atypical cartilaginous tumor

  • 文章类型: Case Reports
    非典型软骨肿瘤(ACT)是指在显微镜下与1级软骨肉瘤相同的低度软骨肿瘤,影响阑尾骨骼。已发现,与广泛切除相比,病灶内刮治治疗可提供足够的局部控制,发病率较低。这是第一例报道的同时进行内侧髌股韧带(MPFL)重建,并在同侧股骨上进行ACT刮除。一名45岁女性,患有右膝慢性复发性髌骨脱位。磁共振成像显示MPFL撕裂,伴有偶发的epi-meta端软骨样病变。活检证实ACT后,使用冷冻氮乙醇复合材料(FNEC)和MPFL重建进行了单级延长刮治,然后用骨水泥和股骨远端钢板增强。目前,病人可以独立走动,膝盖周围有全方位的运动。在同时发生MCL撕裂的情况下,对ACT进行组织学确认后,解决这两种情况的单阶段手术是一个可行的选择,可以减少与多次手术相关的并发症.使用FNEC的长期刮治已被证明可产生良好的短期肿瘤结果,同时最大限度地发挥功能。
    Atypical cartilaginous tumor (ACT) refers to a low-grade cartilaginous neoplasm microscopically identical to grade 1 chondrosarcoma, affecting the appendicular skeleton. Treatment with intralesional curettage has been found to provide sufficient local control with less morbidity compared to wide resection. This is the first reported case of a simultaneous medial patello-femoral ligament (MPFL) reconstruction with extended curettage for ACT on the ipsilateral femur. A 45-year-old female presented with chronic recurrent patellar dislocation of the right knee. Magnetic resonance imaging revealed a tear of the MPFL, with an incidental epi-metaphyseal chondroid lesion. After biopsy confirmed an ACT, single-stage extended curettage using freezing nitrogen ethanol composite (FNEC) and MPFL reconstruction was performed, followed by augmentation with bone cement and a distal femoral plate. Currently, the patient is independently ambulatory, with full range of motion about the knee. Following histologic confirmation of an ACT in the setting of a concurrent MCL tear, a single-stage procedure to address both conditions is a viable option that can reduce complications associated with multiple surgeries. Extended curettage using FNEC has been shown to produce good short-term oncologic outcomes while maximizing function.
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  • 文章类型: Journal Article
    低级别软骨肉瘤(LG-CS),包括非典型软骨肿瘤(ACT),是局部侵袭性病变。讨论的重点在于良性病变或侵袭性软骨肿瘤之间的鉴别诊断及其治疗:病灶内刮治或广泛切除。本研究介绍了21例LG-CS的手术治疗结果。方法本回顾性研究包括2013年至2021年接受手术治疗的21例LG-CS患者。十四个位于附肢骨骼中,轴向有七个(肩胛骨,脊柱,或骨盆)。死亡率,复发,转移性疾病,总生存率,无复发生存率,分析了每种手术类型和每种疾病位置的无转移性疾病生存率。在进行切除的情况下,还记录了手术并发症和残留肿瘤。使用Kaplan-Meier方法计算存活率。结果13例患者行病灶内刮治(11例阑尾,2例轴性病灶),8例接受了广泛切除(5个轴向和3个阑尾)。随访期间有6次复发,43%的轴性病变复发,在轴向收缩的情况下上升到100%。阑尾性LG-CS在21%的病例中复发,只有18%的刮除的阑尾病变未根除。整个随访的总生存率为90.5%,5年生存率为83%(12例患者有足够的随访)。切除病例的无复发和无转移生存率较高,75%和87.5%,vs.刮治69.2%和76.9%,分别。在9%的案例中,术前活检与手术标本的病理不一致。讨论LG-CS和ACT被描述为具有高存活率和低转移性疾病的可能性。出于这个原因,这些病变的治疗理念会发生变化,以反映这些特征。病灶内刮治被认为是一种侵入性较小的技术,用于根除非典型软骨肿瘤,并且并发症越来越少,这与我们的发现是一致的。诊断,然而,是具有挑战性的;错误评级是经常发生的,应该加以考虑。由于对高级别病变治疗不足的风险,一些作者仍然认为广泛切除术是首选的治疗方法.我们观察到生存期更长的趋势,减少复发,和广泛切除的转移性疾病。转移性疾病高于预期,在19%的病例中,并且总是与局部复发有关。结论LG-CS仍然是诊断和治疗的挑战;患者选择是根本。总生存率高,独立于治疗选择或病变位置。我们发现转移性疾病的发生率高于文献中描述的;这,再加上9%的错误率,反映了术前诊断的困难和将高级别软骨肉瘤作为低度病变治疗的风险。应使用更大的样本进行更多的研究,以获得统计上可靠的结果。
    Introduction Low-grade chondrosarcomas (LG-CS), including atypical cartilaginous tumors (ACT), are locally aggressive lesions. The focus of the discussion sits on the differential diagnosis between benign lesions or aggressive cartilaginous tumors and on their treatment: intralesional curettage or wide resection. This study presents the results obtained in the surgical treatment of 21 cases of LG-CS. Methods This retrospective study includes 21 consecutive patients from a single center with LG-CS who underwent surgery from 2013 to 2021. Fourteen were located in the appendicular skeleton, and seven in the axial (shoulder blade, spine, or pelvis). Mortality rate, recurrence, metastatic disease, overall survival, recurrence-free survival, and metastatic disease-free survival were analyzed for each type of procedure and each disease location. Operative