Incidence Of Chondrosarcoma Research Articles

Abstract 4844: Epidemiology of bone and soft-tissue sarcomas: A two-decade analysis of the surveillance, epidemiology, and end results program

Abstract In the United States, ~15000 cases of bone and soft-tissue sarcomas are diagnosed annually, representing roughly 1% of all incident cancers. While several studies have analyzed independent predictors of outcomes for these sarcomas, small samples and confounding bias have limited their ability to establish reliable conclusions. The present analysis provides a comprehensive epidemiological description of patients with any of 16 distinct bone and soft-tissue sarcomas by querying the Surveillance, Epidemiology, and End Results database between 2000 and 2020. Incidence, prevalence, incidence-based mortality, survival rates, and case-fatality ratios were computed. Using patient age, sex, race, and ethnicity, as well as tumor size, grade, and stage as covariates, multivariable survival Cox proportional hazards regression analyses were performed to model hazard ratios (HRs) and identify independent predictors of worse prognosis. Only patients with myosarcoma and fibrous histiocytoma witnessed a decrease in the prevalence of their disease over time (both, p<0.05). The incidence of chondrosarcoma and synovial sarcoma increased in White and Asian patients but decreased in Black individuals (both, p<0.001), while their case-fatality rates remained constant and increased, respectively, regardless of race and ethnicity (all, p<0.05). Case-fatality rates only declined significantly for White and Non-Hispanic patients with solitary fibrous tumor (both, p<0.05). Aside from alveolar soft-part sarcoma and clear cell sarcoma, a 10-year increase in age of patients with bone and soft-tissue sarcomas resulted in 18% to 61% higher likelihood of death (all, p<0.05). For most malignancies, having a tumor size greater than 5 cm yielded a 37% to 189% higher likelihood of death. While incidence trends suggest improvements in diagnostic techniques and early tumor detection, survival and case-fatality trends indicate potential for advancement in treatment for most bone and soft-tissue sarcomas. Citation Format: Marc El Beaino, Rachel Baum, Katherine M. Connors, Karim Masrouha, Santiago A. Lozano-Calderon, Patrick P. Lin. Epidemiology of bone and soft-tissue sarcomas: A two-decade analysis of the surveillance, epidemiology, and end results program [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2024; Part 1 (Regular Abstracts); 2024 Apr 5-10; San Diego, CA. Philadelphia (PA): AACR; Cancer Res 2024;84(6_Suppl):Abstract nr 4844.

The Epidemiology of Chondrosarcoma in Iran Based on Iran National Cancer Registry

Background: Chondrosarcoma is regarded as the second most common primary bone malignancy following osteosarcoma. Objectives: The present study aimed at determining the epidemiology, incidence, and survival rate of chondrosarcoma in the Iranian population, according to the Iran National Cancer Registry (INCR). Methods: In an epidemiological study, patients with limb chondrosarcoma were evaluated based on INCR data between 2008 and 2015. Data included patients’ demographic characteristics, date of diagnosis, location of the tumor, patient’s survival, and type of tumor based on the International Classification of Diseases for Oncology (ICD-O-3; first revision, third edition) were collected and analyzed. Results: Out of 732 enrolled patients, 425 patients (58.06%) were male and 307 (41.94%) were female with a mean age of 44.08 (SD = 19.31) and 45.06 (SD = 18.72), respectively. Age-standardized incidence rates (ASIR) were 1.73 and 1.27 per 1 million person-years for males and females, respectively. Conventional chondrosarcoma was the most common subtype with ASIR 1.28 and constituted 84.7% of patients with chondrosarcoma. About 71.03% of all Chondrosarcoma patients (70.35% of males and 71.99% of females) were between 20 to 59 years old. The 1-, 3-, 5-, and 7-year survival rates of patients were 0.87, 0.73, 0.57, and 0.47, respectively. Also, the mean survival time was 6.12 years (95% CI: 5.85 - 7.39). Conclusions: The incidence of chondrosarcoma in Iran is not as high as in other countries, but as patients are younger in Iran, the survival rate is worse compared to other countries. Therefore, better case findings and better care are needed to improve the patients' outcomes in Iran.

The influence of site on the incidence and diagnosis of solitary central cartilage tumours of the femur. A 21st century perspective

ObjectiveTo determine the incidence of central cartilage tumours (CCTs) in the femur and the impact of site (proximal, mid and distal thirds) on tumour grade. To compare study results with historically published data. Materials and methodsRetrospective review of solitary CCTs arising in the femur over the past 13 years. Data collected included location (proximal, mid and distal thirds) and final diagnosis in terms of tumour grade based on imaging features ± histology. Case material collected from three bone tumour textbooks provided historical data. Results430 solitary CCTs were included in the femur. 73% cases arose in the distal, 3.7% in the mid and 23% in the proximal femur. The ratio of “benign” (combining enchondroma and atypical cartilaginous tumour (ACT)) to higher grade chondrosarcoma (CS) was 11:1 in the distal, 1:1 in the mid and 1:1.5 in the proximal femur, the distribution of benign to malignant tumours being significantly different between the regions (F test, p < 0.05). Comparison with historical data showed a reversal of the benign (enchondroma) to malignant (ACT and higher grade CS) of 30%:70%–84%:16% in the current series. ConclusionsThe site of origin of a CCT in the femur has an impact on final diagnosis with CS uncommon in the distal as compared with the mid and proximal femur. This is in contradistinction to historical data where the incidence of CS exceeded that of enchondroma at all sites.

Sternectomy for primary chondrosarcoma

The incidence of chondrosarcoma is <0.5 per million and year, accounting for the most frequent primary malignant chest wall tumor (1). Surgery plays a major role in the treatment of chondrosarcoma since chondrosarcoma is relatively insensitive to chemotherapy and radiation (2). Due to the large tumor size at diagnosis, extended chest wall resection is often necessary.

