chondroblastoma

软骨母细胞瘤
  • 文章类型: Journal Article
    评估软骨母细胞瘤的多模态影像学特征。
    回顾性分析了2010年至2022年52例经组织病理学证实的软骨母细胞瘤的影像学特征。评估病变部位的射线照片,location,形态学,边距,基质矿化,皮质裂口,骨膜反应,偏心率,和关节下延伸。在MRI上评估T1,T2加权和对比后T1的外观,分析瘤周水肿和关节积液。
    患者平均年龄为18岁(10-57岁),男性占优势(M=39;F=13)。75%(n=39)例涉及未融合的骨骼,而25%(n=13)例涉及成熟的骨骼。阑尾骨骼占88.5%(n=46),轴向骨骼占11.5%(n=6),所有病例均涉及骨epi/骨epi等效。射线照相,所有病例均为明确的地理溶骨性病变,具有狭窄的过渡区,薄硬化边缘和分叶[56%(n=29)]或光滑[44%(n=23)]边缘。62%(n=32)的基质钙化明显为“蓬松/污迹”。软骨母细胞瘤出现等强度(83%,n=43)在T1MRI上具有特征性低信号和高强度病灶(67%,n=35)在T2加权图像和对比增强后[异质小叶(88%,n=46)或间隔模式(12%,n=6)],所有三个病变均显示病灶周围水肿。直到最后一次随访(平均:71个月),我们研究中的软骨母细胞瘤病例均未发生转移。
    软骨母细胞瘤具有独特的影像学表现,通常与大多数其他软骨良性病变不同,这是由于MRI上特征性的低T2信号和相关的弥漫性水肿。
    UNASSIGNED: To evaluate the multimodality imaging features of chondroblastoma.
    UNASSIGNED: Retrospective analysis of imaging features of 52 cases of histopathologically proven chondroblastoma from 2010 to 2022 was performed. Radiographs were evaluated for lesion site, location, morphology, margins, matrix mineralization, cortical breach, periosteal reaction, eccentricity, and subarticular extension. Appearance on T1, T2 weighted and post-contrast T1 was evaluated on MRI, with analysis of peritumoral edema and joint effusion.
    UNASSIGNED: Mean patient age was 18 years (10-57 years) with male preponderance (M = 39; F = 13). 75% (n = 39) cases involved an unfused skeleton and 25% (n = 13) affected a mature skeleton. Appendicular skeleton was involved in 88.5% (n = 46) cases and axial skeleton was involved in 11.5% (n = 6) cases with all cases involving epiphysis/epiphyseal equivalent. Radiographically, all cases were well-defined geographic osteolytic lesions with a narrow zone of transition, thin sclerotic rim and lobulated [56% (n = 29)] or smooth [44% (n = 23)] margins. Matrix calcification appreciable in 62% (n = 32) cases was \'fluffy/smudgy\'. Chondroblastoma appeared isointense (83%, n = 43) on T1 MRI with characteristically low signal and hyperintense foci within (67%, n = 35) on T2-weighted images and post-contrast enhancement [heterogeneous lobular (88%, n = 46) or septal pattern (12%, n = 6)] with all barring three lesions showing perilesional edema. None of the cases of chondroblastoma in our study developed metastasis till last follow-up (mean: 71 months).
    UNASSIGNED: Chondroblastoma has distinctive imaging appearance and is often unlike majority other cartilaginous benign lesions due to characteristic low T2 signal on MRI and associated exuberant perilesional edema.
