Atypical Cartilaginous Tumors Research Articles

Follow-up analysis of lesion characteristics of enchondromas and atypical cartilaginous tumours of the knee and shoulder region on MRI.

Enchondromas (ECs) and atypical cartilaginous tumours (ACTs), respectively, represent benign and intermediate cartilaginous bone tumours. Differentiation between these tumour entities bears difficulties, as histology and MRI cannot always provide exact diagnoses. Observation of the natural course of ECs/ACTs via follow-up MRIs might support tumour distinction without needing biopsy harbouring sampling error. Reports of patients that had undergone MRI exams of the knee (n = 44.762) or shoulder (n = 21.550) at a single radiology institute between 01.01.2007 and 01.03.2020 were searched for ECs/ACTs with at least one follow-up MRI. Scans of 176 patients (with 182 cartilage lesions) fulfilling these criteria were subsequently re-examined together with corresponding MRI reports to evaluate morphological tumour development over time, focusing on potential alterations of lesion size, tumour-related oedema, and scalloping. Median follow-up time was 27 ± 53 months for knee tumours and 26 ± 32 months for shoulder lesions. Presence of tumour growth was significantly higher in ACTs than in ECs both at the knee (p = 0.04) and shoulder (p = 0.03). While ACTs were associated with median tumour growth rates of 0.039 mm/month (knee) and 0.083 mm/month (shoulder), ECs of the knee and shoulder showed lower median growth rates equivalent to 0.0 mm/month (p < 0.01, p < 0.01). ECs and ACTs both presented stable regarding tumour-related oedema and scalloping during follow-up. ACTs and ECs show different tumour growth rates. Growth rates are slow for both, ECs and ACTs, supporting the current concept of watchful waiting. ECs may decrease in size. Follow-up MRIs may support the radiological differentiation of cartilage lesions. Question Both singular MRI and histological examination have limitations regarding differentiation of enchondromas (EC) and atypical cartilaginous tumours (ACTs). Findings Median ACT growth rates were 0.039 mm/month (knee) and 0.083 mm/month (shoulder), while median growth rates of EC in the knee and shoulder were 0.0 mm/month. Clinical relevance Active surveillance is a safe strategy when dealing with ECs and ACTs of the long bones; follow-up MRIs may support tumour distinction of cartilage lesions, as ECs and ACTs show different growth behaviour.

Optimal Magnetic Resonance Sequence for Assessment of Central Cartilage Tumor Scalloping

Abstract Background Magnetic resonance imaging (MRI) is key in evaluating central cartilage tumors. The BACTIP (Birmingham Atypical Cartilaginous Tumour Imaging Protocol) protocol assesses central cartilage tumor risk based on the tumor size and degree of endosteal scalloping on MRI. It provides a management protocol for assessment, follow-up, or referral of central cartilage tumors. Objective Our study compared four MRI sequences: T1-weighted (T1-w), fluid sensitive (Short Tau Inversion Recovery (STIR)- weighted, STIR-w), and grayscale inversions (T1-w GSI and short tau inversion recovery [STIR] GSI) to see how reliably endosteal scalloping was detected. Materials and Methods Two senior consultant musculoskeletal radiologists with experience reviewed randomly selected 60 representative central cartilage tumor cases with varying degree of endosteal scalloping to reflect a spectrum of BACTIP pathologies. The endosteal scalloping was graded as per the definition of BACTIP A, B, and C. They agreed on a consensus BACTIP grade for each of the 240 key images (60 cases × 4 sequences), which was considered the final “consensus” BACTIP grade. These 240 images were then randomized into a test set and given to two fellowship-trained consultant musculoskeletal radiologists for analysis. They assigned a BACTIP grade to each of the 240 selected images while being blinded to the final “consensus” BACTIP grade. The training set was further subdivided into three groups based on the MR image quality (good quality, average quality, and poor quality) to ascertain if the quality of the acquired images influenced intraobserver and interobserver agreements on the BACTIP grading. The two observers were blinded to the grade assigned to the image quality. Results Linearly weighted kappa analysis was performed to measure the agreement between the BACTIP grading answers by two observers and the “consensus” BACTIP grading answers, as well as the BACTIP grading agreement between the two observers themselves.The analysis revealed that T1-w and STIR-w sequences demonstrated more consistent and higher agreement across different image qualities. However, the T1-w GSI and STIR-w GSI sequences exhibited lower agreement, particularly for poor-quality images. T1-w imaging demonstrated substantial agreement between BACTIP gradings for poor-quality images, suggesting potential resilience of T1-w sequence in challenging imaging conditions. Conclusion T1-w imaging is the best sequence for BACTIP grading of endosteal scalloping, followed by fluid-sensitive STIR sequences.

