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Ollier Disease (OD) and Maffucci syndrome (MS) is a rare bone disorder that affects the growth and development of the bones, with an estimated prevalence of 1 in 100,000 people. It is associated with somatic mosaicism of isocitrate dehydrogenase-1 (IDH1) or 2 (IDH2) pathogenic variants. Ivosidenib is indicated for the treatment of acute myeloid leukemia and locally advanced or metastatic cholangiocarcinoma and is currently investigated in low-grade glioma with a susceptible isocitrate dehydrogenase-1 (IDH1) pathogenic variant, but its effects in patients with OD or MS are unknown. We here report the first case of a patient with MS who was treated with Ivosidenib for recurrent IDH-1 mutated glioma. Besides the stabilization of the tumor size, the patient observed significant improvement in his enchondromas that became stiffer, with reduced pain, and significant modification of the mineralization of the enchondromas observed on X-rays. This first case report provides hope for the medical management of patients suffering because of OD or MS. Future clinical research is urgently needed to evaluate long-term benefit risk profile of IDH inhibitors in these rare diseases., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024. Published by Elsevier Inc.)

Abstract: 68 Ga-PSMA (prostate-specific membrane antigen)-HBED-CC (N,N'-bis[2-hydroxy-5-(carboxyethyl)benzyl]ethylenediamine-N,N'-diacetic acid) PET/CT is the new advancement in oncological imaging. However, false-positive uptake can be seen in benign lesions on 68 Ga-PSMA PET/CT. We describe a rare case of intense 68 Ga-PSMA uptake in an enchondroma confirmed on MRI., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Chondromas are benign cartilaginous tumors that frequently occur in the long bones, pelvis, sternum, ribs, and scapula. They seldom develop in the head and neck region, and there have been rare reports of them arising in the nasal septum. Although the mainstay of management is surgery, surgical treatment strategies vary depending on the size, location, and extent of the disease. Herein, we describe a case of huge chondroma originated from the anterior nasal septum, which was completely removed by endoscopic septoplasty approach thorough modified Killian incision., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

It may be difficult to distinguish between enchondroma and low-grade malignant cartilage tumors (grade 1) radiologically. This study aimed to construct machine learning models using 3D computed tomography (CT)-based radiomics analysis to differentiate low-grade chondrosarcoma from enchondroma. A total of 30 patients with enchondroma and 26 with chondrosarcoma were included in this retrospective study. Tumor volume segmentation was manually performed by 2 musculoskeletal radiologists. In total, 107 radiomic features were obtained for each patient. The intraclass correlation coefficient was used to assess interobserver reliability and estimate the absolute agreement between the 2 radiologists. Algorithm-based information gain was used as a feature reduction method, and the 5 most important features were detected. For classification, 7 machine learning models were utilized. Classification was carried out using either all features or 5 features. There was good to excellent agreement between the 2 radiologists for the 107 features of each patient. Therefore, a dataset containing 107 features was used for machine learning classification. When assessed based on area under curve (AUC) values, classification using all features revealed that naive Bayes was the best model (AUC = 0.950), while classification using 5 features revealed that random forest was the best model for differentiating chondrosarcoma from enchondroma (AUC = 0.967). In conclusion, machine learning models using CT-based radiomics analysis can be used to differentiate between low-grade chondrosarcoma and enchondroma., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

Objectives: 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., Methods: MRIs of 123 patients (45 ± 11 years, 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., Results: 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., Conclusion: 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., Clinical Relevance Statement: 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., Key Points: • 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 maximise detection of atypical cartilaginous tumours and high-grade chondrosarcomas, minimise underdiagnosis and reduce potential diagnostic delay in malignant tumours but increase unnecessary referral and follow-up of benign tumours., (© 2024. The Author(s), under exclusive licence to European Society of Radiology.)

Competing Interests: Declaration of competing interest All authors have no conflicts of interest to declare.

Abstract: A 54-year-old man with Gleason 9 prostate cancer with reported nodal and skeletal metastases was referred to us. Outside hospital reports described abnormal left proximal humerus activity on bone scan concerning for metastasis; however, concurrent PSMA PET/CT did not show activity in this lesion. Further review of the PET/CT images revealed characteristic features of enchondroma in the left humeral lesion., Competing Interests: Conflicts of interest and sources of funding: Yang Lu has grant support from Lantheus., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: Enchondromas (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 Methods: The 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., Results: Ninety-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., Conclusion: Frequency of EC/ACT of the shoulder joint appears to have been overestimated, with the current study revealing a prevalence of 0.43%., (© 2023. The Author(s).)

