Malignant Chondroid Matrix Bone Tumors

Author(s):  
Benjamin D. Levine ◽  
Leanne L. Seeger

Chapter 55 discusses malignant chondroid matrix bone tumors. Chondrosarcoma is a malignant bone tumor of cartilage origin. It represents the third most common primary malignant bone tumor, after multiple myeloma and osteosarcoma. Conventional intramedullary chondrosarcoma is its most common subtype, with rarer subtypes that include clear cell, mesenchymal, dedifferentiated, and periosteal chondrosarcoma. Secondary chondrosarcoma represents a lesion that arises in a preexisting benign chondroid lesion (enchondroma or osteochondroma). Chondrosarcomas demonstrate some specific imaging features that can improve diagnostic accuracy and help guide clinical management. Histological grade of central chondrosarcoma usually dictates surgical management. As with other sarcomas, outcome of chondrosarcoma depends on histological grade, surgical margins, and staging.

2014 ◽  
Vol 21 (3) ◽  
pp. 378-385
Author(s):  
Daniel K. Fahim ◽  
Claudio E. Tatsui ◽  
Dima Suki ◽  
Joy Gumin ◽  
Frederick F. Lang ◽  
...  

Object There is currently no reproducible animal model of human primary malignant bone tumors in the spine to permit laboratory investigation of the human disease. Therefore, the authors sought to adapt their previously developed orthotopic model of spinal metastasis to a model for primary malignant bone tumors of the spine. Methods A transperitoneal surgical approach was used to implant osteosarcoma (Krib-1) into the L-3 vertebral body of nude mice via a drill hole. Motor function was evaluated daily using the previously validated qualitative key milestones of tail dragging, dorsal stepping, hindlimb sweeping, and paralysis. A subset of these animals was euthanized upon reaching the various milestones, and the spines were removed, sectioned, and stained. The degree of spinal cord compression was correlated with the occurrence of milestones and assessed by a ratio between the neural elements divided by the area of the spinal canal. Another subset of animals received stably transfected Krib-1 cells with the luciferase gene, and bioluminescence was measured at 10, 20, and 30 days postimplantation. Results Osteosarcoma xenografts grew in all animals according to a reliable and reproducible time course; the mean time for development of behavioral milestones was noted in relation to the day of implantation (Day 1). Tail dragging (Milestone 1) occurred on Day 19.06 (95% CI 16.11–22.01), dorsal stepping (Milestone 2) occurred on Day 28.78 (95% CI 26.79–30.77), hindlimb sweeping (Milestone 3) occurred on Day 35.61 (95% CI 32.9–38.32), and paralysis of the hindlimb (Milestone 4) occurred on Day 41.78 (95% CI 39.31–44.25). These clinically observed milestones correlated with increasing compression of the spinal cord on histological sections. The authors observed a progressive increase in the local bioluminescence (in photons/cm2/sec) of the implanted level over time with a mean of 2.17 (range 0.0–8.61) at Day 10, mean 4.68 (range 1.17–8.52) at Day 20, and mean 5.54 (range 1.22–9.99) at Day 30. Conclusions The authors have developed the first orthotopic murine model of a primary malignant bone tumor in the spine, in which neurological decline reproducibly correlates with tumor progression as evidenced by pathological confirmation and noninvasive bioluminescence measurements. Although developed for osteosarcoma, this model can be expanded to study other types of primary malignant bone tumors in the spine. This model will potentially allow animal testing of targeted therapies against specific primary malignant tumor types.


2020 ◽  
Author(s):  
Lucen Jiang ◽  
Jianghuan Liu ◽  
Qingzhu Wei ◽  
Yiyang Wang