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  • 文章类型: Editorial
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  • 文章类型: Journal Article
    背景:软骨母细胞瘤(CB),一种产生软骨细胞的罕见良性骨肿瘤,通常在儿童和年轻人的骨phy或骨上发展。这些罕见肿瘤的治疗是复杂的。标准治疗方案包括用局部佐剂刮除和骨移植或水泥应用。作者检查了38个CBs以确定局部复发的危险因素,并发症,骨粘连刮治后的功能结局。
    方法:回顾性分析了2000年1月至2021年6月在我院就诊的22名10至17岁的女孩和16名男孩的软骨母细胞瘤。临床数据,射线照相图像,组织学结果,治疗,功能结果,并检查局部复发率-手术治疗涉及全肿瘤刮除,其次是骨移植和辅助技术。局部复发也有报道。
    结果:最常见的部位是股骨近端。受累部位包括10例(26.3%)股骨近端,胫骨近端8例(20.8%),肱骨5例(13.2%),胫骨远端4例(10.5%),股骨远端3例(7.9%),股上区域3例(7.9%),距骨1例(2.6%),跟骨1例(2.6%),肩胛骨1例(2.6%),腰椎1例(2.6%),1例(2.6%)患者的髂骨。平均随访时间为144.2个月(24至276个月)。局部复发率为7.9%。肌肉骨骼肿瘤协会(MSTS)的平均评分为28.3分(17至30)。出现时症状的平均持续时间为5.8(范围,1至28)个月。
    结论:侵袭性刮除和植骨导致大多数儿科患者的局部控制和良好的预后。在相对较小比例的案件中,由于生长板损伤和晚期诊断,可发生长期并发症和复发。在儿科诊所收治的疼痛患者中,常伴有局部水肿和关节积液,通过先进的放射扫描进行早期检测(X射线,CT,或MRI)可以防止诊断延迟。
    BACKGROUND: Chondroblastoma (CB), a rare benign bone tumor that produces chondrocytes, often develops in the epiphysis or apophysis of children and young adults. The treatment of these rare tumors is complex. The standard treatment protocol involves curettage with local adjuvants and bone graft or cement application. The authors examined 38 CBs to determine risk factors for local recurrence, complications, and functional outcomes following epiphyseal curettage.
    METHODS: Twenty-two girls and sixteen boys aged 10 to 17 years with histologically confirmed chondroblastoma who arrived at our hospital between January 2000 and June 2021 were reviewed retrospectively. Clinical data, radiographic images, histological results, treatment, functional outcomes, and the local recurrence rate were examined-surgical treatment involved total tumor curettage, followed by bone grafting and adjuvant techniques. Local recurrences have also been reported.
    RESULTS: The most frequently affected site was the proximal femur. Sites of involvement included the proximal femur in 10 (26.3%) cases, the proximal tibia in 8 (20.8%), the humerus in 5 cases (13.2%), the distal tibia in 4 cases (10.5%), the distal femur in 3 cases (7.9%), the supracetabular region in 3 cases (7.9%), the talus in 1 case (2.6%), the calcaneus in 1 case (2.6%), the scapula in 1 case (2.6%), the lumbar spine in 1 case (2.6%), and the iliac bone in 1 (2.6%) patient. The mean follow-up was 144.2 months (24 to 276). The local recurrence rate was 7.9%. The mean Musculoskeletal Tumor Society (MSTS) score was 28.3 points (17 to 30). The mean duration of symptoms at presentation was 5.8 (range, 1 to 28) months.
    CONCLUSIONS: Aggressive curettage and bone grafting resulted in local control and good outcomes in most pediatric patients. In a relatively small proportion of cases, long-term complications and recurrence can occur due to growth plate damage and late diagnosis. In patients admitted to the pediatric clinic with pain, which is often accompanied by localized edema and joint effusion, early detection via advanced radiological scans (X-ray, CT, or MRI) may prevent delays in diagnosis.
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  • 文章类型: Journal Article
    软骨母细胞瘤转移,虽然罕见,代表骨肿瘤的临床上有意义且特别重要的方面。了解其流行病学特征,病理特征,和治疗方式,尽管频率不高,全面的患者管理势在必行。这篇综述旨在阐明流行病学,分子机制,诊断挑战,以及与软骨母细胞瘤转移相关的治疗策略。模式,预后因素,并通过对病例研究和临床报告的分析探讨治疗结果.值得注意的是,我们强调了旨在改善患者结局的新兴治疗观点.据我们所知,以前没有累积处理这些问题的审查,突出了现有学术文献中的显著差距。通过阐明软骨母细胞瘤转移的细微差别,这篇综述有助于提高该领域的知识,并为改善患者护理的临床决策提供信息.
    Chondroblastoma metastasis, though rare, represents a clinically significant and notably important aspect of bone tumors. Understanding its epidemiological characteristics, pathological features, and treatment modalities, despite its infrequency, is imperative for comprehensive patient management. This review aims to elucidate the epidemiology, molecular mechanisms, diagnostic challenges, and therapeutic strategies associated with chondroblastoma metastasis. The patterns, prognostic factors, and treatment outcomes were explored through an analysis of case studies and clinical reports. Notably, we highlighted emerging therapeutic perspectives aimed at improving patient outcomes. To the best of our knowledge, there has been no previous review addressing these matters cumulatively, highlighting a significant gap in the existing scholarly literature. By shedding light on the nuances of chondroblastoma metastasis, this review contributes to the advancement of knowledge in this field and informs clinical decision-making for improved patient care.