Purpose: This study aimed to introduce a technique of external fixation using a combination of bone cement and K-wires, to treat pathological fractures related to solitary digital enchondroma close to the finger joints., Methods: From October 2015 to January 2021, 21 patients (8 males and 13 females) with acute pathological fracture due to solitary digital enchondroma close to the finger joints were treated with cemented K-wire external fixators. Mean age was 32 (19-51) years. The digits involved were the index (n = 4), long (n = 4), ring (n = 6), and little (n = 7) fingers. Time to bone healing and complications were assessed. At final follow-up, active range of motion, grip strength and key pinch strength of the tumor-involved and contralateral healthy digits were measured and compared. Functional outcomes were evaluated on Takigawa criteria. Pain was measured on a 10-cm visual analog scale. We assessed the affected upper extremity on the Musculoskeletal Tumor Society score questionnaire., Results: Mean bone healing time was 5.5 (4-8) weeks. Pin site infection was observed in 1 patient and cured with dressing changes. Mean follow-up was 34 months, with no recurrences or refractures. Mean active range of motion of the proximal interphalangeal joint, grip and key pinch strength of the involved digits reached 92%, 97%, and 99% of the contralateral digits, respectively. On Takigawa criteria, 20 functional results were excellent and 1 good. Mean pain score was 1 (0-3) cm. Mean Musculoskeletal Tumor Society score was 95 (80-100)., Conclusion: The combination of bone cement and K-wires is a reliable technique for pathological fracture related to solitary enchondroma close to the joints of the digits, leading to good functional outcomes., Level of Evidence: Therapeutic study, Level IVa., (Copyright © 2023. Published by Elsevier Masson SAS.)

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 (ZLNU GLZLM ) and coarseness for neighborhood grey-level difference (Coarseness NGLDM ). Multivariate analysis revealed higher ZLNU GLZLM 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 ZLNU GLZLM 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.

Purpose: To assess the prevalence of intraosseous cartilaginous lesions in patients with multiple osteochondromas based on total-body (TB) MRI examinations, used for screening purposes., Subjects and Methods: 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., Results: 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 61 years (mean, 36). Size of the lesions varied from 4 to 69 mm (mean, 16.3 mm). 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, 2.7 years between the exams). In none of these patients increase of these intraosseous lesions was noticed., Conclusion: 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., (© 2023. The Author(s), under exclusive licence to International Skeletal Society (ISS).)

Soft tissue chondromas are benign tumors, generally of the hands or feet, very rarely of the head and neck region. Repeated microtrauma may be as an initiating factor. The authors describe a case of a soft tissue chondroma of the chin in a 58-year-old male who had used a continuous positive airway pressure face mask for obstructive sleep apnea for 3 years. The patient presented with a hard mass on his chin for 1 year. Computed tomography imaging showed a heterogeneous enhancing mass, with calcification in the subcutaneous layer. Intraoperatively, the mass was beneath the mentalis muscle and abutted the mental nerve, without bone involvement. The diagnosis was a soft tissue chondroma. The patient recovered fully, without recurrence. The primary cause of soft tissue chondromas is unknown. The authors consider that continuous use of a continuous positive airway pressure face mask may play a role in the etiology., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by Mutaz B. Habal, MD.)