Chondrosarcoma in Norway 1990–2013; an epidemiological and prognostic observational study of a complete national cohort

Background: Knowledge of chondrosarcoma (CS) of bone to date is based on institutional reports and registry publications with limits in reporting, detail and quality of data.Method: We have performed a retrospective search of CS of bone in the National Cancer Registry in Norway from 1990–2013, cross checked against local tumor databases with further quality control and supplementation of all data from clinical files. The time period is defined by the routine use of axial imaging in clinical practice. A total of 311 cases are included. We performed 108 pathological reviews and 223 radiological reviews. The manuscript was prepared according to the STROBE checklist for strengthening of observational studies. We performed uni-/multivariate cox analyses to define independent prognostic variables from the main cohort of central CS of bone.Results: The incidence of CS of bone in Norway is 2.85/million/yr. for both sexes overall, rising to 3.45/million/yr. in the last 5-year period. There is an increase in the most common central CS subtype, stronger for women than for men.Central CS had, in general 10–15% local recurrence rates, all evident by 5 years while metastasis rate increases with location and grade. Exceptions are extremity grade 1 CS which displayed no metastatic events and axial grade-3 disease with high rates (50%) of both local and metastatic relapse. Peripheral CS had limited metastatic potential (2%), but rates of local relapse (13%) continue to appear towards 10 years of follow up.Malignancy grade 3 independently predicts rate of metastasis and presence of soft tissue component predicts local recurrence, metastasis and survival.Conclusion: Rates of local recurrence, metastasis and disease specific survival follow clear patterns depending on subtype, location and grade allowing better tailoring of follow-up regimes. Malignancy grade 3 and the presence of a soft tissue component independently predict behavior for central CS of bone.

Incidence, Predictive Factors, and Prognosis of Chondrosarcoma in Patients with Ollier Disease and Maffucci Syndrome: An International Multicenter Study of 161 Patients

Enchondromatosis is characterized by the presence of multiple benign cartilage lesions in bone. While Ollier disease is typified by multiple enchondromas, in Maffucci syndrome these are associated with hemangiomas. Studies evaluating the predictive value of clinical symptoms for development of secondary chondrosarcoma and prognosis are lacking. This multi-institute study evaluates the clinical characteristics of patients, to get better insight on behavior and prognosis of these diseases. A retrospective study was conducted using clinical data of 144 Ollier and 17 Maffucci patients from 13 European centers and one national databank supplied by members of the European Musculoskeletal Oncology Society. Patients had multiple enchondromas in the hands and feet only (group I, 18%), in long bones including scapula and pelvis only (group II, 39%), and in both small and long/flat bones (group III, 43%), respectively. The overall incidence of chondrosarcoma thus far is 40%. In group I, only 4 patients (15%) developed chondrosarcoma, in contrast to 27 patients (43%) in group II and 26 patients (46%) in group III, respectively. The risk of developing chondrosarcoma is increased when enchondromas are located in the pelvis (odds ratio, 3.8; p = 0.00l). Overall incidence of development of chondrosarcoma is 40%, but may, due to age-dependency, increase when considered as a lifelong risk. Patients with enchondromas located in long bones or axial skeleton, especially the pelvis, have a seriously increased risk of developing chondrosarcoma, and are identified as the population that needs regular screening on early detection of malignant transformation.

Incidence of osteosarcoma, chondrosarcoma and Ewing's sarcoma in east Denmark: Reverse male to female ratio in osteosarcoma

The incidence of osteosarcoma, chondrosarcoma and Ewing's sarcoma was studied in east-Denmark during a 14 years period from 1983 to 1996. The population of east Denmark was 2.3 millions people. A total of 109 primary osteosarcoma, chondrosarcoma and Ewing's sarcomas were found. The mean age of the 47 patients (15 women and 32 men) with chondrosarcoma was 54 years (range 30–85 years). The mean age of the 45 patients (23 women and 22 men) with osteosarcoma was 26 years (range 6–72 years). The mean age of the 17 patients (7 women and 10 men) with Ewing's sarcoma was 19 years (range 3–33 years). The overall incidence of chondrosarcoma was 1,46/1000000 inhabitants/year. The overall incidence of osteosarcoma was 1,40/1000000 inhabitants/year. The overall incidence of Ewing's sarcoma was 0,53/1000000 inhabitants/year.

Chondrosarcoma in Maffucciʼs syndrome.

Nine patients with Maffucci's syndrome were seen at our institution; chondrosarcoma developed in five. On the basis of the cases of these patients and those reported in the English literature since 1973, we determined that the incidence of chondrosarcoma in patients with Maffucci's syndrome is 17.8 per cent.

Maffucci's syndrome (dyschondroplasia with hemangiomas): a case with early osseous changes.

MAFFUCCI, in 1881, 1 was the first to describe an entity characterized by dyschondroplasia and multiple hemagiomas. Since that time a total of 27 cases have been reported in the literature. 2 Other characteristic observations of this syndrome include the following: the disease is nonfamilial; the patient is usually normal at birth; involvement is unilateral or extremely asymmetric; there is uneven growth of the two sides; the disease is commoner in males; its onset is before puberty; there is a susceptibility of the patient to fractures, and phleboliths in hemangiomas are not uncommon. The dyschondroplasia is primarily in the form of defects in ossification, with one or more of the long bones having short shafts. The deformity varies according to the severity of involvement. On a review of the previous literature the incidence of chondrosarcoma was found to be approximately 20 per cent, particularly in those patients in whom enchondroma