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  • 文章类型: Case Reports
    软骨母细胞瘤是一种罕见的良性软骨肿瘤,约占骨肿瘤的1%。但在极少数情况下,它可能与肺转移有关,导致预后不良和死亡。在这里,我们报道了一例19岁男性患者,表现为侵袭性肱骨近端软骨母细胞瘤和双侧肺转移.患者接受了广泛的局部切除术,部分转移瘤切除术,还有Denosumab.Denosumab治疗可有效控制转移进展并预防局部复发。
    Chondroblastoma is a rare benign cartilaginous tumor that accounts for approximately 1% of bone tumors, but it can be associated with lung metastasis in extremely rare cases, leading to a poor prognosis and death. Herein, we report the case of a 19-year-old male patient who presented with an aggressive chondroblastoma of the proximal humerus and bilateral lung metastasis. The patient was treated with wide local resection, partial metastasectomy, and denosumab. Denosumab treatment was effective in controlling metastatic progression and preventing local recurrence.
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  • 文章类型: Journal Article
    软骨母细胞瘤目前被描述为良性骨肿瘤,组织病理学特征为其经典特征,包括成软骨细胞,粉红软骨,和可变数量的破骨细胞样巨细胞,伴有营养不良性钙化灶。尽管有报道称复发性和转移性软骨母细胞瘤,恶性软骨母细胞瘤极为罕见,有点争议性。一名35岁的男性,脚踝有一个15年的肿块。成像揭示了一种溶解性破坏性病变,涉及胫骨和腓骨的下端,带有软组织成分,表示非典型/“令人担忧”的功能。活检的显微镜检查显示软骨母细胞瘤的明显病灶,过渡到高级肉瘤区域,包括多形性细胞,有丝分裂增加,和突出的基质透明化。免疫组织化学,整个肿瘤的H3K36M呈阳性,而DOG1突出了软骨母细胞瘤的区域。SATB2突出了高级肉瘤的区域,保留软骨母细胞瘤的区域。此外,高级别肉瘤区域显示多灶性结蛋白免疫染色。提供了软骨母细胞瘤恶性转化的诊断。患者未能接受进一步治疗,不幸死亡8个月,诊断后。本文描述了在少数报道的肿瘤中使用H3K36M免疫染色的恶性软骨母细胞瘤的概念演变。
    Chondroblastoma is currently described as a benign bone tumor, histopathologically characterized by its classical features including chondroblasts, pink cartilage, and a variable number of osteoclast-like giant cells with foci of dystrophic calcification. Although recurrent and metastasizing chondroblastomas are reported, a malignant chondroblastoma is exceedingly rare and somewhat a contentious entity. A 35-year-old male presented with a lump in his ankle of 15 years\' duration. Imaging disclosed a lytic destructive lesion involving the lower ends of the tibia and fibula with a soft tissue component, indicative of atypical/\"worrisome\" features. Microscopic examination of the biopsy revealed distinct foci of chondroblastoma, transitioning to areas of high-grade sarcoma, including pleomorphic cells, increased mitoses, and prominent stromal hyalinization. Immunohistochemically, the entire tumor was positive for H3K36M, while DOG1 highlighted the areas of chondroblastoma. SATB2 highlighted the areas of high-grade sarcoma, sparing the areas of chondroblastoma. Additionally, the areas of a high-grade sarcoma showed multifocal desmin immunostaining. A diagnosis of a malignant transformation in a chondroblastoma was offered. The patient defaulted to further treatment and unfortunately died 8 months, post-diagnosis. The conceptual evolution of a malignant chondroblastoma with H3K36M immunostaining in the few reported tumors is described herewith.
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  • 文章类型: Journal Article
    对儿童股骨远端髁间切迹的软骨母细胞瘤的骨phy入路不能提供足够的暴露,因此需要去除大量未受影响的骨骼以暴露病变。在这项研究中,我们比较了功能结果,局部复发,以及通过髁间或骨phy入路治疗股骨远端软骨母细胞瘤的手术并发症。
    对30例股骨远端软骨母细胞瘤患儿进行回顾性分析,这些患儿接受了骨内刮除和植骨治疗。16例患者采用髁间入路(A组),14例采用骨phy入路(B组)。使用肌肉骨骼肿瘤协会(MSTS)评分系统和Sailhan的功能标准评估肢体功能。
    在最后的随访中,A组的平均MSTS评分为29.1(SD0.9),B组的平均MSTS评分为26.7(SD1.5)(p=0.006).根据Sailhan的标准,A组14例(87.5%)和2例(12.5%)患者膝关节功能良好,B组8例(57.1%)和6例(42.9%)患者,分别(p=0.062)。A组1例患者(6.2%)和B组4例患者(28.6%)的病变复发。A组1例患者(6.2%)和B组6例患者(42.8%)的肢体缩短>1cm更好的肢体功能,较低的复发率,和较低的速率的骨损害和关节退化。
    UNASSIGNED: The epiphyseal approach to a chondroblastoma of the intercondylar notch of a child\'s distal femur does not provide adequate exposure, thereby necessitating the removal of a substantial amount of unaffected bone to expose the lesion. In this study, we compared the functional outcomes, local recurrence, and surgical complications of treating a chondroblastoma of the distal femoral epiphysis by either an intercondylar or an epiphyseal approach.