Purpose: To construct and compare machine learning models for differentiating chondrosarcoma from enchondroma using radiomic features from T1 and fat suppressed Proton density (PD) magnetic resonance imaging (MRI)., Methods: Eighty-eight patients (57 with enchondroma, 31 with chondrosarcoma) were retrospectively included. Histogram matching and N4ITK MRI bias correction filters were applied. An experienced musculoskeletal radiologist and a senior resident in radiology performed manual segmentation. Voxel sizes were resampled. Laplacian of Gaussian filter and wavelet-based features were used. One thousand eight hundred eighty-eight features were obtained for each patient, with 944 from T1 and 944 from PD images. Sixty-four unstable features were removed. Seven machine learning models were used for classification., Results: Classification with all features showed neural network was the best model for both readers' datasets with area under the curve (AUC), classification accuracy (CA), and F1 score of 0.979, 0.984; 0.920, 0.932; and 0.889, 0.903, respectively. Four features, including one common to both readers, were selected using fast correlation based filter. The best performing models with selected features were gradient boosting for Fatih Erdem's dataset and neural network for Gülen Demirpolat's dataset with AUC, CA, and F1 score of 0.990, 0.979; 0.943, 0.955; 0.921, 0.933, respectively. Neural Network was the second-best model for FE's dataset based on AUC (0.984)., Conclusion: Using pathology as a gold standard, this study defined and compared seven well-performing models to distinguish enchondromas from chondrosarcomas and provided radiomic feature stability and reproducibility among the readers., (© 2023 Wiley Periodicals LLC.)

Background: Enchondromas are the most common primary bone tumors of the hand and are benign cartilaginous tumors. This study evaluated the clinical and radiologic results of patients who underwent surgical interventions for isolated hand enchondromas with a minimum follow-up of 1 year and focused on controversial issues., Methods: A retrospective study included 66 patients with solitary hand enchondromas with follow-up >12 months who underwent surgical interventions between January 2014 and July 2020. Patients with multiple enchondromas, low-grade chondrosarcoma, or who missed follow-up were excluded. All patients underwent thorough curettage with or without filling. Follow-up visits were done at 6 weeks, 3 months, 6 months, and annually. Demographic characteristics, clinical presentation, surgical details, final histologic examination, rehabilitation program, complications, and recurrences were recorded. The range of movement of the affected hand was compared to the contralateral one. Radiological evaluation of defect filling using the modified Neer classification was done., Results: Of the patients 25 were male and 41 were female (38 right hand affection, 28 left hand affection), the mean age was 30.4 ± 11.9 years. Indications for surgery were local pain, swelling, and pathological fractures. The mean follow-up period was 28.8 ± 12.9 months (range 12-67 months). The mean MSTS score was 28.73 ± 1.6 (range 24-30). The middle finger was most frequently affected (18 cases, 27.3%) and the proximal phalanx was the most common location (37 cases, 56.1%). The mean length of lesions was 18.9 ± 6.67 mm and the mean width was 12.3 ± 4.1 mm. According to the modified Neer classification, 50 cases were classified as grade 1 (healed), 16 cases as grade 2 (healed with defects), and no case presented as persistent or recurrent lesions., Conclusion: Solitary hand enchondroma can be effectively treated with adequate curettage, with or without autologous bone grafting, with positive functional and radiological outcomes. When compared to grafting from the iliac crest, harvesting bone from the distal radius provides a shorter hospital stay and reduced complication rates., (© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Background: Enchondromas (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., Methods: Between 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., Results: Of 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%)., Conclusions: This 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., (© 2023. The Author(s).)

Herein, a 30-year-old Caucasian female who presented with a persistent pain in the right pelvic region due to an enchondroma and treated with RF ablation is described. An initial MRI of the pelvis revealed a well-circumscribed lesion in the right inferior ischiopubic ramus with a maximum diameter of 9.5 mm. The final diagnosis was established by a percutaneous CT-guided bone biopsy, which excluded malignancy and revealed an enchondroma. About a month after the biopsy, a percutaneous radiofrequency ablation (RFA) of the lesion was performed. The symptoms resolved completely gradually 2 months after the treatment and a follow-up imaging with MRI showed complete resolution of the pathological enhancement indicating necrosis of the lesion. RFA has not been previously reported as a treatment option of enchondromas found in flat bones of the pelvis and could be a safe alternative minimally invasive treatment option in such cases, avoiding major operations., (© 2023. The Author(s), under exclusive licence to International Skeletal Society (ISS).)

