dedifferentiated chondrosarcoma
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2021 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Lee D. Cranmer ◽  
Bonny Chau ◽  
Jose G. Mantilla ◽  
Elizabeth T. Loggers ◽  
Seth M. Pollack ◽  
...  

2021 ◽  
Author(s):  
Tailong Chen ◽  
Nan Zhou ◽  
Zhongxin Tang ◽  
Xing Guo ◽  
Wengang Wang

Abstract Background: Chondrosarcoma is a rare type of bone tumor which more commonly found in adults range from 40 to 60 years old. Few studies has described the characteristic and prognostic factors of patients older than 60 years. This study aimed to study this feature and identify the prognostic factors based on SEER database.Methods: Thus, we collected clinicopathological data of chondrosarcoma patients in the Surveillance, Epidemiology, and End Results registry database from 1975 to 2018, and then use the Kaplan-Meier to analyze the patients’ survival. We also utilize Cox proportional hazard model to explore the prognostic factors and relevant characteristic including patients’ baseline demographics (age, race, and gender), tumor characteristics (tumor extension, histologic subtype, therapy, primary site, stage and grade.Results: After the implementation of exclusion criteria, there were 610 patients with chondrosarcoma older than 60 years. Our data showed that the incidence of chondrosarcoma is slightly higher in men than in women (52.3% vs 47.7%). In general, 90.8% of tumor had metastasized to distant sites. Meanwhile, 41.8% of tumors occurred in axial location (pelvis, spine, and ribs), 50.8% of tumors occurred in extremity (long or short bones of the upper or lower extremity), and 7.4% in other location (mandible, skull, and other atypical locations). Dedifferentiated chondrosarcoma (hazard ratio [HR] =2.553; 95% confidence interval [CI]= 1.754-3.716), grade (g2:HR:=1.299; 95% CI:=0.888-1.900, g3:HR=1.839;95% CI= 1.174-2.881, g4:HR=3.284,95%CI=2.053-5.253), distant metastasis (HR=3.264; 95% CI= (2.288-4.058), non-surgery perform (HR = 2.854; 95% CI= 2.022-4.028) were independent risk factors for 5-year overall survival.Conclusion: In conclusion, higher grade, non-surgery perform, dedifferentiated chondrosarcoma and distant metastasis indicated worse prognosis survival. Surgery can significantly improve the survival time of patients.


2021 ◽  
Author(s):  
Tailong Chen ◽  
Nan Zhou ◽  
Zhongxin Tang ◽  
Xing Guo ◽  
Wengang Wang

Abstract Background Chondrosarcoma is a rare type of bone tumor which more commonly found in adults range from 40 to 60 years old. Few studies has described the characteristic and prognostic factors of patients older than 60 years. This study aimed to study this feature and identify the prognostic factors based on SEER database. Methods Thus, we collected clinicopathological data of chondrosarcoma patients in the Surveillance, Epidemiology, and End Results registry database from 1975 to 2018, and then use the Kaplan-Meier to analyze the patients’ survival. We also utilize Cox proportional hazard model to explore the prognostic factors and relevant characteristic including patients’ baseline demographics (age, race, and gender), tumor characteristics (tumor extension, histologic subtype, therapy, primary site, stage and grade. Results After the implementation of exclusion criteria, there were 610 patients with chondrosarcoma older than 60 years. Our data showed that the incidence of chondrosarcoma is slightly higher in men than in women (52.3% vs 47.7%). In general, 90.8% of tumor had metastasized to distant sites. Meanwhile, 41.8% of tumors occurred in axial location (pelvis, spine, and ribs), 50.8% of tumors occurred in extremity (long or short bones of the upper or lower extremity), and 7.4% in other location (mandible, skull, and other atypical locations). Dedifferentiated chondrosarcoma (hazard ratio [HR] = 2.553; 95% confidence interval [CI] = 1.754–3.716), grade (g2:HR:=1.299; 95% CI:=0.888-1.900, g3:HR = 1.839;95% CI = 1.174–2.881, g4:HR = 3.284,95%CI = 2.053–5.253), distant metastasis (HR = 3.264; 95% CI= (2.288–4.058), non-surgery perform (HR = 2.854; 95% CI = 2.022–4.028) were independent risk factors for 5-year overall survival. Conclusion In conclusion, higher grade, non-surgery perform, dedifferentiated chondrosarcoma and distant metastasis indicated worse prognosis survival. Surgery can significantly improve the survival time of patients.


2021 ◽  
Vol 36 (4) ◽  
pp. e283-e283
Author(s):  
Tariq N. Aladily ◽  
Nisreen Abushahin ◽  
Randa Mahgoub ◽  
Bassem I. Haddad

A 54-year-old man presented with a pathologic hip fracture secondary to a right femur tumor. A reaming biopsy showed the diagnosis of dedifferentiated chondrosarcoma (DDC). In addition, the patient had a thyroid mass. Fine needle aspirate from the thyroid mass revealed numerous large fragments of cohesive spindle cells with moderate nuclear atypia and brisk mitosis, identical to the femur tumor. Immunohistochemical staining on cell blocks of thyroid aspirate showed positivity for mesenchymal markers and negativity for epithelial and thyroid markers. The metastasis of DDC to the thyroid gland is a very rare incident and has previously been described once in the literature. In this report, we describe the clinical and pathologic findings of this case.


