Epidemiología e imagenología del osteosarcoma y condrosarcoma en el territorio craneofacial. Revisión narrativa / Epidemology and imaging of osteosarcoma and chondrosarcoma in the craniofacial territory. Narrative review

Introducción: El osteosarcoma y el condrosarcoma son lesiones óseas malignas, ubicadas con mayor frecuencia en las extremidades y con menor frecuencia en el territorio craneofacial. No se ha reportado características imagenológicas concretas que diferencien ambas patologías en el territorio craneofacial. El propósito de la presente revisión narrativa es analizar las características epidemiológicas e imagenológicas del osteosarcoma craneofacial (OSCF) y condrosarcoma craneofacial (CSCF).Materiales y métodos:Se analizó la información publicada del OSCF y CSCF entre los años 2011-2021, con un enfoque en el análisis imagenológico.Resultados:El OSCF y CSCF no tienen preferencia clara por sexo. Mientras el OSCF se ubica en huesos de origen membranoso y preferencia entre la tercera y cuarta década de vida, el CSCF se ubica en zonas de origen endocondral y preferencia entre la tercera y sexta década de vida. En el OSCF, se han observado tres patrones de presentación imagenológicos: esclerótico, lítico y mixto, a diferencia del CSCF, en que se ha observado solo el patrón de presentación lítico. Ambas patologías tienen límites infiltrantes. El estudio imagenológico se realiza con tomografía computarizada, resonancia magnética, gammagrafía ósea, tomografía computarizada de fotón único y por emisión de positrones. En ambas patologías es más frecuente la recidiva local que la metástasis.Conclusiones:El OSCF y CSCF tienen características clínicas, epidemiológicas diferentes e imagenológicas similares. Establecer diferencias entre ellas es esencial para una correcta presunción diagnóstica.(AU)

Introduction: Osteosarcoma and chondrosarcoma are malignant lesions which locate most frequently in extremities and less frequently in craniofacial territory. Concrete imaging features in craniofacial territory that identify each pathology have not been reported. The aim of the present narrative review is to analyze the epidemiological and imaging features of the craniofacial osteosarcoma (CFOS) and craniofacial chondrosarcoma (CFCS).Materials and methods:We analyzed the information on CFOS and CFCS published between 2011 and 2021, focused on imaging analysis.Results:CFOS and CFCS do not show sex preference. Whilst CFOS is usually located in bones of membranous origin and is commonly discovered between the third and fourth decade of life, CFCS is usually located in areas of endochondral origin and commonly appears between the third and sixth decade of life. Three radiological presentation patterns are observed in CFOS: sclerotic, lytic and mixed, unlike the CFCS, where only the lytic pattern has been observed. Both pathologies have infiltrated margins. The imaging study is performed with computed tomography, magnetic resonance imaging, bone scintigraphy, single-photon computed tomography and positron emission tomography, among others. Local recurrence is more common than metastasis in both pathologies.Conclusion:CFOS and CFCS have different clinical and epidemiological features but similar imaging features. Stablishing differences between both pathologies is essential to achieve a correct presumptive diagnosis.(AU)

Impact of Prior Cancer on the Prognosis of Patients with Chondrosarcoma: A Population-Based Study of the SEER Database.

Background: Patients with prior cancer are generally exempt from cancer experiments. This research aims to describe the prevalence, clinical features, and effects of past malignancy among patients with chondrosarcoma. Methods: Chondrosarcoma patients diagnosed between 2010 to 2015 were collected from the SEER database. The propensity score matching method was used to reconcile the disparity in baseline attributes. Kaplan-Meier analysis was employed to explore the outcomes of prior cancer on overall survival. The proportional hazards assumption was used to certain whether the covariate matched the Cox regression model. The potential outliers were estimated by deviance residuals type. Results: A total of 1,721 unique individuals were collected, of those 284 (16.50%) patients had a history of cancer, with prostate cancer being commonly documented (n = 49, 17.25%). Approximately half of the previous tumors are diagnosed within 5 years before the diagnosis of chondrosarcoma. Chondrosarcoma patients with prior cancers have a lower survival rate than those without prior malignancy (P < .001). A multivariable Cox analysis reveals that past cancer is a distinct risk factor for lifespan (hazard ratio = 2.489, P < .001). Conclusion: This study initially discovered that chondrosarcoma patients with past cancer have a bad prognosis. Different types of past cancer have varying effects on survival. We urgently propose that cancer trial exclusion criteria be set specifically by cancer classification, rather than accepting the unchangeable criterion for default.