Objective: To assess the diagnostic performance of MRI-based texture analysis for differentiating enchondromas and chondrosarcomas, especially on fat-suppressed proton density (FS-PD) images., Materials and Methods: The whole tumor volumes of 23 chondrosarcomas and 24 enchondromas were manually segmented on both FS-PD and T1-weighted images. A total of 861 radiomic features were extracted. SelectKBest was used to select the features. The data were randomly split into training (n = 36) and test (n = 10) for T1-weighted and training (n = 37) and test (n = 10) for FS-PD datasets. Fivefold cross-validation was performed. Fifteen machine learning models were created using the training set. The best models for T1-weighted, FS-PD, and T1-weighted + FS-PD images were selected in terms of accuracy and area under the curve (AUC)., Results: There were 7 men and 16 women in the chondrosarcoma group (mean ± standard deviation age, 45.65 ± 11.24) and 7 men and 17 women in the enchondroma group (mean ± standard deviation age, 46.17 ± 11.79). Naive Bayes was the best model for accuracy and AUC for T1-weighted images (AUC = 0.76, accuracy = 80%, recall = 80%, precision = 80%, F1 score = 80%). The best model for FS-PD images was the K neighbors classifier for accuracy and AUC (AUC = 1.00, accuracy = 80%, recall = 80%, precision = 100%, F1 score = 89%). The best model for T1-weighted + FS-PD images was logistic regression for accuracy and AUC (AUC = 0.84, accuracy = 80%, recall = 60%, precision = 100%, F1 score = 75%)., Conclusion: MRI-based machine learning models have promising results in the discrimination of enchondroma and chondrosarcoma based on radiomic features obtained from both FS-PD and T1-weighted images., (© 2022. The Author(s), under exclusive licence to International Skeletal Society (ISS).)

Background: This study aims to look at the intermediate-term clinical, functional and radiological outcomes of patients with enchondroma in hand treated with osteoscopic-assisted curettage and artificial bone substitute or bone graft. The addition of osteoscopy allows direct visualisation of the bone cavity during and after curettage of tumour tissue without the need of creating a large opening in the bone cortex. This could lead to better clearance of tumour tissue and lower risk of iatrogenic fracture. Methods: A total of 11 patients who received surgery from December 2013 to November 2020 were retrospectively reviewed. All cases had histological diagnosis of enchondroma. Patients with a follow-up period of less than 3 months were excluded. The mean duration of follow-up was 20.9 months. For the clinical outcome, we measured the total active motion (TAM) and graded with Belsky score grip strength. For the functional outcome, the Quick Disabilities of the Arm, Shoulder and Hand Questionnaire (QuickDASH) score was used. For the radiological outcome, we evaluated the X-ray for bone cavity filling defect, new bone formation according to the system proposed by Tordai. Results: The mean TAM of patients was 257º. A total of 60% patients had Belsky score grading excellent, 40% patients had Belsky score grading good. The mean percentage of grip strength compared with the contralateral side was 86.2%. The mean QuickDASH score was 7.7. For the wound aesthetic rating by patients, 81.8% patients reported as excellent. For the radiological outcome, the postoperative X-ray of all patients showed bone filling defect less than 3 mm. The mean time to complete bone consolidation was 3.8 months. None of the patients showed any radiological signs of recurrence. Conclusions: Our study showed that patients with enchondromas in hand treated with this minimally invasive method demonstrated good functional and radiological outcome. Its application may also be extended into treating other benign bone lesions in hand. Level of Evidence: Level IV (Therapeutic).

We present a case of solitary chondrosarcoma arising from the proximal phalanx of the ring finger in an elderly man. The chondrosarcoma developed over a period of 14 years, during which the phalanx became progressively more deformed. Several radiographic investigations were carried out, but the patient declined further suggested diagnostic examinations (computed tomography, magnetic resonance imaging, biopsy). Eventually, the lesion became significantly enlarged, and radiographs showed osteolytic lesions in the phalangeal bone. Ray amputation of the finger was required to establish a wide resection of the chondrosarcoma. Most osteochondral tumors arising from the phalanges are benign tumors such as enchondromas, but primary chondrogenic malignant bone tumors (chondrosarcomas) occasionally occur. Chondrosarcoma of the phalanx is difficult to distinguish from enchondroma of the phalanx, because histological investigations of the two neoplasms often produce similar findings. Even with a combination of clinical, biopsy, and imaging findings, differentiating these neoplasms is still challenging, because the characteristic clinical and radiological features of chondrosarcoma do not appear until it becomes aggressive and starts to cause destructive changes. Once that happens, radical expanded resection of the tumor is essential. Therefore, longstanding enchondroma-like lesions should be actively treated in elderly patients, even if a definite diagnosis of chondrosarcoma cannot be made.