Computed tomography-based radiomics machine learning models for differentiating enchondroma and atypical cartilaginous tumor in long bones.

To explore the value of CT-based radiomics machine learning models for differentiating enchondroma from atypical cartilaginous tumor (ACT) in long bones and methods to improve model performance.59 enchondromas and 53 ACTs in long bones confirmed by pathology were collected retrospectively. The features were extracted from preoperative CT images of these patients, and least absolute shrinkage and selection operator (LASSO) regression was used for feature selection and dimensionality reduction. The selected features were used to construct classification models by thirteen machine learning algorithms. The data set was randomly divided into a training set and a test set at a proportion of 7:3 by ten-fold cross-validation to evaluate the performance of these models.A total of 1199 features were extracted, 9 features were selected, and 13 radiomics machine learning models were constructed. The area under the curve (AUC) of 11 models was more than 0.8, and that of 3 models was more than 0.9. The Extremely Randomized Trees model achieved the best performance (AUC = 0.9375 ± 0.0884), followed by the Adaptive Boosting model (AUC = 0.9188 ± 0.1010) and the Linear Discriminant Analysis model (AUC = 0.9062 ± 0.1459).CT-based radiomics machine learning models had great ability to distinguish enchondroma and ACT in long bones. By using filters to deeply mine high-order features in the original image and selecting appropriate machine learning algorithms, the performance of the model can be improved. · CT-based radiomics machine learning models can distinguish enchondroma and ACT in long bones.. · Using filters and selecting advanced machine learning algorithms can improve model performance.. · Clinical features have limited utility in distinguishing enchondroma and ACT in long bones.. · Hong R, Li Q, Ma J et al. Computed tomography-based radiomics machine learning models for differentiating enchondroma and atypical cartilaginous tumor in long bones. Fortschr Röntgenstr 2024; DOI 10.1055/a-2344-5398.

X-rays radiomics-based machine learning classification of atypical cartilaginous tumour and high-grade chondrosarcoma of long bones

Atypical cartilaginous tumour (ACT) and high-grade chondrosarcoma (CS) of long bones are respectively managed with active surveillance or curettage and wide resection. Our aim was to determine diagnostic performance of X-rays radiomics-based machine learning for classification of ACT and high-grade CS of long bones. This retrospective, IRB-approved study included 150 patients with surgically treated and histology-proven lesions at two tertiary bone sarcoma centres. At centre 1, the dataset was split into training (n=71 ACT, n=24 high-grade CS) and internal test (n=19 ACT, n=6 high-grade CS) cohorts, respectively, based on the date of surgery. At centre 2, the dataset constituted the external test cohort (n=12 ACT, n=18 high-grade CS). Manual segmentation was performed on frontal view X-rays, using MRI or CT for preliminary identification of lesion margins. After image pre-processing, radiomic features were extracted. Dimensionality reduction included stability, coefficient of variation, and mutual information analyses. In the training cohort, after class balancing, a machine learning classifier (Support Vector Machine) was automatically tuned using nested 10-fold cross-validation. Then, it was tested on both the test cohorts and compared to two musculoskeletal radiologists' performance using McNemar's test. Five radiomic features (3 morphology, 2 texture) passed dimensionality reduction. After tuning on the training cohort (AUC=0.75), the classifier had 80%, 83%, 79% and 80%, 89%, 67% accuracy, sensitivity, and specificity in the internal (temporally independent) and external (geographically independent) test cohorts, respectively, with no difference compared to the radiologists (p≥0.617). X-rays radiomics-based machine learning accurately differentiates between ACT and high-grade CS of long bones. AIRC Investigator Grant.