    UNASSIGNED: A total of 30 children with a chondroblastoma of the distal femur who had been treated by intraregional curettage and bone grafting were retrospectively reviewed. An intercondylar approach was used in 16 patients (group A) and an epiphyseal approach in 14 (group B). Limb function was assessed using the Musculoskeletal Tumor Society (MSTS) scoring system and Sailhan\'s functional criteria.
    UNASSIGNED: At final follow-up, the mean MSTS score was 29.1 (SD 0.9) in group A and 26.7 (SD 1.5) in group B (p = 0.006). According to Sailhan\'s criteria, the knee function was good and fair in 14 (87.5%) and two (12.5%) patients of group A, and eight (57.1%) and six (42.9%) patients of group B, respectively (p = 0.062). The lesion had recurred in one patient (6.2%) in group A and four patients (28.6%) in group B. Limb shortening > 1 cm was recorded in one patient (6.2%) from group A and six patients (42.8%) from group B. Joint degeneration was noted in one patient from group A and three patients from group B.
    UNASSIGNED: An intercondylar approach to a chondroblastoma of the middle two-quarters of the distal femoral epiphysis results in better outcomes than a medial or lateral epiphyseal approach: specifically, better limb function, a lower rate of recurrence, and a lower rate of physeal damage and joint degeneration.
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  • 文章类型: Case Reports
    软骨母细胞瘤是一种良性产生软骨的骨病变,典型地发生在长骨的骨phy区。最典型的位置是肱骨近端,股骨近端,股骨远端,和胫骨近端.这种疾病没有药物治疗;传统上,必须通过病灶内刮除和植骨治疗。一名15岁的女性患者出现慢性膝关节疼痛,没有外伤史。临床检查显示最大屈曲时的深度压痛和15度伸展滞后,膝关节完全屈曲。X线片和膝关节MRI显示中线胫骨近端后部有病变,高度怀疑软骨母细胞瘤。CAT引导活检未显示任何恶性肿瘤的证据。使用小切口在内窥镜可视化的辅助下进行病灶内刮治,骨移植替代物重建了缺损。可以在具有挑战性的位置考虑内窥镜辅助刮除良性骨病变,效果良好。
    Chondroblastoma is a benign cartilage-producing bone lesion that characteristically occurs in the epiphyseal region of long bones. The most typical locations are the proximal humerus, proximal femur, distal femur, and proximal tibia. There is no medical treatment for the disease; classically, it must be treated by intralesional curettage and bone grafting. A 15-year-old female patient presented with chronic knee pain with no antecedent history of trauma. Clinical examination showed deep tenderness on maximum flexion and 15 degrees extension lag with full knee flexion. Plain radiographs and knee MRI showed a lesion in the posterior part of the proximal tibia on the midline, highly suggestive of chondroblastoma. CAT-guided biopsy did not show any evidence of malignancy. Intralesional curettage assisted by endoscopic visualization was done using a small incision, and a bone graft substitute reconstructed the defect. Endoscopic-assisted curettage of benign bone lesions can be considered in challenging locations with good results.
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  • 文章类型: Journal Article
    历史上,由于临床和影像学发现重叠,导致几种肿瘤难以分离,因此对骨骼中富含巨细胞的肿瘤的诊断一直具有挑战性。特别是当活检材料是有限的。然而,随着骨巨细胞瘤(GCTB)和软骨母细胞瘤中驱动组蛋白突变的发现,以及动脉瘤样骨囊肿的USP6重排,病理学家现在有客观的辅助工具来帮助分离几种组织学相似的富含巨细胞的肿瘤。此外,对组蛋白突变的认识使病理学家能够重新审视几个实体,如“恶性软骨母细胞瘤,并进一步加深了我们对动脉瘤样骨囊肿样变化等现象的理解,“原认为”继发性动脉瘤样骨囊肿。\"在这里,考虑了骨肿瘤中组蛋白突变检测的进展;审查了组蛋白抗体的敏感性和特异性;并提供了使用这些辅助检测的实用指南.