complications and residual tumors were also recorded in cases where resection was performed. Survival was calculated using the Kaplan-Meier method. Results Thirteen patients underwent intralesional curettage (11 appendicular and 2 axial lesions), and eight underwent wide resection (5 axial and 3 appendicular). There were six recurrences during the follow-up, 43% of the axial lesions recurred, rising to 100% in axial curetted ones. Appendicular LG-CS recurred in 21% of cases, and only 18% of curetted appendicular lesions were not eradicated. The overall survival for the entire follow-up is 90.5%, and the 5-year survival rate is 83% (12 patients have adequate follow-up). Recurrence-free and metastasis-free survival were higher in resection cases, with 75% and 87.5%, vs. curettage 69.2% and 76.9%, respectively. In 9% of cases, the preoperative biopsy was inconsistent with the pathology of the surgical specimen. Discussion LG-CS and ACT are described as having high survival and low potential for metastatic disease. For this reason, these lesions are subject to a change in treatment philosophy to reflect these characteristics. Intra-lesional curettage is advocated as a less invasive technique for eradicating atypical cartilage tumors and has fewer and less severe complications, which was in accordance with our findings. Diagnosis, however, is challenging; misgrading is frequent and should be considered. Because of this risk of under-treating higher-grade lesions, some authors still defend wide-resection as the treatment of choice. We observed a trend towards longer survival, less recurrence, and metastatic disease with wide resection. Metastatic disease was higher than expected, present in 19% of cases, and always associated with local recurrence. Conclusion LG-CS is still a diagnostic and treatment challenge; patient selection is fundamental. Overall survival is high, independent of treatment choice or lesion location. We found a higher rate of metastatic disease than described in the literature; this, coupled with a misgrading rate of 9%, reflects the difficulty of preoperative diagnosis and the risk of treating high-grade chondrosarcomas as a low-grade lesion. More studies should be carried out with larger samples to obtain statistically robust results.
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  • 文章类型: Journal Article
    Chondrosarcomas can be classified into various forms according to the presence or absence of a precursor lesion, location, and histological subtype. The new 2020 World Health Organization (WHO) Classification of Tumors of Soft Tissue and Bone classifies chondrogenic bone tumors as benign, intermediate (locally aggressive), or malignant, and separates atypical cartilaginous tumors (ACTs) and chondrosarcoma grade 1 (CS1) as intermediate and malignant tumors. respectively. Furthermore, the classification categorizes chondrosarcomas (including ACT) into eight subtypes: central conventional (grade 1 vs. 2-3), secondary peripheral (grade 1 vs. 2-3), periosteal, dedifferentiated, mesenchymal, and clear cell chondrosarcoma. Most chondrosarcomas are the low-grade, primary central conventional type. The rarer subtypes include clear cell, mesenchymal, and dedifferentiated chondrosarcomas. Comprehensive analysis of the characteristic imaging findings can help differentiate various forms of chondrosarcomas. However, distinguishing low-grade chondrosarcomas from enchondromas or high-grade chondrosarcomas is radiologically and histopathologically challenging, even for experienced radiologists and pathologists.