A retrospective external validation study of the Birmingham Atypical Cartilage Tumour Imaging Protocol (BACTIP) for the management of solitary central cartilage tumours of the proximal humerus and around the knee.

This study aimed to externally validate the Birmingham Atypical Cartilage Tumour Imaging Protocol (BACTIP) recommendations for differentiation/follow-up of central cartilage tumours (CCTs) of the proximal humerus, distal femur, and proximal tibia and to propose BACTIP adaptations if the results provide new insights. MRIs of 123 patients (45 ± 11years, 37 men) with an untreated CCT with MRI follow-up (n = 62) or histopathological confirmation (n = 61) were retrospectively/consecutively included and categorised following the BACTIP (2003-2020 / Ghent University Hospital/Belgium). Tumour length and endosteal scalloping differences between enchondroma, atypical cartilaginous tumour (ACT), and high-grade chondrosarcoma (CS II/III/dedifferentiated) were evaluated. ROC-curve analysis for differentiating benign from malignant CCTs and for evaluating the BACTIP was performed. For lesion length and endosteal scalloping, ROC-AUCs were poor and fair-excellent, respectively, for differentiating different CCT groups (0.59-0.69 versus 0.73-0.91). The diagnostic performance of endosteal scalloping and the BACTIP was higher than that of lesion length. A 1° endosteal scalloping cut-off differentiated enchondroma from ACT + high-grade chondrosarcoma with a sensitivity of 90%, reducing the potential diagnostic delay. However, the specificity was 29%, inducing overmedicalisation (excessive follow-up). ROC-AUC of the BACTIP was poor for differentiating enchondroma from ACT (ROC-AUC = 0.69; 95%CI = 0.51-0.87; p = 0.041) and fair-good for differentiation between other CCT groups (ROC-AUC = 0.72-0.81). BACTIP recommendations were incorrect/unsafe in five ACTs and one CSII, potentially inducing diagnostic delay. Eleven enchondromas received unnecessary referrals/follow-up. Although promising as a useful tool for management/follow-up of CCTs of the proximal humerus, distal femur, and proximal tibia, five ACTs and one chondrosarcoma grade II were discharged, potentially inducing diagnostic delay, which could be reduced by adapting BACTIP cut-off values. Mostly, Birmingham Atypical Cartilage Tumour Imaging Protocol (BACTIP) assesses central cartilage tumours of the proximal humerus and the knee correctly. Both when using the BACTIP and when adapting cut-offs, caution should be taken for the trade-off between underdiagnosis/potential diagnostic delay in chondrosarcomas and overmedicalisation in enchondromas. • This retrospective external validation confirms the Birmingham Atypical Cartilage Tumour Imaging Protocol as a useful tool for initial assessment and follow-up recommendation of central cartilage tumours in the proximal humerus and around the knee in the majority of cases. • Using only the Birmingham Atypical Cartilage Tumour Imaging Protocol, both atypical cartilaginous tumours and high-grade chondrosarcomas (grade II, grade III, and dedifferentiated chondrosarcomas) can be misdiagnosed, excluding them from specialist referral and further follow-up, thus creating a potential risk of delayed diagnosis and worse prognosis. • Adapted cut-offs to maximisedetection of atypical cartilaginous tumours and high-grade chondrosarcomas, minimiseunderdiagnosis and reduce potential diagnostic delay in malignant tumours but increase unnecessary referral and follow-up of benign tumours.

Wait-and-scan: an alternative for curettage in atypical cartilaginous tumours of the long bones.

