15 new cases annually per million, though small indolent GISTs are likely to occur more frequently in the general population. The clinical behavior is variable, and assessment of the malignancy potential is usually based mainly on the size and the proliferation characteristics of the tumor. The overwhelming majority of GISTs express the KIT protein, the transmembrane receptor tyrosine kinase for the stem cell factor. The majority of GISTs harbor a mutation in the KIT proto-oncogene that translates into constitutively activated KIT protein kinase, and a minority have mutated PDGFRA gene resulting in activated platelet-derived growth factor alpha receptor tyrosine kinase. Most GISTs respond to imatinib mesylate, which selectively inhibits both KIT and PDGFRA, and is now considered as the standard systemic therapy for advanced GIST. In contrast, responses to conventional chemotherapy are infrequent (generally less than 10%), but combination therapies with imatinib have not been explored. Research on adjuvant imatinib and novel targeted therapies is ongoing.

胃肠道间质瘤(gist)可定义为表达KIT蛋白或III类受体酪氨酸激酶基因(KIT或PDGFRA)激活突变的腹腔内非上皮(间充质)肿瘤。每年每百万人中约有15个新病例被诊断为胃肠道间质瘤,尽管在普通人群中,较小的惰性胃肠道间质瘤可能更频繁地发生。临床表现多变,恶性潜能的评估通常主要基于肿瘤的大小和增殖特征。绝大多数gist表达KIT蛋白,即干细胞因子的跨膜受体酪氨酸激酶。大多数gist携带KIT原癌基因突变,可转化为组成型激活的KIT蛋白激酶,少数突变的PDGFRA基因导致血小板衍生的生长因子α受体酪氨酸激酶活化。大多数GIST对甲磺酸伊马替尼有反应,选择性地抑制KIT和PDGFRA,现在被认为是晚期GIST的标准全身治疗。相比之下,对常规化疗的反应很少(通常小于10%),但与伊马替尼联合治疗尚未探索。辅助伊马替尼和新型靶向治疗的研究正在进行中。
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引用次数: 35
Chemotherapy in osteosarcoma. The Scandinavian Sarcoma Group experience. 骨肉瘤的化疗。斯堪的纳维亚肉瘤集团的经验。
Pub Date : 2004-04-01 DOI: 10.1080/00016470410001708380
S Smeland, T Wiebe, T Böhling, O Brosjö, K Jonsson, T A Alvegård
During the past 15 years the Scandinavian Sarcoma Group has treated 140 patients with Ewing's sarcoma. Two protocols have been used. SSG IV included 52 patients between 1984 and 1990 and SSG IX, 88 patients since 1990. After 5 years of treatment, local recurrences occurred in 19% of the patients (M0 + M1) in the SSG IV group and 10% in the SSG IX group. Distant metastases developed in 57% of the M0-patients in the SSG IV group and in 33% in the SSG IX group. Tumor-related survival (overall) of M0-patients was 49% in SSG IV and 70% in SSG IX, and the metastasis-free survival rate 45% and 58%, respectively. Patients having a localized extremity tumor had a survival rate of 90% (SSG IX). In both treatment groups, good responders to chemotherapy had a better survival rate than poor ones (SSG IV, p < 0.02, GI-II vs. G II-IV and SSG IX, p < 0.003, GI-III vs. G IV). In conclusions local control and survival rates were better with SSG IX than SSG IV.