Risk factors for distant metastasis of chondrosarcoma: A population-based study.

Chondrosarcoma is the second largest bone malignancy after osteosarcoma and mainly affects middle-aged adults, where patients with distant metastasis (DM) often have a poor prognosis. Although nomograms have been widely used to predict distant tumor metastases, there is a lack of large-scale data studies for the diagnostic evaluation of DM in chondrosarcoma. Data on patients diagnosed with chondrosarcoma from 2004 to 2015 were obtained from the Surveillance, Epidemiology, and End Results database. Independent risk factors for having DM from chondrosarcoma were screened using univariate and multivariate logistics regression analysis. A nomogram was created to predict the probability of DM from the screened independent risk factors. The nomogram was then validated using receiver operating characteristic curves and calibration curves. A total of 1870 chondrosarcoma patients were included in the study after data screening, of which 157 patients (8.40%) had DM at the time of diagnosis. Univariate and multivariate logistic regression analysis screened four independent risk factors, including grade, tumor number, T stage, and N stage. receiver operating characteristic curves and calibration curves showed good accuracy of the nomogram in both training and validation sets. The current study screened for independent risk factors for DM from chondrosarcoma, which will help clinicians evaluate patients.

Development and validation of a nomogram for predicting prognosis of high-grade chondrosarcoma: A surveillance, epidemiology, and end results-based population analysis.

BACKGROUND: The incidence of chondrosarcoma is increasing every year, and the treatment and prognosis of patients with high-grade chondrosarcoma are becoming more and more important. Nomogram is a tool that can quickly and easily predict the overall survival of tumor patients. Therefore, the development and validation of a nomogram to predict overall survival in patients with high-grade chondrosarcoma was desired. METHODS: We retrospectively collected 396 patients with high-grade chondrosarcoma from the Surveillance, Epidemiology, and End Results (SEER) database from 2004 to 2015. Randomly divided into model and validation groups, the best cut-off values for age and tumor size grouping were derived by using X-tile software. Then, independent prognostic factors for high-grade chondrosarcoma were derived by SPSS.26 univariate and multivariate Cox analyses analysis in the model group, and the model was evaluated by using R software, using C-indix and ROC curves, and finally these independent prognostic factors were included in Nomogram. RESULTS: 396 patients were randomly assigned to the modelling group (n = 280) or the validation group (n = 116). Age, tissue-type, tumor size, AJCC stage, regional expansion and surgery were identified as independent prognostic factors (p < 0.05) which further combined to construct a nomogram. The C-index of internal validation for overall survival(OS) was 0.757, while the C-index of external validation for overall survival(OS) was 0.832. Both internal and external calibration curves show a good agreement between nomogram prediction and actual survival. CONCLUSION: In this study, we established age, tumour size, AJCC stage, tissue type, surgery and tumor extension as independent prognostic factors for high-grade chondrosarcoma and constructed a nomogram to predict 3- and 5-year survival rates for high-grade chondrosarcoma.

The national incidence of chondrosarcoma of bone; a review.

BACKGROUND: Chondrosarcoma (CS) epidemiology has been studied by a number of authors using national cancer registry cohorts. Many reports share the common findings of a slight increase in incidence, but not all. The patterns and causes for these changes are divergent while reflection concerning methodological challenges are often missing. METHOD: We have performed a structured literature review to find national analyses of CS incidence published from 2010 to 2020. We included eight studies of national incidence of CS, summarise their findings and patterns of change. We further discuss explanations given for these changes to better understand the real patterns and raise awareness in their interpretation. RESULTS: Reported crude incidence ranges from 0.27 per million per year overall in Saudi Arabia to 5.4 in the Netherlands. Four studies from the USA, England, Switzerland and France report age standardised rates of 2.0-4.1 per million per year overall. While some countries report stable patterns, most report a slight increase. The Netherlands is the only country reporting a large increase, driven by a 10-fold increase in the incidence of ACT/grade 1 CS during the study period. We challenge the explanations given for this and suggest that this most likely is a result of variable interpretation and definition of CS at the lower levels of disease aggressiveness. This should raise awareness to possible over-treatment of CS in the Netherlands. CONCLUSION: The most likely national incidence of CS of bone is between 2-4 per million per year. Three modern reports present an incidence of 3.4-4.1 per million per year.

