Parasellar chondrosarcoma in a patient with Ollier's disease

Summary Cartilaginous intracranial neoplasms are rare and are located most commonly at the base of the skull in the parasellar region. They may occur with similar lesions in the rest of the skeleton (Ollier's disease) or present with multiple enchondromas and soft tissue hemangiomas (Maffuci's syndrome).     

Comparison of allelic losses in chondroblastoma and primary chondrosarcoma of bone and correlation with fluorescence in situ hybridization analysis

Chondroblastoma (CBL) is a benign neoplasm of bone for which the genomic characteristics remain unclear. We compared the status of allelic losses of CBL with that seen in a set of chondrosarcomas (CS) to determine whether differences in their natural history and behavior are also reflected genetically. Eleven cases of CBL and 10 cases of CS of different grades were included. Tumors were subjected to microdissection and polymerase chain reaction using 17 markers located near genes on chromosomes 5, 9, 11, 13, 17, and 19. The selected chromosomes are known to be involved in several mesenchymal neoplasms. Fluorescence in situ hybridization was also performed on tumors displaying allelic losses, with dual-color probes for 9p, 17p, and 13q. Fractional allelic losses per gene ranged from 18.2% to 63.7% in CBLs and from 28.6% to 66.7% in CSs. Loss of heterozygosity (LOH) of 5q, 9p, 11p, 13q, and 19q occurred in both CBLs and CSs. Loss of heterozygosity of 17p (p53 locus) occurred in 7 of 11 CBLs and in only 1 case of recurrent CS. The pattern of allelic loss was similar in low-grade CSs and CBLs. Loci with LOH in both tumor types suggest possible involvement of the genes p53, RB1, CDKN2/p16, ERC, and XRCC in tumorigenesis. Overall correlation between LOH and fluorescence in situ hybridization results was 90% with 17p13, 80% with 9p, and 60% with 13q. The role of p53 in CBL is uncertain; however, given the benign behavior of this tumor, it is probably unrelated to tumor progression.     

近端型上皮样肉瘤1例

患者男,44岁,偶然发现左下腹包块半个月,近期无发热及感染病史。查体:左下腹触及3cm×2cm肿块,质韧,不活动,无触痛,局部无红肿。无其他症状及阳性体征,常规肿瘤标志物均阴性。盆腔CT:主动脉分叉水平以下左侧腰大肌前内缘见不规则长条状软组织影,沿腰大肌走行,向下延续至腹股沟     

软骨肉瘤的影像学诊断

目的 探讨不同分型软骨肉瘤的影像学表现。方法 回顾性分析手术病理证实的26例软骨肉瘤的影像学资料。结果 ①普通髓腔型:X线平片检查6例,表现为溶骨与硬化混合的外观,位于长管状骨者可见骨内膜的扇贝样改变。CT检查8例,7例示膨胀性骨质破坏,5例钙化明显,3例钙化轻微。MR检查9例,T1WI为等、低信号,T2WI 8例呈明显高信号,其中6例可见低信号分隔。②间叶型:X线平片检查2例,1例肿瘤位于骨内,呈轻度膨胀性改变,1例位于骨外,病灶密集钙化。CT检查7例,6例位于骨骼系统外,4例钙化明显,1例钙化局限,1例未见明显钙化。MR检查5例,4例骨外型T2信号特点取决于瘤体内钙化的形态及范围,或呈胡椒面样表现或表现为高信号包绕低信号。③去分化型:CT 检查3例,均示钙化,2例尚可见絮状成骨样改变。MR检查2例,T2WI上均为高低混杂信号。④皮质旁型:X线平片检查2例,1例可见相应部位异常钙化。CT检查3例,均显示分叶状外形的软组织肿块贴近骨皮质,2例见基质轻微钙化。MR检查3例,2例可见髓腔侵犯。增强扫描普通髓腔型及皮质旁型呈边缘及间隔强化,间叶型呈弥漫性强化,去分化型则同时可见边缘强化及弥漫性强化。结论 普通髓腔型软骨肉瘤影像学表现较具特征性。结合钙化特点、信号特征及增强后表现可提示诊断间叶型及去分化型软骨肉瘤。     

Primary chondrosarcoma of the liver: case report and literature review

We present a rare case of primary chondrosarcoma of the liver in a 57-year-old man with pre-existing hepatitis B virus-related chronic hepatitis.The MRI scans showed a huge cystic-solid occupation of 18 cm×17 cm×11 cm in the right hepatic lobe.The tumor was completely resected,and the histological findings identified low-grade cartilaginous component with typical ring-and-arc chondroid matrix mineralization.Immunohistochemically,the neoplastic cells were positive for vimentin and S-100 protein.The patien...     