Due to its indolent clinical behaviour, the treatment paradigm of atypical cartilaginous tumours (ACTs) in the long bones is slowly shifting from intralesional resection (curettage) and local adjuvants, towards active surveillance through wait-and-scan follow-up. In this retrospective cohort study performed in a tertiary referral centre, we studied the natural behaviour of ACT lesions by active surveillance with MRI. Clinical symptoms were not considered in the surveillance programme. The aim of this study was to see whether active surveillance is safe regarding malignant degeneration and local progression. In total, 117 patients were evaluated with MRI assessing growth, cortical destruction, endosteal scalloping, periosteal reaction, relation to the cortex, and perilesional bone marrow oedema. Patients received up to six follow-up scans. At the time of the first follow-up MRI, 8% of the lesions showed growth (n = 9), 86% remained stable (101), and 6% decreased in size (n = 7). During the third follow-up, with a mean follow-up time of 60 months (SD 23), 24 patients were scanned, of whom 13% had lesions that had grown and 13% lesions that had decreased in size. After 96 months (SD 37), at the sixth follow-up MRI, 100% of the lesions remained stable. None of the lesions showed malignant progression and although some lesions grew in size (mean 1 mm (SD 0.8)), no malignant progression occurred. We conclude that active surveillance with MRI is safe for ACTs in the long bones in the short- and mid-term follow-up.

Fracture risk after intralesional curettage of atypical cartilaginous tumors

IntroductionThe need for curettage of atypical cartilaginous tumors (ACT) is under debate. Curretage results in defects that weaken the bone potentially leading to fractures. The purpose of this study was to retrospectively determine postoperative fracture risk after curettage of chondroid tumors, including patient-specific characteristics that could influence fracture risk.MethodsA total of 297 adult patients who underwent curettage of an ACT followed by phenolisation and augmentation were retrospectively evaluated. Explanatory variables were, sex, age, tumor size, location, augmentation type, and plate fixation. The presence of a postoperative fracture was radiologically diagnosed. Included patients had at least 90 days of follow-up.ResultsA total of 183 females (62%) were included and 114 males (38%), with an overall median follow-up of 3.2 years (IQR 1.6–5.2). Mean diameter of the lesions was 4.5 (SD 2.8) cm. Patients received augmentation with allograft bone (n = 259, 87%), PMMA (n = 11, 3.7%), or did not receive augmentation (n = 27, 9.1%). Overall fracture risk was 6%. Male sex (p = 0.021) and lesion size larger than 3.8 cm (p < 0.010) were risk factors for postoperative fracture.InterpretationCurettage of ACT results in an overall fracture risk of 6%, which is increased for males with larger lesions.

Magnetic Resonance Imaging Features and Prognostic Indicators of Local Recurrence after Curettage and Cementation of Atypical Cartilaginous Tumour in the Appendicular Skeleton.

Objective: The aim of this study is to determine MRI features that may be prognostic indicators of local recurrence (LR) in patients treated with curettage and cementation of atypical cartilaginous tumours (ACTs) in the appendicular skeleton. Materials and Methods: This study is a retrospective review of adult patients with histologically confirmed appendicular ACT. The data collected included age, sex, skeletal location and histology from curettage, the presence of LR and oncological outcomes. The pre-operative MRI characteristics of the ACT reviewed by a specialist MSK radiologist included lesion location, lesion length, degree of medullary filling, bone expansion, cortical status and the presence of soft tissue extension. Results: A total of 43 patients were included, including 9 males and 34 females with a mean age of 42.8 years (range: 25-76 years). Tumours were located in the femur (n = 19), humerus (n = 15), tibia (n = 5), fibula (n = 2) and radius and ulna (n = 1 each). A total of 19 lesions were located in the diaphysis, 12 in the metadiaphysis, 6 in the metaphysis and 6 in the epiphysis. The mean tumour length was 61.0 mm (range: 12-134 mm). The mean follow up was 97.7 months (range: 20-157 months), during which 10 (23.3%) patients developed LR, 7 (70%) of which were asymptomatic and 3 (30%) of which presented with pain. Four patients required repeat surgery with no associated death or evidence of metastatic disease. LR was significantly commoner with tumours arising in the epiphysis or metadiaphysis, but no MRI features were predictive of LR. Conclusions: No relationship was found between the apparent 'aggressiveness' of an ACT of the appendicular skeleton on MRI and the development of LR following treatment with curettage and cementation.