Association Between Insurance Status and Chondrosarcoma Stage at Diagnosis in the United States: Implications for Detection and Outcomes.

INTRODUCTION: Chondrosarcoma is a common primary bone tumor, and survival is highly influenced by stage at diagnosis. Early detection is paramount to improve outcomes. The aim of this study is to analyze the association between insurance status and stage of chondrosarcoma at the time of diagnosis. METHODS: A comparative cross-sectional study was conducted using the Surveillance, Epidemiology and End Results database. Patients with a diagnosis of chondrosarcoma between 2007 and 2016 were included. Exposure variable was insurance status and the outcome chondrosarcoma staging at the time of diagnosis. Control variables included tumor grade, age, sex, race, ethnicity, marital status, place of residence, and primary site. Both unadjusted and adjusted (multiple logistic regression) odds ratios (ORs) and 95% confidence intervals (CIs) were computed to estimate the association between insurance status and stage. RESULTS: An effective sample of 2,187 patients was included for analysis. In total, 1824 (83%) patients had health insurance (nonspecified), 277 (13%) had Medicaid, and the remaining 86 (4%) had no insurance. Regarding stage at diagnosis, 1,213 (55%) had localized disease, whereas 974 (45%) had a later stage at presentation. Before adjustment, the odds of being diagnosed at an advanced (regional/distant) stage were 55% higher in patients without insurance (unadjusted OR 1.55; 95% CI 1.003 to 2.39). After adjusting for potential confounders, the odds increased (adjusted OR 1.94; 95% CI 1.12 to 3.32). Variables with a significant association with a later stage at diagnosis included older age ( P < 0.001), male sex ( P < 0.001), pelvic location ( P < 0.001), and high grade ( P < 0.001). CONCLUSION: Being uninsured in the United States increased the odds of a late-stage diagnosis of chondrosarcoma by 94% when compared with insured patients. Lack of medical insurance presumably leads to diminished access to necessary diagnostic testing, which results in a more advanced stage at diagnosis and ultimately a worse prognosis. Efforts are required to remediate healthcare access disparities. LEVEL OF EVIDENCE: Level III.

Atypical Cartilaginous Tumors: Trends in Management.

INTRODUCTION: Chondrosarcomas are the most common primary bone malignancy in adults within the United States. Low-grade chondrosarcomas of the long bones, now referred to as atypical cartilaginous tumors (ACTs), have undergone considerable changes in recommended management over the past 20 years, although controversy remains. Diagnostic needle biopsy is recommended only in ambiguous lesions that cannot be clinically diagnosed with a multidisciplinary team. Local excision is preferred due to better functional and equivalent oncologic outcomes. We sought to determine whether these changes are reflected in reported management of ACTs. METHODS: The National Cancer Database (NCDB) 2004 to 2016 was queried for ACTs of the long bones. Reported patient demographics and tumor clinicopathologic findings were extracted and compared between patients who underwent local excision versus wide resection. RESULTS: We identified 1174 ACT patients in the NCDB. Of these, 586 underwent local excision and 588 underwent wide resection. No significant differences were found in patient demographics. No significant change was found in the reported percentage of diagnostic biopsies or wide resections performed over time. After multivariate regression, the single greatest predictor of performing wide resection on an ACTs was presenting tumor size. DISCUSSION: Evaluation of the NCDB demonstrated that despite changes in the recommended management of ACTs, there has not been a significant change in surgical treatment over time. Surgeons have been performing diagnostic biopsies and wide resections at similar to historical rates. Persistency of these practices may be due to presenting tumor size, complex anatomic location, uncertainty of underlying tumor grade, or patient choice as part of clinical shared decision making. The authors anticipate that the rate of biopsies and wide resections performed will decrease over time as a result of improvements in advanced imaging and the implementation of recently updated clinical practice guidelines.