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  • 文章类型: Journal Article
    目的:根据全身(TB)MRI检查评估多发性骨软骨瘤患者骨内软骨病变的患病率,用于筛选目的。
    方法:在2013年至2020年之间,对366例确诊为多发性骨软骨瘤综合征的患者进行了TB-MRI检查,排除恶性进展.对于这项研究,存在,或没有骨内中央或偏心软骨样病变,定义为T1加权图像上具有低信号强度的分叶状病变,在(其中之一)长骨的中段骨干的骨髓中的T2加权图像上替换骨髓和等于液体的高信号强度,作为TB-MRI方案的一部分记录在长骨中。
    结果:在366名MO患者中,有62名患者(17%),检测到一个或多个骨内软骨样病变(内生软骨瘤或非典型软骨瘤)。患者在诊断时的年龄范围为17至61岁(平均,36).病变的大小从4到69毫米不等(平均,16.3mm)。最常见的位置是股骨近端(n=29),其次是股骨远端和肱骨近端(分别为n=18和n=10)。在9例骨内软骨样病变患者中,在评估期间可获得第二次和/或第三次TB-MRI(平均间隔时间,考试之间的2.7年)。在这些患者中,均未发现这些骨内病变的增加。
    结论:骨内软骨样病变(内软骨瘤和ACT)在MO患者中的发生频率高于一般人群。TB-MRI允许检测这些,除了识别具有可疑特征的OC。
    OBJECTIVE: To assess the prevalence of intraosseous cartilaginous lesions in patients with multiple osteochondromas based on total-body (TB) MRI examinations, used for screening purposes.
    METHODS: Between 2013 and 2020, TB-MRI examinations were performed in 366 patients with proven multiple osteochondromas syndrome, to rule out malignant progression. For this study, presence, or absence of intraosseous central or eccentrical chondroid lesions, defined as lobulated lesions with low signal intensity on T1-weighted images, replacing bone marrow and high signal intensity equal to fluid on T2-weighted images in the bone marrow of the meta-diaphysis of (one of) the long bones, were recorded in the long bones as part of a TB-MRI protocol.
    RESULTS: In 62 patients out of the 366 MO patients (17%), one or more intraosseous chondroid lesions (either enchondroma or atypical cartilaginous tumor) were detected. The age of the patients at time of diagnosis ranged from 17 to 61 years (mean, 36). Size of the lesions varied from 4 to 69 mm (mean, 16.3 mm). The most common location was the proximal femur (n = 29), followed by the distal femur and proximal humerus (n = 18 and n = 10, respectively). In nine of the patients with an intraosseous chondroid lesion, a second and/or third TB-MRI were available during the period of evaluation (mean interval, 2.7 years between the exams). In none of these patients increase of these intraosseous lesions was noticed.
    CONCLUSIONS: Intraosseous chondroid lesions (enchondroma and ACT) appear to occur more frequently in MO patients than in the general population. TB-MRI allows to detect these, besides the identification of OC with suspicious features.