Extraskeletal chondroma comprises synovial chondromatosis, intracapsular chondroma and soft tissue chondroma, its presentation in the hand, however, is very rare. A 42-year-old woman presented with a mass around right fourth metacarpophalangeal (MCP) joint. She had no pain or discomfort in activities. The radiographs showed soft tissue swelling, but no calcification or ossifying lesions. The magnetic resonance imaging (MRI) showed a lobulated juxta-cortical encircling mass existing around the fourth MCP joint. We did not suspect any cartilage-forming tumour in MRI. The mass was easily removed because there was no adhesion with surrounding tissues and the specimen had the appearance of a cartilage. The histological diagnosis was chondroma. Based on the tumour location and histological results, we diagnosed it as intracapsular chondroma. Although intracapsular chondroma is very rare-ly seen in the hands, it is important to consider an intracapsular chondroma when differentiating a tumour in the hand because it is difficult to diagnose one in an imaging examination. Level of Evidence: Level V (Therapeutic).

Backgrounds: We aimed to compare the average 10-year results of patients treated with three different surgical methods, that is, extended curettage, extended curettage with grafting and extended curettage with cementation for the management of solitary enchondromas., Methods: Two hundreds and three patients with a solitary enchondroma who underwent surgery at our department between 1990 and 2015 were evaluated retrospectively. Only extended curettage was performed in Group A (n: 75), extended curettage with grafting with autologous bone graft from the iliac crest in Group B (n: 58), and extended curettage with bone cement in Group C (n: 70). Surgical complications and recurrence were analysed and compared between these groups., Results: No significant differences were noted among the groups in terms of gender (P = 0.654), age (P = 0.264) and follow-up duration (P = 0.919). The mean follow-up period in all groups was 122.9 ± 39 (range: 60-271) months. In addition to curettage and cementation, three patients received mechanical support with plate screws because of fracture risk. One patient in the extended curettage with grafting group had surgical site infection that was treated with simple debridement and antibiotherapy. One patient in the extended curettage group had lost motions of the proximal interphalangeal joint, which was treated with aggressive physiotherapy., Conclusion: We compared the average 10-year results of patients treated with three surgical options, namely, extended curettage, extended curettage with grafting, and extended curettage with cementation and no difference was found among the groups in local control of solitary enchondromas., (© 2022 Royal Australasian College of Surgeons.)

Introduction: Extraskeletal soft tissue chondroma (STC) is a rare benign tumor. Soft-tissue chondromas rarely occur in the oral cavity. In this study, we aimed to confirm a slow-growing tongue mass using magnetic resonance imaging., Patient Concerns: A 60-year-old woman presented with a painful, slow-growing tongue mass that had persisted for 17 years. Intraoral examination revealed a pedunculated mass covered with mucosa on the right side of her tongue., Diagnosis: CT and MRI revealed a lobulated heterogeneously enhancing mass without calcification. Compared with previous images obtained 17 years prior, the mass presented slow growth, more prominent enhancement, and lobulated contour. Histopathological examination confirmed the presence of STC., Interventions: Excision of the mass surrounding normal tissue was performed under general anesthesia., Outcomes: During 1-year follow-up period, no recurrence was observed., Conclusions: In this study, STC lesions were slow-growing, and changed from weakly homogeneous enhancement and clean margins to markedly heterogeneous enhancement and lobulated margins over time., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background: Diagnostic performance, inter-observer agreement, and intermodality agreement between computed tomography (CT) and magnetic resonance imaging (MRI) in the depiction of the major distinguishing imaging features of central cartilaginous tumors have not been investigated., Purpose: To determine the inter-observer and intermodality agreement of CT and MRI in the evaluation of central cartilaginous tumors of the appendicular bones, and to compare their diagnostic performance., Material and Methods: Two independent radiologists retrospectively reviewed preoperative CT and MRI. Inter-observer and intermodality agreement between CT and MRI in the assessment of distinguishing imaging features, including lesion size, deep endosteal scalloping, cortical expansion, cortical disruption, pathologic fracture, soft tissue extension, and peritumoral edema, were evaluated. The agreement with histopathology and the accuracy of the radiologic diagnoses made with CT and MRI were also analyzed., Results: A total of 72 patients were included. CT and MRI showed high inter-observer and intermodality agreements with regard to size, deep endosteal scalloping, cortical expansion, cortical disruption, and soft tissue extension (ICC = 0.96-0.99, k = 0.60-0.90). However, for the evaluation of pathologic fracture, MRI showed only moderate inter-observer agreement (k = 0.47). Peritumoral edema showed only fair intermodality agreement (k = 0.28-0.33) and moderate inter-observer agreement (k = 0.46) on CT. Both CT and MRI showed excellent diagnostic performance, with high agreement with the histopathology (k = 0.89 and 0.87, respectively) and high accuracy (91.7% for both CT and MRI)., Conclusion: CT and MRI showed high inter-observer and intermodality agreement in the assessment of several distinguishing imaging features of central cartilaginous tumors of the appendicular bones and demonstrated comparable diagnostic performance.