Racial disparity in recommendation for surgical resection of skull base chondrosarcomas: A Surveillance, Epidemiology, and End Results (SEER) analysis.

INTRODUCTION: There is increased appreciation of racial disparities in the delivery of neurosurgical care. Here, we explore whether race influences surgical recommendations in the management of skull base chondrosarcomas. METHODS: We identified 493 patients with skull base chondrosarcoma using the Surveillance, Epidemiology, and End Results (SEER) registry (November 2017 submission). Regression analyses were performed to identify demographic variables associated with recommendation against surgery. Univariate and multivariate cox proportional hazards models were used for survival analysis. RESULTS: In a univariate analysis, we found that the African-American race was associated with an increased likelihood of surgeon recommendation against surgery (OR = 4.416, 95% CI = 1.893-10.302, p = 0.001). This association remained robust in the multivariate model that controlled for other covariates, including age of diagnosis (OR = 5.091, 95% CI = 2.127-12.187, p < 0.001). For patients who received a recommendation against surgery, the likelihood of dying from non-chondrosarcoma causes was comparable between Caucasian and African-American patients, suggesting that the prevalence and severity of medical conditions that increase the risk of death were comparable between these cohorts (HR = 0.466, 95% CI = 0.057-3.802, p = 0.475). The likelihood of dying from chondrosarcoma was comparable between Caucasian and African-American patients who underwent surgery (HR = 0.982, 95% CI = 0.353-2.732, p = 0.973), suggesting absence of race-specific surgical benefits. CONCLUSION: We identified a racial disparity against African-Americans in recommendations for surgical resection of skull base chondrosarcomas.

Dedifferentiated chondrosarcoma: current standards of care.

Dedifferentiated chondrosarcomas are aggressive variants of chondrosarcoma, associated with poor outcomes. Tumor biphasism is the norm. The majority of these tumors are symptomatic at presentation. Radiologically, large soft tissue masses with bony destruction predominate. Treatment protocols of these tumors are not well defined. Surgical resection forms the standard of care for localized disease. (Neo)adjuvant therapies remain controversial as the results from multiple (mainly retrospective) studies remain conflicting. Age at presentation, stage and ability to obtain negative resection margins are important prognostic factors. The overall prognosis is dismal. Newer and novel therapies targeting the complex genetic makeup of these tumors have renewed interest in the adjuvant setting that could hold promise in the near future.

Lay abstract Dedifferentiated chondrosarcomas are rare cancers composed of two components: a high-grade component and a low-grade component, with one abruptly blending into another. These rare tumors affect middle-aged individuals and present with pain and swelling in the affected site. X-rays and other scans often show tumor within the soft tissue with bony destruction. Although the precise treatment protocol is not well defined, surgery remains the standard of care for those where the tumor has not spread elsewhere. Once the disease spreads to other parts of the body, the outcome is very poor. The role of certain drugs targeting the tumor (chemotherapeutic agents) is controversial. This review briefly describes the genetic basis, treatment modalities involved and newer agents being developed for this lethal cancer.

Mobile Spine Osseous Sarcoma: Descriptive Epidemiological Analysis Based on a National Population-Based Study.

BACKGROUND: Primary osseous sarcomas of the mobile spine are rare bony tumors. Ewing sarcoma, chondrosarcoma, chordoma and osteosarcoma constitute the majority of primary bone sarcomas of the spine; however, other rare sarcoma tumors may also affect the spine. In order to perform an epidemiological study of theses tumors, national registries may help to evaluate data for populations with similar characteristics. METHODS: A population-based study was designed based on data from the Iran National Cancer Registry (INCR). All morphology codes (M-Code) of primary osseous sarcomas of the mobile spine (C-code 41.2) were derived and analyzed. RESULTS: Among 186 patients with primary osseous sarcomas of the mobile spine, 67.2% were men and 32.8% were women. The median (IQR) age was 37.0 (20.0-59.0) years and the age-standardized incidence rate (ASIR) was 0.37 per million. The majority of cases of Ewing sarcoma (29.5%) were observed in the age group 20-25 years. Among male patients with chondrosarcoma, the median age was 39.0 (30.0-50.0), while females showed a median age of 56.0 (50.0-59.0). The median age of patients with chordoma was 54.0 (47.0-63.0) years. The crude incidence rate of mobile spine osteosarcoma was 0.04 per million. CONCLUSION: Ewing sarcoma was the most frequent primary osseous sarcoma of the mobile spine. A male predilection was observed among all major sarcomas of the mobile spine. Ewing sarcoma in Iran affects the mobile spine in slightly older ages compared to other studies. Myxoid chondrosarcoma is the most frequent subtype of the mobile spine chondrosarcoma. Chordoma affects male in older ages compared to females. In contrast with other studies which showed a bimodal distribution of osteosarcoma of the spine including young adult and older age groups, 86% of cases in Iran were in the age group of 10-40 years.