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  • 文章类型: Journal Article
    目的:扁骨中的软骨肉瘤被认为行为更具攻击性,对这些部位的低级别软骨肉瘤的病灶内治疗结果知之甚少。我们试图找到在他们的扁平骨与长骨受累的低级别软骨肉瘤患者之间的差异(1)疾病的结果,(2)功能结果,(3)治疗并发症。
    方法:我们回顾性分析了44例原发性低级别软骨肉瘤患者,这些患者接受了病灶内刮治和冷冻治疗。患者根据肿瘤的位置进行划分,第一组(扁平骨头,七名患者)和第二组(长骨,37名患者)。
    结果:I组的局部复发率较高,I组的5年无病生存率为80.0%,II组为97.0%(p=0.001)。注意到所有复发病例最初表现为软组织扩张(EnnekingIB期)。最后一次随访时肌肉骨骼肿瘤协会的平均评分I组为21.7,II组为27.9(p=0.045)。
    结论:对于病灶局限于骨(EnnekingIA期)的患者,低级别软骨肉瘤的病灶内刮治和冷冻治疗似乎是一种安全合理的手术选择。
    方法:三级,回顾性队列研究。有关证据级别的完整描述,请参阅作者指南。
    OBJECTIVE: Chondrosarcomas in flat bones are thought to be more aggressive in their behavior, and little is known about intralesional treatment outcomes of low-grade chondrosarcoma in these locations. We tried to find the differences between patients who had low-grade chondrosarcoma in their flat bones versus those with long bone involvement with regard to (1) disease outcome, (2) functional outcome, and (3) treatment complications.
    METHODS: We retrospectively reviewed 44 patients with primary low-grade chondrosarcoma who were treated with intralesional curettage and cryotherapy. The patients were divided by location of tumor, group I (flat bones, seven patients) and group II (long bones, 37 patients).
    RESULTS: The local recurrence rate was higher in group I with 5 years disease-free survival of 80.0% in group I and 97.0% in group II (p = 0.001). All recurrent cases were noted to have initially presented with soft tissue extension (Enneking stage IB). The mean Musculoskeletal Tumor Society score at the last follow-up was 21.7 in group I and 27.9 in group II (p = 0.045).
    CONCLUSIONS: Intralesional curettage and cryotherapy for low-grade chondrosarcoma appear to be a safe and reasonable surgical option for patients with lesions confined to bone (Enneking stage IA).
    METHODS: Level III, retrospective cohort study. See the Guidelines for Authors for a complete description of levels of evidence.
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  • 文章类型: Journal Article
    非典型软骨肿瘤(ACT)和内生软骨瘤之间的区别至关重要,因为ACT需要刮治和临床以及影像学随访,而在大多数情况下,内生瘤既不需要治疗也不需要随访。在放射学上区分内生软骨瘤与ACTs仍然具有挑战性。因此,这项研究评估了计算机断层扫描(CT)和磁共振(MR)成像相结合的成像标准,以区分长骨中的内生软骨瘤和ACT。共有82名患者在我们的机构中连续出现ACT(23名,年龄52.7±18.8岁;14名女性)或内生软骨瘤(59名,年龄46.0±11.1岁;37名女性),进行了术前MR和CT成像以及随后的活检或/和手术切除,包括在这项研究中。所有病例均可进行组织病理学诊断。两位经验丰富的放射科医生评估了CT和MR图像的几种成像标准。如果骨内水肿(p=0.049),则ACT的可能性显着增加。在邻近的软组织内(p=0.006)或连续生长模式(p=0.077)存在,或者如果MR图像上没有脂肪截留(p=0.027)。分析CT影像特征,如果骨内扇贝形成2/3(p<0.001),则诊断为ACT的可能性显着增加。存在皮质渗透(p&lt;0.001)和骨扩张(p=0.002),如果在不到1/3的肿瘤中观察到基质钙化(p=0.013)。评估的所有其他成像标准对ACT或内生软骨瘤的可能性没有显着影响(p&gt;0.05)。总之,CT和MR成像都显示了暗示性体征,这些体征可以帮助充分区分长骨中的内生软骨瘤和ACTs,因此可以改善诊断,从而改善患者管理。然而,这些特征很少见,CT和MR成像特征的结合并没有显著提高诊断效能.