    Historically, the diagnosis of giant cell-rich neoplasms arising in bone has been challenging owing to overlapping clinical and radiographic findings resulting in the difficult separation of several neoplasms, particularly when biopsy material is limited. However, with the discovery of the driver histone mutations in giant cell tumor of bone (GCTB) and chondroblastoma, as well as USP6 rearrangements in aneurysmal bone cyst, pathologists now have objective ancillary tools to aid in the separation of several histologically similar giant cell-rich neoplasms. Furthermore, the recognition of histone mutations has allowed pathologists to revisit several entities, such as \"malignant chondroblastoma,\" and furthered our understanding of phenomena such as \"aneurysmal bone cyst-like change,\" formerly recognized as \"secondary aneurysmal bone cyst.\" Herein, the evolution of testing for histone mutations in bone tumors is considered; the sensitivity and specificity of the histone antibodies is reviewed; and a practical guide for the use of these ancillary tests is offered.
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  • 文章类型: Case Reports
    背景:软骨粘液样纤维瘤(CMF)是一种罕见的,良性骨肿瘤主要发生在生命的第二个和第三个十年,更常见的是男性。作为GRM1的肿瘤特异性基因重排的结果的GRM1的过表达最近被报道为用于CMF的组织病理学诊断的有用的免疫组织化学标记。然而,细胞学标本GRM1染色的有用性尚未被评估。在这份报告中,描述了两例CMF的细胞学发现和GRM1免疫细胞化学。
    方法:病例1是一个患有肋骨肿瘤的15岁女孩。影像学发现提示良性神经源性肿瘤,例如神经鞘瘤。在两年的时间里,肿瘤的大小增加,并被切除。病例2是一个14岁的男孩,他的左第一脚趾有一个跖骨肿瘤。影像学检查结果怀疑是良性肿瘤病变。活检结果提示良性肿瘤,患者接受了肿瘤切除术。细胞学上,在这两种情况下,肿瘤细胞主要是纺锤形或星状,具有粘液样软骨粘液样背景基质和多核巨细胞,这些基质用Giemsa染色变色。病例2的细胞异型性比病例1的细胞异型性更加突出。两种情况下GRM1的免疫细胞化学染色均为阳性。
    结论:由于细胞学结果的重叠,通常很难将CMF与软骨母细胞瘤和2级软骨肉瘤区分开。GRM1的免疫细胞化学染色可能支持CMF的诊断,再次使用巴氏染色的标本是适用的。本病例进一步证明了将CMF与其他模拟肿瘤如软骨母细胞瘤和2级软骨肉瘤区分开来的困难。在这种情况下,GRM1的免疫细胞化学适用于诊断过程,通过重复使用Papanicolaou染色的标本可以增强其价值。
    BACKGROUND: Chondromyxoid fibroma (CMF) is a rare, benign bone tumor that occurs predominantly in the second and third decades of life, more frequently in males. Overexpression of GRM1 as a consequence of tumor-specific gene rearrangement of GRM1 has recently been reported as a useful immunohistochemical marker for histopathological diagnosis of CMF. However, the usefulness of GRM1 staining of cytology specimens has not yet been evaluated. In this report, the cytological findings and GRM1 immunocytochemistry of two cases of CMF are described.
    METHODS: Case 1 was a 15-year-old girl with a rib tumor. Imaging findings suggested a benign neurogenic tumor such as schwannoma. The tumor had increased in size over a 2-year period and was resected. Case 2 was a 14-year-old boy with a metatarsal tumor involving his left first toe. Imaging findings were suspicious of a benign neoplastic lesion. Biopsy findings suggested a benign tumor, and the patient underwent tumor resection. Cytologically, in both cases the tumor cells were predominantly spindle-shaped or stellate, with a myxoid to chondromyxoid background matrix and multinucleated giant cells, and these matrices were metachromatic with Giemsa staining. Cellular atypia was more accentuated in case 2 than in case 1. Immunocytochemical staining for GRM1 was positive in both cases.
    CONCLUSIONS: Due to the overlap in cytological findings, it is often difficult to differentiate CMF from chondroblastoma and chondrosarcoma grade 2. Immunocytochemical staining for GRM1 may support the diagnosis of CMF, and the reuse of Papanicolaou-stained specimens is applicable. The present cases further demonstrated the difficulty of differentiating CMF from other mimicking tumors such as chondroblastoma and chondrosarcoma grade 2. In such instances, immunocytochemistry for GRM1 is applicable to the diagnostic process, the value of which is strengthened by reusing Papanicolaou-stained specimens.
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