Risk analysis of pulmonary metastasis of chondrosarcoma by establishing and validating a new clinical prediction model: a clinical study based on SEER database.

BACKGROUND: The prognosis of lung metastasis (LM) in patients with chondrosarcoma was poor. The aim of this study was to construct a prognostic nomogram to predict the risk of LM, which was imperative and helpful for clinical diagnosis and treatment. METHODS: Data of all chondrosarcoma patients diagnosed between 2010 and 2016 was queried from the Surveillance, Epidemiology, and End Results (SEER) database. In this retrospective study, a total of 944 patients were enrolled and randomly splitting into training sets (n = 644) and validation cohorts(n = 280) at a ratio of 7:3. Univariate and multivariable logistic regression analyses were performed to identify the prognostic nomogram. The predictive ability of the nomogram model was assessed by calibration plots and receiver operating characteristics (ROCs) curve, while decision curve analysis (DCA) and clinical impact curve (CIC) were applied to measure predictive accuracy and clinical practice. Moreover, the nomogram was validated by the internal cohort. RESULTS: Five independent risk factors including age, sex, marital, tumor size, and lymph node involvement were identified by univariate and multivariable logistic regression. Calibration plots indicated great discrimination power of nomogram, while DCA and CIC presented that the nomogram had great clinical utility. In addition, receiver operating characteristics (ROCs) curve provided a predictive ability in the training sets (AUC = 0.789, 95% confidence interval [CI] 0.789-0.808) and the validation cohorts (AUC = 0.796, 95% confidence interval [CI] 0.744-0.841). CONCLUSION: In our study, the nomogram accurately predicted risk factors of LM in patients with chondrosarcoma, which may guide surgeons and oncologists to optimize individual treatment and make a better clinical decisions. TRIAL REGISTRATION: JOSR-D-20-02045, 29 Dec 2020.

Clinicopathologic characteristics of laryngeal chondrosarcoma: An analysis of the National Cancer Database.

OBJECTIVES: Laryngeal Chondrosarcoma (LC) is a rare malignancy with limited studies documenting its clinicopathologic characteristics and treatment options. This study reports demographic and clinical determinants of outcomes for this rare tumor. METHODS: The National Cancer Database (NCDB) was queried for cases of LC reported from 2004-2016. 274 cases that met inclusion criteria were analyzed for demographic and clinicopathologic characteristics. Kaplan-Meier (KM) and Cox proportional hazard analyses were conducted to identify variables that impacted the overall survival of these patients. RESULTS: LC was found to be more common in males (74.8%). The mean age of patients was 61.8 years and 92.3% of the patients were white. 91.3% of patients were treated with only surgical resection, most commonly: partial laryngectomy (31.6%), total laryngectomy (25.7%), and local resection (22.4%). 98.8% of patients had no evidence of nodal disease and 99.6% of patients did not have distant metastasis at presentation. KM analysis revealed a 5-year overall survival (5YOS) of 89.0%. Age, insurance status, facility type, and surgery type were significant predictors of 5YOS (p<0.05). On Cox Proportional Hazard analysis, private insurance significantly improved survival (HR 0.21; p = 0.048) while increasing age was a poor prognostic indicator (HR 1.10; p = 0.004). CONCLUSION: The majority of LC patients present with no nodal involvement or distant metastasis at diagnosis, and overall this tumor has a favorable prognosis. Increasing age was found to be a poor prognostic factor while private insurance status was associated with improved survival.

Simultaneous development of COVID-19 pneumonia and pulmonary metastasis in a known case of chondrosarcoma: a case report.