SPECT/CT Radiomics for Differentiating between Enchondroma and Grade I Chondrosarcoma.

This study was performed to assess the value of SPECT/CT radiomics parameters in differentiating enchondroma and atypical cartilaginous tumors (ACTs) located in the long bones. Quantitative HDP SPECT/CT data of 49 patients with enchondromas or ACTs in the long bones were retrospectively reviewed. Patients were randomly split into training (n = 32) and test (n = 17) data, and SPECT/CT radiomics parameters were extracted. In training data, LASSO was employed for feature reduction. Selected parameters were compared with classic quantitative parameters for the prediction of diagnosis. Significant parameters from training data were again tested in the test data. A total of 12 (37.5%) and 6 (35.2%) patients were diagnosed as ACTs in training and test data, respectively. LASSO regression selected two radiomics features, zone-length non-uniformity for zone (ZLNUGLZLM) and coarseness for neighborhood grey-level difference (CoarsenessNGLDM). Multivariate analysis revealed higher ZLNUGLZLM as the only significant independent factor for the prediction of ACTs, with sensitivity and specificity of 85.0% and 58.3%, respectively, with a cut-off value of 191.26. In test data, higher ZLNUGLZLM was again associated with the diagnosis of ACTs, with sensitivity and specificity of 83.3% and 90.9%, respectively. HDP SPECT/CT radiomics may provide added value for differentiating between enchondromas and ACTs.

Ki-67 Index as an Adjunct Histological Marker for Grading Cartilage Tumors: A Pilot Study

Objectives Distinguishing enchondromas (grade 0) from grade 1 chondrosarcomas, recently classified as atypical cartilaginous tumors (ACT), is challenging due to histologic and radiographic similarity. However, diagnostic ambiguity poses a management dilemma: enchondromas are observed, while ACTs are excised. Protein Ki-67 is a cellular proliferation nuclear marker used as a proxy for aggressiveness in other tumors. This retrospective pilot study aimed to characterize the association between grade and Ki-67 index and evaluate its ability to aid differentiation between critical cartilage tumor grades. Methods Tissue blocks from 26 patients treated for enchondromas or chondrosarcomas within the past ten years were exposed to anti-Ki-67 rabbit monoclonal antibody and hematoxylin counterstain. Counting two high-power fields per sample yielded a Ki-67 index. Results There was a strong positive correlation between tumor grade and the Ki-67 index (r = 0.704, P&lt;0.001). One-way analysis of variance (ANOVA) showed significant differences in the Ki-67 index between grades (P&lt;0.001). Tukey’s post-hoc test demonstrated that enchondroma Ki-67 indexes were not significantly different from ACT Ki-67 indexes (P=0.094). Receiver operator curve cutoff index value of 13.4% differentiated enchondroma vs. ACT with 67.9% sensitivity and 42.9% specificity. Conclusion Ki-67 index is correlated with cartilage tumor grade. Although our study did not show a significant difference in the Ki-67 index between enchondromas from ACTs, this study provided a protocol for future investigations. Study limitations include small sample size and reliance on historical tissue blocks of variable preservation quality.

Intravoxel Incoherent Motion and Dynamic Contrast-Enhanced Magnetic Resonance Imaging Can Differentiate Between Atypical Cartilaginous Tumors and High-Grade Chondrosarcoma: Correlation With Histological Vessel Characteristics.