    The differentiation between the atypical cartilaginous tumor (ACT) and the enchondromas is crucial as ACTs require a curettage and clinical as well as imaging follow-ups, whereas in the majority of cases enchondromas require neither a treatment nor follow-ups. Differentiating enchondromas from ACTs radiologically remains challenging. Therefore, this study evaluated imaging criteria in a combination of computed tomography (CT) and magnetic resonance (MR) imaging for the differentiation between enchondromas and ACTs in long bones. A total of 82 patients who presented consecutively at our institution with either an ACT (23, age 52.7 ±18.8 years; 14 women) or an enchondroma (59, age 46.0 ± 11.1 years; 37 women) over a period of 10 years, who had undergone preoperative MR and CT imaging and subsequent biopsy or/and surgical removal, were included in this study. A histopathological diagnosis was available in all cases. Two experienced radiologists evaluated several imaging criteria on CT and MR images. Likelihood of an ACT was significantly increased if either edema within the bone (p = 0.049), within the adjacent soft tissue (p = 0.006) or continuous growth pattern (p = 0.077) were present or if the fat entrapment (p = 0.027) was absent on MR images. Analyzing imaging features on CT, the likelihood of the diagnosis of an ACT was significantly increased if endosteal scalloping >2/3 (p < 0.001), cortical penetration (p < 0.001) and expansion of bone (p = 0.002) were present and if matrix calcifications were observed in less than 1/3 of the tumor (p = 0.013). All other imaging criteria evaluated showed no significant influence on likelihood of ACT or enchondroma (p > 0.05). In conclusion, both CT and MR imaging show suggestive signs which can help to adequately differentiate enchondromas from ACTs in long bones and therefore can improve diagnostics and consequently patient management. Nevertheless, these features are rare and a combination of CT and MR imaging features did not improve the diagnostic performance substantially.
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  • 文章类型: Journal Article
    这篇综述研究了肌肉骨骼肿瘤的发现,这些肿瘤的影像学和/或组织病理学特征令人难以确定它们是否会表现出攻击行为。我们包括世界卫生组织(WHO)定义的中间肿瘤,和单一的低度恶性肿瘤,低级中央骨肉瘤,在影像学和组织学上模仿良性病变。中间肿瘤是一个广泛的类别,细分为仅有局部复发风险的肿瘤,和那些有远处肢体和肺转移风险的。困难的中间肌肉骨骼病变包括非典型软骨肿瘤/1级软骨肉瘤,非典型脂肪瘤瘤/1级脂肪肉瘤,和孤立性纤维瘤。我们回顾诊断标准,鉴别诊断,和监督建议。
    This review examines findings of musculoskeletal neoplasms whose equivocal imaging and/or histopathologic features make it difficult to determine if they will show aggressive behavior. We include both intermediate tumors as defined by the World Health Organization (WHO), and a single low-grade malignancy, low-grade central osteosarcoma, which mimics a benign lesion on imaging and histology. Intermediate tumors are a broad category and are subdivided into tumors that have risk of local recurrence only, and ones that have a risk of distant limb and pulmonary metastases. Difficult intermediate musculoskeletal lesions include atypical cartilaginous tumor/grade 1 chondrosarcoma, atypical lipomatous tumor/grade 1 liposarcoma, and solitary fibrous tumor. We review diagnostic criteria, differential diagnosis, and recommendations for surveillance.
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  • 文章类型: Journal Article
    BACKGROUND: Diagnostic performance, inter-observer agreement, and intermodality agreement between computed tomography (CT) and magnetic resonance imaging (MRI) in the depiction of the major distinguishing imaging features of central cartilaginous tumors have not been investigated.
    OBJECTIVE: To determine the inter-observer and intermodality agreement of CT and MRI in the evaluation of central cartilaginous tumors of the appendicular bones, and to compare their diagnostic performance.
    METHODS: Two independent radiologists retrospectively reviewed preoperative CT and MRI. Inter-observer and intermodality agreement between CT and MRI in the assessment of distinguishing imaging features, including lesion size, deep endosteal scalloping, cortical expansion, cortical disruption, pathologic fracture, soft tissue extension, and peritumoral edema, were evaluated. The agreement with histopathology and the accuracy of the radiologic diagnoses made with CT and MRI were also analyzed.