{"title":"Chemotherapy in osteosarcoma. The Scandinavian Sarcoma Group experience.","authors":"S Smeland,&nbsp;T Wiebe,&nbsp;T Böhling,&nbsp;O Brosjö,&nbsp;K Jonsson,&nbsp;T A Alvegård","doi":"10.1080/00016470410001708380","DOIUrl":"https://doi.org/10.1080/00016470410001708380","url":null,"abstract":"During the past 15 years the Scandinavian Sarcoma Group has treated 140 patients with Ewing's sarcoma. Two protocols have been used. SSG IV included 52 patients between 1984 and 1990 and SSG IX, 88 patients since 1990. After 5 years of treatment, local recurrences occurred in 19% of the patients (M0 + M1) in the SSG IV group and 10% in the SSG IX group. Distant metastases developed in 57% of the M0-patients in the SSG IV group and in 33% in the SSG IX group. Tumor-related survival (overall) of M0-patients was 49% in SSG IV and 70% in SSG IX, and the metastasis-free survival rate 45% and 58%, respectively. Patients having a localized extremity tumor had a survival rate of 90% (SSG IX). In both treatment groups, good responders to chemotherapy had a better survival rate than poor ones (SSG IV, p < 0.02, GI-II vs. G II-IV and SSG IX, p < 0.003, GI-III vs. G IV). In conclusions local control and survival rates were better with SSG IX than SSG IV.","PeriodicalId":75404,"journal":{"name":"Acta orthopaedica Scandinavica. Supplementum","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2004-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/00016470410001708380","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"24558564","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 73
An updated review of the epidemiology of soft tissue sarcoma. 软组织肉瘤流行病学的最新综述。
Pub Date : 2004-04-01 DOI: 10.1080/00016470410001708280
H Olsson
17 Incidence Soft tisssue sarcoma (SST) represents a heterogenous group of tumor diseases orginating from connective tissue accounting for less than 1 % of all malignant tumors in Sweden. With increasing age there is an increase in incidence (National Board of Health). Comprehensive reviews of the epidemiology of soft tissue sarcoma have previously been published (Tucker et al. 1982, Zahm and Fraumeni 1997 and Olsson 1999). The heterogenity of sarcoma types and the rarity of cases have hampered etiologically oriented studies. When excluding Kaposi sarcoma the incidence of other soft tissue sarcomas have not dramatically increased in developed western countries. Becasue of the inherent difficulty in classifying SST inter national variations and geographic patterns have not been evaluated in a meaningful way. Risk factors of SST are thus only partly known. Risk factors include a family history of cancer/ soft tissue sarcoma, certain genetic syndromes, exposure to ionizing irradiation, and exposure to certain chemicals such as vinyl chloride. Other factors associated with SST development include longstanding lymphedema, exposure to Thorotrast, arsenical pesticides and medications, herbicides, immunosuppressive drugs, alkylating agents, androgen-anabolic steroids, human immunodefi ciency virus, and exposure to human herpes virus type 8 (Zahm and Fraumeni 1997 and Olsson 1999). Further a tissue trauma has been discussed as a possible risk factor. Studies from our own group has suggested that oral contraceptive use is partly protective for STS in women (Olsson et al.
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引用次数: 20
Chemotherapy in Ewing's sarcoma. The Scandinavian Sarcoma Group experience. 尤因氏肉瘤的化疗。斯堪的纳维亚肉瘤集团的经验。
Pub Date : 2004-04-01 DOI: 10.1080/00016470410001708370
S Smeland, T Wiebe, O Brosjö, T Böhling, T A Alvegård
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引用次数: 23
Lumbar spinal fusion. Outcome in relation to surgical methods, choice of implant and postoperative rehabilitation. 腰椎融合。结果与手术方法、种植体选择和术后康复有关。
Pub Date : 2004-01-01 DOI: 10.1080/03008820410002057
F. Christensen