To differentiate between atypical cartilaginous tumors and high-grade chondrosarcoma of the major long bones using intravoxel incoherent motion (IVIM) and Dynamic Contrast-Enhanced magnetic resonance imaging (DCE-MRI), and explore the correlation of quantitative parameters with hypoxia inducible factor-1α (HIF-1α), vascular endothelial growth factor (VEGF) and microvessel density (MVD). Between September 2016 and March 2022, 35 patients (17 atypical cartilaginous tumors, 18 high-grade chondrosarcoma) underwent MRI examination and pathological confirmation at our hospital. First, IVIM-derived parameters ( D , D* , and f ), and DCE-MRI parameters ( Ktrans , Kep , and V e ) were measured, and intraclass correlation efficient (ICC) and Mann-Whitney U test were performed. Second, receiver-operating characteristic curve analysis was performed to evaluate the diagnostic performance. Finally, Spearman's correlation analysis was performed between the quantitative parameters of IVIM-DWI and DCE-MRI and the immunohistochemical factors HIF-1α, VEGF, and MVD in chondrosarcoma tissue. D in atypical cartilaginous tumors was significantly higher than that in high-grade chondrosarcoma ( P = 0.003), whereas D* , Ktrans , and K ep in atypical cartilaginous tumors were significantly lower than those in high-grade chondrosarcoma (all P < 0.001). Ktrans demonstrated the highest area under the curve (AUC) of 0.979. The D* , Ktrans , and K ep were positively correlated with HIF-1α, VEGF, and MVD (all P < 0.001), whereas D had no correlation with HIF-1α, VEGF, and MVD ( P = 0.113, 0.077, 0.058, respectively). The IVIM-DWI quantitative parameters ( D , D* ) and DCE-MRI quantitative parameters ( Ktrans , Kep ) are helpful to differentiate between atypical cartilaginous tumors and high-grade chondrosarcoma and could be imaging biomarkers to reflect the expressions of HIF-1α, VEGF, and angiogenesis of chondrosarcoma.

Prevalence and characteristics of benign cartilaginous tumours of the shoulder joint. An MRI-based study

ObjectiveEnchondromas (EC) of the shoulder joint are benign intraosseous cartilage neoplasms, with atypical cartilaginous tumours (ACT) representing their intermediate counterpart. They are usually found incidentally on clinical imaging performed for other reasons. Thus far the prevalence of ECs of the shoulder has been analysed in only one study reaching a figure of 2.1%.Materials and methodsThe aim of the current study was to validate this number via retrospective analysis of a 45 times larger, uniform cohort consisting of 21.550 patients who had received an MRI of the shoulder at a single radiologic centre over a time span of 13.2 years.ResultsNinety-three of 21.550 patients presented with at least one cartilaginous tumour. Four patients showed two lesions at the same time resulting in a total number of 97 cartilage tumours (89 ECs [91.8%], 8 ACTs [8.2%]). Based on the 93 patients, the overall prevalence was 0.39% for ECs and 0.04% for ACTs. Mean size of the 97 ECs/ACTs was 2.3 ± 1.5 cm; most neoplasms were located in the proximal humerus (96.9%), in the metaphysis (60.8%) and peripherally (56.7%). Of all lesions, 94 tumours (96.9%) were located in the humerus and 3 (3.1%) in the scapula.ConclusionFrequency of EC/ACT of the shoulder joint appears to have been overestimated, with the current study revealing a prevalence of 0.43%.

Prevalence and characteristics of benign cartilaginous tumours of the knee joint as identified on MRI scans