    RESULTS: A total of 72 patients were included. CT and MRI showed high inter-observer and intermodality agreements with regard to size, deep endosteal scalloping, cortical expansion, cortical disruption, and soft tissue extension (ICC = 0.96-0.99, k = 0.60-0.90). However, for the evaluation of pathologic fracture, MRI showed only moderate inter-observer agreement (k = 0.47). Peritumoral edema showed only fair intermodality agreement (k = 0.28-0.33) and moderate inter-observer agreement (k = 0.46) on CT. Both CT and MRI showed excellent diagnostic performance, with high agreement with the histopathology (k = 0.89 and 0.87, respectively) and high accuracy (91.7% for both CT and MRI).
    CONCLUSIONS: CT and MRI showed high inter-observer and intermodality agreement in the assessment of several distinguishing imaging features of central cartilaginous tumors of the appendicular bones and demonstrated comparable diagnostic performance.
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  • 文章类型: Case Reports
    软骨瘤是一种常见的良性软骨源性肿瘤,通常发生在手脚的短骨中。然而,当影响长骨时,很难排除低级软骨肉瘤,称为非典型软骨肿瘤(ACT),因为临床和放射学特征非常相似。本研究报告2例晚期膝骨关节炎,计划进行全膝关节置换术,影像学上有股骨远端病变,提示ACT/内生软骨瘤。我们认为,这些患者的治疗可能是关节成形术外科医生的挑战。这是因为可能难以确定骨关节炎膝关节的关节周围软骨肿瘤是否恶性并改变计划。在这份报告中,我们描述了我们治疗膝关节骨关节炎和股骨远端ACT/内生软骨瘤的方法。据我们所知,这个问题还没有在文献中讨论过。
    Enchondroma is a common benign chondrogenic tumor, which typically occurs in the short bones of hands and feet. However, when affecting the long bones, it is difficult to rule out the low-grade chondrosarcoma, called atypical cartilaginous tumor (ACT), because of the highly similar clinical and radiologic features. This study reports 2 patients with advanced knee osteoarthritis, scheduled for total knee arthroplasty, who had a distal femoral lesion on imaging suggestive of ACT/enchondroma. We believe that the treatment of these patients could be a challenge for arthroplasty surgeons. This is because it might be difficult to decide whether a periarticular chondral tumor of an osteoarthritic knee is malignant and changes the plan. In this report, we described our approach to address both knee osteoarthritis and ACT/enchondroma of the distal femur. To the best of our knowledge, this issue has not yet been discussed in the literature.
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  • 文章类型: Journal Article
    Surgical treatment of benign and low-grade malignant intramedullary chondroid lesions at the distal femur is not well analyzed compared to higher-grade chondrosarcomas. Localization at the distal femur offers high biomechanical risks requiring sophisticated treatment strategy, but scientific guidelines are missing. We therefore wanted to analyze a series of equally treated patients with intralesional resection and bone cement filling with and without additional osteosynthesis. Twenty-two consecutive patients could be included with intralesional excision and filling with polymethylmethacrylate bone cement alone (n = 10) or with compound bone cement osteosynthesis using a locking compression plate (n = 12). Clinical and radiological outcome was retrospectively evaluated including tumor recurrences, complications, satisfaction, pain, and function. Mean follow-up was 55 months (range 7-159 months). Complication rate was generally high with lesion-associated fractures both in the osteosynthesis group (n = 2) and in the non-osteosynthesis group (n = 2). All fractures occurred in lesions that reached the diaphysis. No fractures were found in meta-epiphyseal lesions. No tumor recurrence was found until final follow-up. Clinical outcome was good to excellent for both groups, but patients with additional osteosynthesis had significantly longer surgery time, more blood loss, longer postoperative stay in the hospital, more complications, more pain, less satisfaction, and worse functional outcome. Intralesional resection strategy was oncologically safe without local recurrences but revealed high risk of biomechanical complications if the lesion reached the diaphysis with an equal fracture rate no matter whether osteosynthesis was used or not. Additional osteosynthesis significantly worsened final clinical outcome and had more overall complications. This study may help guide surgeons to avoid overtreatment with additional osteosynthesis after curettage and bone cement filling of intramedullary lesions of the distal femur. Meta-epiphyseal lesions will need additional osteosynthesis rarely, contrary to diaphyseal lesions with considerable cortical thinning.
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