BACKGROUND: The outbreak of coronavirus disease 2019 (COVID-19) started in December 2020, and is a global problem now. There are several sets of established data regarding computed tomography (CT) findings in COVID-19 pneumonia with many differential diagnoses. During the early days of the pandemic, there was little data regarding lung CT features of COVID-19 in a cancer patient. In this paper, we described a rare case of simultaneous presentation of COVID-19 with pulmonary metastasis. CASE PRESENTATION: A Persian patient with a history of chondrosarcoma presented to our clinic during the COVID-19 pandemic with a new-onset cough. He had experienced no recurrence during previous follow-up visits. Chest CT scan revealed numerous bilateral small peripheral and perilymphatic pulmonary nodules, unilateral ground-glass patch, and nodular interlobular septal thickening. Biopsy of the pulmonary nodules established pulmonary metastasis of chondrosarcoma origin, and pharyngeal reverse transcription polymerase chain reaction (RT-PCR) was positive for COVID-19. CONCLUSION: Pulmonary metastasis should be considered as a differential diagnosis of COVID-19 features in cancer patients in the pandemic era.

Incidence, Management, and Outcomes of Adult Patients with Spinal Chondrosarcoma in the United States.

OBJECTIVE: Spinal chondrosarcomas are rare primary malignant neoplasms composed of cartilage-producing cells. They are slow-growing but locally aggressive lesions that have high rates of recurrence and progression after treatment. We provide the largest comprehensive analysis of prognostic factors, treatment modalities, and survival outcomes in patients with spinal chondrosarcoma using a large, prospectively collected national database. METHODS: Patients with diagnosis codes specific for chondrosarcoma of the spine, sacrum, and coccyx were queried from the National Cancer Database (NCDB) during 2004-2016. Outcomes were investigated using Cox univariate and multivariate regression analyses, and survival curves were generated for comparative visualization. RESULTS: A total of 1843 individuals were identified with a diagnosis of chondrosarcoma, 82.1% of which were at the sacrum or coccyx and 17.9% at the spine. The mean overall survival of patients in our cohort was 7.91 years. Increased age, larger tumor, dedifferentiated histology, and presence of metastases were associated with worsened overall survival. Regarding management, 77.7% of patients received surgical intervention and both partial and radical resection were associated with significantly improved overall survival (P < 0.001). Neither radiotherapy nor chemotherapy administration improved overall survival; however, among patients who received radiation, those who received higher-dose radiation had significantly improved overall survival compared with those who received lower-dose radiation. CONCLUSIONS: Surgical resection significantly improves overall survival in patients with spinal chondrosarcoma. In those patients receiving radiation, those who receive high doses have improved overall survival compared with those who receive lower doses. Further studies into optimal radiation modality and doses are required.

The incidence and diagnostic relevance of pathological fracture in conventional central chondrosarcoma.

OBJECTIVE: To determine the incidence and diagnostic relevance of pathological fracture in patients with conventional central chondrosarcoma (CC-CS). MATERIALS AND METHODS: Retrospective review of patients with CC-CS diagnosed between January 2007 and December 2019. Data collected included age, sex, skeletal location and the presence of pathological fracture at presentation. Histological tumour grade based on surgical resection or needle biopsy was classified as atypical cartilaginous tumour (ACT)/grade 1 CS (ACT/Gd1 CS), high-grade CS (HG-CS) and dedifferentiated CS (DD-CS). The presence of pathological fracture was correlated with age, skeletal location and tumour grade. RESULTS: Three hundred seventeen patients were included (177 males and 140 females with mean age 55.8 years, range 9-91 years). Mean age of patients without pathological fracture was 54.4 years and those with pathological fracture 62.9 years (p = 0.002). The major long bones were involved in 171 cases, the flat bones in 112 cases, the mobile spine in 7 cases and the small bones of the hands and feet in 27 cases. There were 81 ACT/Gd 1 CS, 168 HGCS and 68 DD-CS. Pathological fracture was evident at presentation in 51 (16.1%) cases, the commonest bones involved being the femur (n = 21; 41.2%), humerus (n = 10; 19.6%) and acetabulum (n = 7; 13.7%). Pathological facture occurred in 7 cases of ACT/Gd 1 CS (13.7%), 23 cases of HGCS (45.1%) and 21 cases of DD-CS (41.2%) (p = 0.001). Following multivariate analysis, both older age and histological grade were independently significant factors. CONCLUSIONS: Pathological fracture was seen in 16.1% of patients with CC-CS. Pathological fractures in the femur, humerus and acetabulum very likely indicate higher tumour grade.