BackgroundEnchondromas (EC) and atypical cartilaginous tumours (ACT) of the knee joint represent benign/intermediate chondromatous neoplasms of the bone that are most commonly discovered incidentally. Based on small to intermediate-sized cohorts, the prevalence of cartilaginous tumours of the knee as visible in MRI is estimated at 0.2–2.9%. This study aimed at verifying/challenging these numbers via retrospective examination of a larger, uniform patient cohort.MethodsBetween 01.01.2007 and 01.03.2020, 44,762 patients had received an MRI of the knee for any indication at a radiologic centre. Of these, 697 patients presented with MRI reports positive for cartilaginous lesions. In a three-step workflow, 46 patients were excluded by a trained co-author, a radiologist and an orthopaedic oncologist, as wrongly being diagnosed for a cartilage tumour.ResultsOf 44,762 patients, 651 presented with at least one EC/ACT indicating a prevalence of 1.45% for benign/intermediate cartilaginous tumours of the knee joint (EC: 1.4%; ACTs: 0.05%). As 21 patients showed 2 chondromatous lesions, altogether 672 tumours (650 ECs [96.7%] and 22 ACTs [3.3%]) could be analysed in terms of tumour characteristics: With a mean size of 1.6 ± 1.1 cm, most lesions were located in the distal femur (72.9%), in the metaphysis of the respective bone (58.9%) and centrally in the medullary canal (57.4%).ConclusionsThis study revealed an overall prevalence of 1.45% for cartilage lesions around the knee joint. Whilst a constant increase in prevalence was found for ECs over 13.2 years, prevalence remained constant for ACTs.

Low-grade chondrosarcoma and atypical cartilaginous tumor

Central atypical cartilaginous tumor (ACT)/chondrosarcoma, grade 1 (CS1) is a locally aggressive chondrogenic tumor, as the 5th WHO Classification of Tumors (2020) listed. These lesions, that need for a resection as management, can resemble to enchondroma that is a fully benign lesion. This last is simply followed on the imaging to avoid the risk of possible complications. We will review the clinical and imaging features that allow to the radiologist to make a differential diagnosis among these lesions in order to choose for each lesion the most appropriate diagnostic/therapeutical management.

Chondrosarcoma: A Clinical Review.

Chondrosarcomas are a diverse group of malignant cartilaginous matrix-producing neoplasms. Conventional chondrosarcomas are a continuum of disease based on the biologic activity of the tumor. The tumors range from the relatively biologically benign low-grade tumors or intermediate atypical cartilaginous tumors (ACTs), to malignant, aggressive high-grade tumors. The clinical presentation, radiographic and pathologic findings, treatments and outcomes vary significantly based on the histologic grade of the tumor. Chondrosarcomas present a diagnostic dilemma, particularly in the differentiation between high- and intermediate-grade tumors and that of low-grade tumors from benign enchondromas. A multidisciplinary team at a tertiary sarcoma centre allows for optimal care of these patients.

Active surveillance of atypical cartilaginous tumours of bone: short term quality of life measurements

BackgroundIn the recent years active surveillance has been introduced for atypical cartilaginous tumours (ACT). This is the first study on the impact of this new treatment approach on patients’ quality-of-life. We evaluated general health-related quality of life (HRQL) in patients diagnosed with enchondroma or ACT.MethodsIn this prospective study, patients recently diagnosed with enchondroma and ACT of the long bones were asked to participate. Health-related quality of life (HRQL) was assessed at diagnosis and at six month follow-up, using the 36-item Short Form Health Survey (SF-36) and Numeric Pain Rating Scale (NRS). HRQL of the active surveillance group was compared to the Dutch population and a Dutch sample with locoregional cancer.ResultsIn total, 45 patients were included in the study, of which four patients underwent curettage and cryosurgery, 41 patients were under active surveillance. The HRQL of the active surveillance group seemed lower compared to the Dutch population, but similar to patients suffering locoregional cancers. No comparison between the surgery and the active surveillance group could be made. In the active surveillance group no statistical difference was found between baseline and six months follow-up regarding HRQL and pain during rest and activities.ConclusionPatients diagnosed with non-malignant chondroid tumours have lower HRQL compared to the healthy population. Active surveillance had no adverse effect on patients well-being, after six months active surveillance the HRQL remained unchanged. Interestingly, in our study no impact on mental health was seen, implicating that diagnosed but untreated chondroid tumours do not seem to influence patients anxiety.

Classification of Chondrosarcoma: From Characteristic to Challenging Imaging Findings.