Abstract Background Karyopherin α 2 (KPNA2), a member of the Karyopherin α family, has been observed in several cancers but lack substantial investigation in malignant bone tumors. The purpose of the current study was to evaluate KPNA2 expression level and its utility as a novel diagnostic biomarker in osteosarcomas and their malignant bone tumor mimickers, such as chondrosarcomas and Ewing sarcomas.Method We investigated the expression of KPNA2 protein by immunohistochemistry on paraffin embedded surgical specimens from 217 patients with malignant and benign tumors of bone, including 81 osteosarcomas, 42 chondrosarcomas, 9 Ewing sarcomas, 28 osteoid osteoma, 20 osteochondroma and 37 Chondroblastoma. Immunoreactivity was scored semi quantitatively based on stain extent and intensity.Results Seventy one of 81 (87.7%) osteosarcomas, zero of 42 (0%) chondrosarcomas and one of 9 (11.1%) Ewing sarcomas showed immunoreactivity for KPNA2. Negative KPNA2 expression was observed in all of benign bone tumors. Much more positive expression of KPNA2 was found in osteosarcomas as compared with chondrosarcomas and Ewing sarcomas. The sensitivity and specificity of KPNA2 immuno-expression for osteosarcoma was 87.7% and 100%, respectively. In several subtypes of osteosarcomas, immunohistochemical expression of KNA2 was more frequent in osteoblastic (94.5%), with 39 (70.9%) showing strong-intensity staining. KPNA2 positivity was observed in eleven of 13 (84.6%) chondroblastic, three of 6 (50%) fibroblastic, three of 4 (75%) telangiectatic and two of 3 (66.7%) giant cell-rich osteosarcoma. Stronger-intensity staining was observed in osteoblastic osteosarcoma.Conclusion KPNA2 is most frequently expressed in osteosarcomas, particularly in osteoblastic and chondroblastic tumors, but is rarely positive in chondrosarcomas and Ewing sarcomas. This feature may have diagnostic value since it is very useful for distinguishing between osteosarcomas and other bone sarcomas mimickers. This report supports KPNA2 as a novel marker for the diagnosis of osteosarcoma.


2021 ◽  
Author(s):  
Xing Zhou ◽  
Jun-Liang Zhang ◽  
Meng-Han Chang ◽  
Gentao Fan ◽  
Xiao-Zhou Liu ◽  
...  

Osteosarcoma is the most frequent primary malignant bone tumor composed of mesenchymal cells producing osteoid and immature bone. Sensitive detection of telomerase plays a pivotal role for the early diagnosis...


2020 ◽  
Author(s):  
Ningfeng Guo ◽  
Yaojun Wu ◽  
Yueming Hu

Abstract Background Earlier research works have studied the part that cytotoxic T-lymphocyte antigen-4 (CTLA-4) plays in the carcinogenesis of malignant bone tumors, nonetheless, findings had inconsistency. The current investigation aims at clarifying the association between CTLA-4 polymorphism and malignant bone tumor susceptibility through the meta-analysis.Methods We searched pertinent research works in not just PubMed, but also EMBASE, Cochrane library, and Chinese National Knowledge Infrastructure (CNKI) databases in humans published before October 2019. The use of the pooled odds ratio (OR) with its 95% confidence interval (95%CI) was made for estimating the strengths of the correlation existing between the CTLA-4 genetic polymorphism and malignant bone tumors susceptibility. An aggregate of six research works with 1191 malignant bone tumors patients and 1418 controls were selected eventually. The pooled results shed light on the fact that CTLA-4 +49G/A polymorphism had a significant correlation with an augmented vulnerability to the malignant bone tumors (A vs. G: OR=1.37, 95%CI=1.22-1.54; GA vs. GG: OR=1.20, 95%CI=1.01-1.42; AA vs. GG: OR=2.13, 95%CI=1.63-2.78; GA+AA vs. GG: OR=1.35, 95%CI=1.15-1.59; AA vs. GG+GA: OR=2.02, 95%CI=1.60-2.56). Subgroup analysis indicated that there exists a statistically significant correlation between CTLA-4 +49G/A polymorphism and augmented susceptibility to the malignant bone tumor in the population-based or hospital-based samples, and Ewing’s sarcoma or osteosarcoma. Moreover, there was also not observed any considerable heterogeneity across the research works. Results Our results suggest that the CTLA-4 +49G/A polymorphism may play a pivotal part in the carcinogenesis of malignant bone tumors. Conclusions More research works, on the basis of the large sample sizes as well as homogeneous specimens, are needed in order to verify these results.


2007 ◽  
Vol 42 (3) ◽  
pp. 291
Author(s):  
Han-Soo Kim ◽  
Hyun Guy Kang ◽  
Jai Ho Cho ◽  
Kap Jung Kim ◽  
Joo Han Oh ◽  
...  