Extraskeletal myxoid chondrosarcoma: A high incidence of metastatic disease to lymph nodes.

BACKGROUND: Extraskeletal myxoid chondrosarcoma (ESMC) is a rare type of soft-tissue sarcoma with limited series reporting outcome of treatment. Currently there is limited data on the incidence and impact on patient outcome in those with metastatic disease to lymph nodes in ESMC. METHODS: Thirty (21 males, 9 females) patients, mean age 50 ± 16 years, with ESMC were reviewed. The tumors were most commonly located in the lower extremity (n = 23, 77%) and the mean tumor size and volume were 9 ± 5 cm and 490 ± 833 cm3 . Mean follow up was 7 ± 4 years. RESULTS: Six (20%) patients either presented (n = 3, 10%) or developed (n = 3, 10%) lymph node metastatic disease. When comparing patients without, with lymph node metastasis and metastasis elsewhere, patients with lymph nodes metastasis had worse survival than those without metastasis, however better 10-year disease specific survival than those with metastasis elsewhere (100% vs 62% vs 0%; P < .001). CONCLUSION: There is a high incidence of lymph node metastatic disease in patients with ESMC. Although survival in these patients is worse compared to those without metastasis, their survival is better than those with metastasis elsewhere. Due to the high incidence of lymph node metastatic disease, preoperative staging of the lymph node should be considered.

Incidence and survival rates and trends of skull Base chondrosarcoma: A Population-Based study.

INTRODUCTION: Chondrosarcomas are extremely rare, locally invasive, and potentially mortal malignant cartilaginous tumors. In this study, we aimed to evaluate the incidence and survival rates and trends of skull base chondrosarcomas (SBC). METHODS: Data from SBC patients between 1975 and 2017 were extracted from the Surveillance, Epidemiology, and End Results (SEER) database. The age-adjusted incidence rates (AAR) were calculated for the overall cases and based on gender, age, race, and histology. Furthermore, the relative survival rates for one, three, and five years, and the rates stratified to the aforementioned selected variables were computed. Besides, we conducted a joint point regression analysis to calculate the annual percent change (APC) and its associated standard error (SE) for AAR and mortality. RESULTS: The AAR rate of SBC was 0.019 per 100,000. Higher AAR rates were observed in patients who were in the 65-74-year-age-group, females, Caucasians, and had none mesenchymal subtype. The relative one-year, three-year and five-year-survival rates were 99.58 %, 93.67 %, and 89.10 %, respectively. Lower survival rates were noted in patients who were males, African Americans, and had a mesenchymal subtype. The trend analysis has shown a significant yearly increase (P < 0.001) in AAR of SBC (APC ±â€¯SE = 0.0005 %±0.0001), along with a significant yearly decline in mortality rates (APC ±â€¯SE= -0.0202 %±0.0029). CONCLUSION: Despite the increase in AAR over time, there has been a significant decline in mortality rates over time, which might have been due to the advancement of treatment modalities, improvement in diagnostic imaging, and modification in disease grading.

Evaluation of factors affecting survival rate in primary bone sarcomas with extremity and pelvis involvement.