UNLABELLED Chronic low back pain (CLBP) has become one of the most common causes of disability in adults under 45 years of age and is consequently one of the most common reasons for early retirement in industrialised societies. Accordingly, CLBP represents an expensive drain on society's resources and is a very challenging area for which a consensus for rational therapy is yet to be established. The spinal fusion procedure was introduced as a treatment option for CLBP more than 70 years ago. However, few areas of spinal surgery have caused so much controversy as spinal fusion. The literature reveals divergent opinions about when fusion is indicated and how it should be performed. Furthermore, the significance of the role of postoperative rehabilitation following spinal fusion may be underestimated. There exists no consensus on the design of a program specific for rehabilitation. Ideally, for any given surgical procedure, it should be possible to identify not only possible complications relative to a surgical procedure, but also what symptoms may be expected, and what pain behaviour may be expected of a particular patient. The overall aims of the current studies were: 1) to introduce patient-based functional outcome evaluation into spinal fusion treatment; 2) to evaluate radiological assessment of different spinal fusion procedures; 3) to investigate the effect of titanium versus stainless steel pedicle screws on mechanical fixation and bone ingrowth in lumbar spinal fusion; 4) to analyse the clinical and radiological outcome of different lumbar spinal fusion techniques; 5) to evaluate complications and re-operation rates following different surgical procedures; and 6) to analyse the effect of different rehabilitation strategies for lumbar spinal fusion patients. The present thesis comprises 9 studies: 2 clinical retrospective studies, 1 clinical prospective case/reference study, 5 clinical randomised prospective studies and 1 animal study (Mini-pigs). In total, 594 patients were included in the investigation from 1979 to 1999. Each had prior to inclusion at least 2 years of CLBP and had therefore been subjected to most of the conservative treatment leg pain, due to localized isthmic spondylolisthesis grades I-II or primary or secondary degeneration. PATIENT-BASED FUNCTIONAL OUTCOME: Patients' self-reported parameters should include the impact of CLBP on daily activity, work and leisure time activities, anxiety/depression, social interests and intensity of back and leg pain. Between 1993 and 2003 approximately 1400 lumbar spinal fusion patients completed the Dallas Pain Questionnaire under prospective design studies. In 1996, the Low Back Pain Rating scale was added to the standard questionnaire packet distributed among spinal fusion patients. In our experience, these tools are valid instruments for clinical assessment of candidates for spinal fusion procedures. RADIOLOGICAL ASSESSMENT It is extremely difficult to interpret radiographs of both l
慢性腰痛(CLBP)已成为45岁以下成年人致残的最常见原因之一,因此也是工业化社会中提前退休的最常见原因之一。因此,CLBP代表了对社会资源的昂贵消耗,并且是一个非常具有挑战性的领域,对于合理的治疗尚未达成共识。70多年前,脊柱融合术作为CLBP的一种治疗选择被引入。然而,很少有脊柱外科领域像脊柱融合术那样引起如此多的争议。文献揭示了关于何时进行融合以及如何进行融合的不同意见。此外,脊柱融合术后康复的重要性可能被低估了。目前还没有共识的设计方案,专门为康复。理想情况下,对于任何给定的外科手术,不仅应该能够确定与外科手术有关的可能并发症,而且应该能够确定特定患者可能出现的症状和可能出现的疼痛行为。当前研究的总体目标是:1)将基于患者的功能结果评估引入脊柱融合治疗;2)评价不同脊柱融合术的放射学评价;3)比较钛与不锈钢椎弓根螺钉对腰椎融合术中机械固定和骨长入的影响;4)分析不同腰椎融合术的临床和影像学结果;5)评估不同手术方式的并发症及再手术率;6)分析不同康复策略对腰椎融合术患者的疗效。本论文包括9项研究:2项临床回顾性研究,1项临床前瞻性病例/参考研究,5项临床随机前瞻性研究和1项动物研究(迷你猪)。1979 - 1999年共纳入594例患者。在纳入研究之前,每位患者至少有2年的CLBP,因此,由于局部I-II级峡部滑脱或原发性或继发性退行性变,他们接受了大多数保守治疗的腿部疼痛。基于患者的功能结局:患者自我报告的参数应包括CLBP对日常活动、工作和休闲时间活动、焦虑/抑郁、社交兴趣和腰腿疼痛强度的影响。在1993年至2003年间,大约1400名腰椎融合术患者在前瞻性设计研究中完成了达拉斯疼痛问卷。1996年,在脊柱融合术患者中分发的标准问卷包中增加了腰痛评定量表。根据我们的经验,这些工具是临床评估脊柱融合术候选人的有效工具。影像学评价腰椎后外侧融合和前路椎间融合的x线片很难解释。x线平片显然不是分析脊柱融合的完美媒介,但在新的更好的诊断方法可用于临床使用之前,x线片仍将是黄金标准。因此,一个详细可靠的放射学分类系统的发展是非常必要的。本论文中用于评估后外侧脊柱融合术的分类,无论有无内固定,均表现出良好的观察者间和观察者内一致性。该分类显示出可接受的可靠性,可能是改善后外侧脊柱融合术后影像学结果研究间和研究内相关性的一种方法。当使用固定架时,基于放射学的腰椎前路椎体间融合评估变得更加复杂。使用不同的保持器设计和材料使得几乎不可能建立一个标准的前路融合放射学分类系统。骨-螺钉界面:钛椎弓根螺钉在骨-螺钉界面处的机械结合明显大于不锈钢椎弓根螺钉。这可以解释为钛螺钉具有更好的骨生长。螺钉拆卸扭矩与拔出强度之间没有相关性。临床上,对于骨质疏松和成骨能力下降的患者,钛及钛合金椎弓根螺钉可能更可取。结果:目前的一系列研究发现,大约70%接受腰椎融合术的患者的长期功能有显著改善。根据手术方式的不同,x线片显示的固体融合率从52%到92%不等。 手术方式的选择应与诊断相关,因为峡部滑脱(I级和II级)患者最好采用无内固定的后外侧融合术,而椎间盘退变患者似乎从内固定的后外侧融合术或周向融合术中获益最多。并发症使用椎弓根螺钉系统支持后外侧融合后围手术期并发症增加,使用周向融合后围手术期并发症进一步增加。结果与围手术期并发症无显著相关性。然而,脊柱融合术后2年内再手术的风险对于接受周向融合术的患者明显低于接受后外侧内固定融合术的患者。此外,发现接受周向融合术的患者不愈合的风险明显低于接受内固定或不接受内固定的后外侧融合术的患者。性功能障碍和非预期水平融合的并发症被发现是显著的,但对总体结果没有影响。在腰椎融合术后2年,与视频组和训练组相比,背部咖啡组的患者连续完成许多日常任务明显更好,而且疼痛更少。视频组在医院系统外的治疗需求明显更高。本研究证明了应对方案的重要性,并质疑了强化锻炼在脊柱融合术患者康复计划中的作用。
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引用次数: 108
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Acta orthopaedica Scandinavica. Supplementum
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