Chondrosarcomas can be classified into various forms according to the presence or absence of a precursor lesion, location, and histological subtype. The new 2020 World Health Organization (WHO) Classification of Tumors of Soft Tissue and Bone classifies chondrogenic bone tumors as benign, intermediate (locally aggressive), or malignant, and separates atypical cartilaginous tumors (ACTs) and chondrosarcoma grade 1 (CS1) as intermediate and malignant tumors. respectively. Furthermore, the classification categorizes chondrosarcomas (including ACT) into eight subtypes: central conventional (grade 1 vs. 2-3), secondary peripheral (grade 1 vs. 2-3), periosteal, dedifferentiated, mesenchymal, and clear cell chondrosarcoma. Most chondrosarcomas are the low-grade, primary central conventional type. The rarer subtypes include clear cell, mesenchymal, and dedifferentiated chondrosarcomas. Comprehensive analysis of the characteristic imaging findings can help differentiate various forms of chondrosarcomas. However, distinguishing low-grade chondrosarcomas from enchondromas or high-grade chondrosarcomas is radiologically and histopathologically challenging, even for experienced radiologists and pathologists.

Concurrent intraosseous cartilaginous lesions in patients with multiple osteochondromas identified on total-body MR imaging.

To assess the prevalence of intraosseous cartilaginous lesions in patients with multiple osteochondromas based on total-body (TB) MRI examinations, used for screening purposes. Between 2013 and 2020, TB-MRI examinations were performed in 366 patients with proven multiple osteochondromas syndrome, to rule out malignant progression. For this study, presence, or absence of intraosseous central or eccentrical chondroid lesions, defined as lobulated lesions with low signal intensity on T1-weighted images, replacing bone marrow and high signal intensity equal to fluid on T2-weighted images in the bone marrow of the meta-diaphysis of (one of) the long bones, were recorded in the long bones as part of a TB-MRI protocol. In 62 patients out of the 366 MO patients (17%), one or more intraosseous chondroid lesions (either enchondroma or atypical cartilaginous tumor) were detected. The age of the patients at time of diagnosis ranged from 17 to 61years (mean, 36). Size of the lesions varied from 4 to 69mm (mean, 16.3mm). The most common location was the proximal femur (n = 29), followed by the distal femur and proximal humerus (n = 18 and n = 10, respectively). In nine of the patients with an intraosseous chondroid lesion, a second and/or third TB-MRI were available during the period of evaluation (mean interval, 2.7years between the exams). In none of these patients increase of these intraosseous lesions was noticed. Intraosseous chondroid lesions (enchondroma and ACT) appear to occur more frequently in MO patients than in the general population. TB-MRI allows to detect these, besides the identification of OC with suspicious features.

Incidental Finding of an Atypical Cartilaginous Tumor in an Adult Female with Recurrent Patellar Dislocation: Single-stage Extended Curettage using Freezing Nitrogen Ethanol Composite followed by Medial Patello-Femoral Ligament Reconstruction

Incidental Finding of an Atypical Cartilaginous Tumor in an Adult Female with Recurrent Patellar Dislocation: Single-stage Extended Curettage using Freezing Nitrogen Ethanol Composite followed by Medial Patello-Femoral Ligament Reconstruction

Shared decision making: Does a decision aid support patients with an atypical cartilaginous tumor in making a decision about treatment.

Due to new insights, atypical cartilaginous tumors (ACTs) of the long bones are no longer considered malignant and treatment is shifting from surgery to active surveillance. We developed a decision aid in order to support in shared decision making on treatment.The aim of this study is to evaluate the treatment preferences of patients with an ACT in the long bones. During thirty-four months, patients received a decision aid digitally with information about the disease, the treatment options, and the risks and benefits of active surveillance and surgical treatment. The given answers to patients' preference questions were evaluated qualitatively in relation to the final choice of treatment. Eighty-four patients were included. None of the patients who preferred active surveillance later underwent surgery. Only four patients underwent surgery based on patient preference. In our experience the decision aid is useful for shared decision making as it provides the patient with information and the clinician with insight into patient's preferences. The preference for treatment generally corresponds to the eventual treatment. When treatment changes, due to new insights, a decision aid seems helpful for both patients and clinicians to discuss the treatment that best suits the patient's situation.