基质金属蛋白酶-9在软骨肉瘤中的表达及临床意义

目的:探讨基质金属蛋白酶-9(MMP-9)蛋白在软骨肉瘤中的表达及临床意义。方法:应用SP免疫组化法检测MMP-9蛋白在28例软骨肉瘤、12例良性软骨肿瘤及10例正常软骨中的表达。结果:软骨肉瘤中MMP-9蛋白表达阳性百分比为71.43%(20/28),高于良性软骨肿瘤组及正常软骨组,且在转移组高于未转移组,差异具有显著性。结论:MMP-9蛋白在软骨肉瘤的形成和发展中起不同程度的作用,其对软骨肉瘤组织学分级、恶性进展和预后判断具有重要的价值。     

Immunohistochemical localization of steroid receptor coactivators in chondrosarcoma: An in vivo tissue microarray study

Chondrosarcoma is the second most common type of primary bone malignancy following up osteosarcoma, characterized by resistance to conventional chemotherapeutic agents and radiation regimens. The p160 family members steroid receptor coactivator-1 and -3 (SRC-1 and SRC-3) have been implied in the regulation of cancer growth, migration, invasion, metastasis and chemotherapeutic resistance; but we still lack detailed information about the levels of SRCs in chondrosarcoma. In this study, expression of SRC-1 and SRC-3 in chondrosarcoma was examined by immunohistochemistry with tissue microarrays; the four score system (0, 1, 2 and 3) was used to evaluate the staining. The results showed that there were no gender-, site- or age-differences regarding the expression of SRC-1 or SRC-3 (p > 0.05); organ (bone or cartilage) -differences were only detected for SRC-1 but not SRC-3 (p < 0.05). Significant higher levels of SRC-1 and SRC-3 were detected in MDC and PDC when compared to WDC. Our study clearly demonstrated differentiation-dependant expression of SRC-1 and SRC-3 in chondrosarcoma, may be novel targets for the prognosis and/or treatment of chondrosarcoma, would have opened a new avenue and established foundation for studying chondrosarcoma.     

The changing face of central chondrosarcoma of bone. One UK-based orthopaedic oncology unit’s experience of 33 years referrals

AimTo ascertain the changing incidence over time of the three commonest primary sarcomas of bone. Data obtained with particular reference to central chondrosarcoma from the annual referral rate to a large UK-based specialist orthopaedic oncology unit. To discuss how the “barnyard pen” analogy of cancers previously applied to certain commoner cancers can also be applicable to central chondrosarcoma (CS) of bone.Materials and methodsA retrospective review was conducted of a computerised database identifying all central cartilage tumours (CCT) of bone, including enchondroma and CS subtypes, between 1985 and 2018. These were compared with the referrals of the other two commonest primary sarcomas of bone, osteosarcoma and Ewing sarcoma.ResultsThere was a total of 1507 CS showing a 68% overall increase in annual referral rate/incidence over the study period. 68% cases were the borderline malignant lesions now known as atypical cartilaginous tumour (ACT). The annual referral rate/incidence of this entity increased by 194% over the 30 years. Whereas, the annual referral rate/incidence for osteosarcoma and Ewing sarcoma was static for the past 20 years.ConclusionThe annual incidence of central CS of bone showed a marked increase over the 33-year period as compared with both osteosarcoma and Ewing sarcoma. This is especially in the ACT category and is thought to be due to the increased provision of MRI scanning flagging up a rise in incidental findings. The spectrum of CCTs from benign to highly malignant elegantly fits the “barn yard” pen analogy and could prove useful as an explanatory tool for patients and clinicians unfamiliar with these diseases.     

Morphological typing of chondrosarcoma: A study of 94 cases

Summary Ninety-four chondrosarcomas of the Hamburg Bone Tumour Registry were reviewed in a retrospective study. The purpose of this study was to examine the morphological characteristics of different types of chondrosarcomas and to describe distinctive features of location, the age distribution and the male to female ratio. Central chondrosarcomas can be divided into classical chondrosarcomas, dedifferentiated chondrosarcomas, mesenchymal chondrosarcomas and clear-cell chondrosarcomas. Five periosteal chondrosarcomas were represented. Classical chondrosarcomas and clear-cell chondrosarcomas show a significant predominance of males; no sex predilection was seen in dedifferentiated and mesenchymal chondrosarcomas. Nearly 60% of classical and mesenchymal chondrosarcomas occur in the trunk. Eighty-five percent of dedifferentiated chondrosarcomas are located in the long bones of the limbs. Clear-cell chondrosarcomas arise in the proximal part of the femur. There is a marked predilection for mesenchymal chondrosarcomas in the second and third decades of life. The average age of patients with classical chondrosarcomas was 54 years, but clear-cell chondrosarcomas occur 10 years earlier and dedifferentiated chondrosarcomas 10 years later. Characteristically, classical chondrosarcomas produce a pure chondroid matrix with variable differentiation of tumour chondrocytes. The most important histological feature of the defifferentiated chondrosarcoma is the close association of two different cellular components. One of these consists of cartilage, which is generally well differentiated. In most of our cases the second component showed features of osteosarcoma (50%). Mesenchymal chondrosarcoma is characterized by concentric infiltration of cartilage islands by small tumour cells. Clear-cell chondrosarcomas show regions of cartilaginous tumour and areas of closely packed, glycogen-rich, large tumour cells with distinct boundaries. Osteoid formation and multinucleated giant cells are present in clear-cell areas. Knowledge of this group of tumours is indispensable for correct histological diagnosis and typing and is important in the design of surgical therapy and the prediction of biological behaviour.Dedicated to Professor D. Scifert on the occasion of his 70th birthday.