2021 ◽  
Vol 151 ◽  
pp. 150-158
Author(s):  
Ivar Hompland ◽  
Stefano Ferrari ◽  
Stefan Bielack ◽  
Emanuela Palmerini ◽  
Kirsten S. Hall ◽  
...  

2021 ◽  
Vol 39 (15_suppl) ◽  
pp. e23500-e23500
Author(s):  
Charles Gusho ◽  
Steven Gitelis ◽  
Alan T. Blank

e23500 Background: Dedifferentiated chondrosarcoma (DCS) is a rare and aggressive malignancy with a poor prognosis. The purpose of this investigation was to assess the clinicopathological features and outcomes of DCS patients treated at a single institution. Methods: This study was a retrospective review over a consecutive twenty-year period. Data including treatment details and outcomes were recorded. Results: A total of 16 cases from 2000 to 2018 were reviewed. The median age was 62 years (IQR, 52-69 years) and the majority of DCS arose in the femur (50%, n = 8) and pelvis (25%, n = 4). Fourteen (88%) cases received limb salvage/wide margin resection (n = 13) or intralesional surgery (n = 1). For all DCS, the median estimated overall survival (OS) was 46 months (95% CI, 1-90 months) with both a five and ten-year survival probability of 32%. On Kaplan-Meier analysis there was no difference between operative versus nonoperative management (p = 0.747), surgery alone versus surgery/chemotherapy (p = 0.265), nor surgery alone versus surgery/chemotherapy/radiation (p = 0.698). Conclusions: Our findings confirm the poor prognosis of DCS patients, though with a five-year estimate of 32%, higher than previous literature. Together with existing literature, our data may enable future strategic recommendation of DCS patients.


2021 ◽  
pp. 106689692110102
Author(s):  
Raffaele Gaeta ◽  
Giacomo Aringhieri ◽  
Virna Zampa ◽  
Antonio D’Arienzo ◽  
Lorenzo Andreani ◽  
...  

Dedifferentiated chondrosarcoma is a well-recognized entity, but its occurrence in the distal extremities is exceedingly rare. We present the case of a 49-year-old woman who experienced local recurrence of an “enchondroma” of the proximal phalanx of the fourth finger of the left hand, which had been initially treated with intralesional curettage at another hospital 4 years before, and 1 year before for a local recurrence. The imaging findings indicated an aggressive behavior, and an incisional biopsy showed a highly cellular proliferation of spindle and pleomorphic elements without evidence of matrix production intermixed with few fragments of a well-differentiated cartilaginous neoplasm with bland cellular atypia, focal nuclear hyperchromatism, and binucleation. An isocitrate dehydrogenase 2 R172S mutation was detected. The final diagnosis was dedifferentiated chondrosarcoma. Despite amputation of the fourth finger, the patient developed lung metastases and further local relapse. Recurrent cartilaginous tumors of the extremities should not be underestimated and should be followed in view of the possible acquisition of aggressive clinical behavior.


2021 ◽  
Vol 11 ◽  
Author(s):  
Sjoerd P. F. T. Nota ◽  
Ahmad Al-Sukaini ◽  
Shalin S. Patel ◽  
Francesco Sabbatino ◽  
G. Petur Nielsen ◽  
...  

PurposeThe aim of this study was to characterize chondrosarcoma tumor infiltration by immune cells and the expression of immunologically relevant molecules. This information may contribute to our understanding of the role of immunological events in the pathogenesis of chondrosarcoma and to the rational design of immunotherapeutic strategies.Patients and MethodsA tissue microarray (TMA) containing 52 conventional and 24 dedifferentiated chondrosarcoma specimens was analyzed by immunohistochemical staining for the expression of parameters associated with tumor antigen-specific immune responses, namely, CD4+ and CD8+ tumor infiltrating lymphocytes (TILs) and the expression of HLA class I heavy chain, beta-2 microglobulin (β2m), HLA class II and immune checkpoint molecules, B7-H3 and PD-1/PD-L1. The results were correlated with histopathological characteristics and the clinical course of the disease.ResultsCD8+ TILs were present in 21% of the conventional and 90% of the dedifferentiated chondrosarcoma tumors tested. B7-H3 was expressed in 69% of the conventional and 96% of the dedifferentiated chondrosarcoma tumors tested. PD-1 and PD-L1 were expressed 53% and 33% respectively of the dedifferentiated tumors tested. PD-L1 expression was associated with shorter time to metastasis.ConclusionThe tumor infiltration by lymphocytes suggests that chondrosarcoma is immunogenic. Defects in HLA class I antigen and expression of the checkpoint molecules B7-H3 and PD-1/PD-L1 suggest that tumor cells utilize escape mechanisms to avoid immune recognition and destruction. This data implies that chondrosarcoma will benefit from strategies that enhance the immunogenicity of tumor antigens and/or counteract the escape mechanisms.


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