2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Lucen Jiang ◽  
Jianghuan Liu ◽  
Qingzhu Wei ◽  
Yiyang Wang

Abstract Background Karyopherin α2 (KPNA2), a member of the karyopherin α family, has been studied in several cancers but has not yet been substantially investigated in malignant bone tumors. The purpose of the current study was to evaluate the KPNA2 expression level and its utility as a novel diagnostic biomarker in osteosarcomas and malignant bone tumor mimics, such as chondrosarcomas and Ewing sarcomas (ESs). Method We investigated the expression of KPNA2 protein by immunohistochemistry on paraffin-embedded surgical specimens from 223 patients with malignant and benign bone tumors, including 81 osteosarcomas, 42 chondrosarcomas, 15 ESs, 28 osteoid osteomas, 20 osteochondromas and 37 chondroblastomas. Immunoreactivity was scored semiquantitatively based on staining extent and intensity. Results Sixty-seven of 81 (82.7%) osteosarcoma, zero of 42 (0%) chondrosarcoma and one of 15 (6.7%) ES samples showed immunoreactivity for KPNA2. Negative KPNA2 expression was observed in all benign bone tumors. The expression of KPNA2 in osteosarcoma samples was much higher than that in chondrosarcoma and ES samples (P < 0.001). The sensitivity and specificity of KPNA2 immunoexpression for detecting osteosarcoma were 82.7 and 100%, respectively. Several subtypes of osteosarcoma were analyzed, and immunostaining of KPNA2 was frequent in osteoblastic samples (90.9%), with 39 samples (70.9%) showing strong-intensity staining. KPNA2 positivity was observed in ten of 13 (76.9%) chondroblastic, two of 6 (33.3%) fibroblastic, three of 4 (75%) telangiectatic and two of 3 (66.7%) giant cell-rich osteosarcoma samples. The strongest intensity staining was observed in osteoblastic osteosarcoma. Conclusion KPNA2 is frequently expressed in osteosarcomas, particularly in osteoblastic and chondroblastic tumors, but is rarely positive in chondrosarcomas and ESs. This feature may aid in distinguishing between osteosarcoma and other bone sarcoma mimics. This report supports KPNA2 as a novel marker for the diagnosis of osteosarcoma.


2020 ◽  
Vol 38 (15_suppl) ◽  
pp. 11525-11525
Author(s):  
Lina Tang ◽  
Xiaohui Niu ◽  
Zhen Wang ◽  
Qiqing Cai ◽  
Chongqi Tu ◽  
...  

11525 Background: Primary malignant bone tumors are rare forms of cancer and include mainly bone sarcomas, which are categorized into 3 common types based on tissue origin: osteosarcoma, chondrosarcoma and Ewing sarcoma. A phase II trial was designed to explore the anlotinib activity in patients with relapsed or metastatic primary malignant bone tumor. Methods: Eligible pts were received 12mg of anlotinib once daily, 2 weeks on and 1 week off until progression or unacceptable toxicity. Key-eligibility criteria were aged 14-70 years, histologically confirmed diagnosis of osteosarcoma, chondrosarcoma, bone derived malignant fibrous histiocytoma, giant cell tumor, Ewing sarcoma and PNET, confirmed previous chemotherapy failure, ECOG 0-1(0-2 for amputation pts), required at least one measurable lesion. We observed PFS, OS, ORR, DCR and AE in this study. Results: From August 2018 to April 2019, 42 pts were included. Of 42 efficacy-evaluable pts, 25 were man, median age was 28 (14-68) years. There were 29 pts of osteosarcoma, 9 pts of chondrosarcoma, 3pts of Ewing sarcoma and 1 pt of bone derived malignant fibrous histiocytoma. The progression-free rate at 12 weeks (PFR12weeks), ORR and DCR were 71.3%, 9.52% and 78.57%. Median PFS was 5.26 months (95%CI = 3.48-8.44). Median OS was 11.40 months (95%CI = 10.09, [ ). Median PFS of osteosarcoma and chondrosarcoma was 4.83 months (95%CI = 3.48, 7.13 ) and 2.76 months (95%CI = 1.31, [ ) respectively. The most common Gr 3-5 anlotinib-related AEs were hypertension (19.05%), hypertriglyceridemia (9.52%), hand-foot syndrome (7.14%), and proteinuria (4.76%). Conclusions: The phase II study shows a promising activity of anlotinib in patients with relapsed or metastatic primary malignant bone tumor and an acceptable toxicity. Clinical trial information: NCT03527888 .


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