OBJECTIVE: This study is an evaluation of the overall survival rate and factors affecting survival in patients with osteosarcoma, chondrosarcoma, or Ewing's sarcoma. This study aimed to determine the effect of factors related to the preoperative period, patient, tumor, treatment, and postoperative period on survival. METHODS: A total of 114 patients (64 male and 50 female) with osteosarcoma, chondrosarcoma, or Ewing's sarcoma treated between 2005 and 2013 were included in this study. All the patients received standard treatment and were followed up regularly. In all, 44 cases of (conventional and telangiectatic) osteosarcoma, 30 cases of Ewing's sarcoma, and 40 cases of high-grade chondrosarcoma were identified using the Bone and Soft Tissue Tumor Registry. Gender, age, tumor size and localization, pathological fractures, histopathological type, grade, surgical treatment, adjuvant treatments, relapse of the disease, and postoperative complication data were obtained from follow-up forms. The learning curve of institutional expertise was also evaluated. The patient survival rate was calculated using the Kaplan-Meier method, and log-rank statistical methods were used to compare survival rates. RESULTS: The mean length of survival of the patients was 72 months. There was a 56% 5-year survival rate, and the event-free survival rate was 53%. The survival of patients with Ewing's sarcoma whose prodromal period was less than 12 weeks was significantly higher than that of the other groups (p=0.031). The survival of patients with tumor size greater than 150 cc, with local recurrence and distant metastases was low for all groups. Survival rates were significantly lower in osteosarcoma and Ewing's sarcoma patients with stage III tumor or metastasis at diagnosis. The survival of patients with osteosarcoma diagnosed between 2010 and 2013 was significantly higher than that of the earlier group (p=0.02). CONCLUSION: Decreasing the prodromal period (early diagnosis) can improve survival by preventing the local and systemic spread of the tumor. Increase in the surgical experience is likely to have a positive effect on survival rates, especially for patients with osteosarcoma. The relapse of the disease is a poor prognostic factor for survival despite aggressive surgery and adjuvant therapies. LEVEL OF EVIDENCE: Level IV, Therapeutic study.

Natural history of Ollier disease and Maffucci syndrome: Patient survey and review of clinical literature.

Ollier disease (OD) and Maffucci syndrome (MS) are characterized by multiple enchondromas. Patients with MS also have benign vascular overgrowths that become malignant in 8.5% of cases. OD is characterized by multiple enchondromas, typically unilateral in distribution with a predilection for the appendicular skeleton. MS is characterized by multiple enchondromas bilaterally distributed in most of the cases. Both disorders feature multiple swellings on the extremity, deformity around the joints, limitations in joint mobility, scoliosis, bone shortening, leg-length discrepancy, gait disturbances, pain, loss of function, and pathological fractures. About 50% of patients with OD or MS develop a malignancy, such as chondrosarcoma, glioma, and ovarian juvenile granulosa cell tumor. To better understand the natural history of OD and MS, we reviewed 287 papers describing patients with OD and MS. We also created a survey that was distributed directly to 162 patients through Facebook. Here, we compare the review of the cases described in the literature to the survey's responses. The review of the literature showed that: the patients with OD are diagnosed at a younger age; the prevalence of chondrosarcomas among patients with OD or MS was ~30%; in four patients, vascular anomalies were identified in internal organs only; and, the prevalence of cancer among patients with OD or MS was ~50%. With these data, health care providers will better understand the natural history, severity, and prognosis of these diseases and the prevalence of malignancies in these patients. Here, we recommend new guidelines for the care of patients with OD and MS.

Chondrosarcomas in adolescents: are they different?

The objective of this study was to evaluate the characteristics and oncologic outcomes of a single institution series of chondrosarcoma of pelvis and extremities in patients who were 21 years or younger. Between January 2006 and December 2016, we operated on 249 chondrosarcomas of the pelvis and extremities. Eleven (4%) of these were 21 years or younger. The mean age was 18 years (range 14-21 years). There were eight males and three females. Four occurred in the pelvis. The other sites were humerus (three) and one each in the scapula, clavicle, metatarsal and proximal femur. There were five primary chondrosarcomas and 6 (55%) secondary chondrosarcomas. After appropriate surgical excision, we had nine free margins and two margins were microscopically involved. None of the 11 patients received adjuvant chemotherapy or radiotherapy. One patient never followed up after surgery and another was lost to follow-up after 32 months. Eight were alive and one had died. All survivors had minimum follow-up of 2 years (range 26-106 months). There was one local recurrence. The overall and disease-free survival at 5 years for all patients was 89 %. There was no statistically significant difference in survival for primary chondrosarcomas compared to secondary chondrosarcomas (P = 0.061), or pelvic chondrosarcomas compared to other sites (P = 0.264). Chondrosarcomas in children and adolescents constitute <5% of all chondrosarcomas. Secondary chondrosarcomas constituted more than half the cases. Overall, outcomes in the young were no different from those in adults.