Background: Carney's triad is a rare syndrome comprising gastrointestinal stromal tumor, extra-adrenal paraganglioma, and pulmonary chondroma along with newer additions of adrenal adenoma and esophageal leiomyoma. The triad is completely manifest in only 25-30% cases, with most patients presenting with two out of three parts of the syndrome. Wild-type succinate-dehydrogenase-deficient gastric gastrointestinal stromal tumor forms the most common component of Carney's triad and is usually multicentric and multifocal. It usually demonstrates indolent behavior and resistance to imatinib; hence, the management remains predominantly surgical. Pulmonary chondromas are commonly unilateral and multiple with slow-growing nature, which allows for conservative management. Adrenocortical adenomas are found in 20% of patients and are usually detected as incidentalomas., Case Presentation: A 49-year-old Asian male presented with upper gastrointestinal bleed and was diagnosed with multiple gastric succinate-dehydrogenase-deficient gastrointestinal stromal tumors. On evaluation, he was found to have left pulmonary chondroma and non-secretory adrenal adenoma, thus completing the Carney's triad. He underwent surgery with sleeve gastrectomy and excision of the antral tumor nodule, while the adrenal and pulmonary tumors have been under close follow-up., Conclusion: Literature regarding Carney's triad is scarce, especially from the Indian setting. Our report aims to highlight the various manifestations of this syndrome with emphasis on management of wild-type succinate-dehydrogenase-deficient gastrointestinal stromal tumor. Radical gastric surgeries do not offer a survival advantage in this condition; hence, more conservative modalities of resection can be adopted., (© 2021. The Author(s).)

A chondroma is a common benign cartilaginous tumor. However, a primary soft tissue chondroma of the posterior mediastinum is very rare. We herein report a case involving a 51-year-old man with a posterior mediastinal mass. The mass was dissected by thoracoscopy through the eighth intercostal space. Pathological examination led to a definitive diagnosis of a primary mediastinal chondroma with no criteria of malignancy. Preoperative diagnosis of a posterior mediastinal soft tissue chondroma is not easy because of its rarity and lack of typical features other than calcification. When a posterior mediastinal well-circumscribed soft tissue mass contains calcification and shows no obvious enhancement, the possibility of a soft tissue chondroma should be considered.

We demonstrated the feasibility of 7 Tesla sodium ( 23 Na) and chlorine ( 35 Cl) MRI of a solitary enchondroma. For this, we established dedicated sequences on a 7-Tesla whole-body system with the following key parameters for 35 Cl MRI: TE/TR = 0.35/60 ms, T RO  = 5 ms, α = 90°, Δx 3  = (6 mm) 3 , 3 averages, T acq  = 30 min and for 23 Na MRI: TE/TR = 0.4/101 ms, T RO  = 10ms; α = 90°; Δx 3  = (1.9 mm) 3 , 3 averages, T acq  = 30 min 18 s. The measured apparent Na + concentration was 255 mmol/l and was approximately 7-fold higher than the apparent Cl - concentration with about 36 mmol/l. Additionally, repeated proton MRI examinations demonstrated constant but subtle growth (≈ 0.65 ml/year) over 14 years. In conclusion, enchondromas obviously have a high contrast-to-noise ratio when compared with the normal bone marrow in 23 Na and 35 Cl MRI, which may contribute to detection and differentiation in unclear or subtle cases. KEY POINTS:: · High magnetic field strengths (e. g., 7 Tesla) enable sodium (23Na) and chlorine (35Cl) MRI of solitary cartilage-forming tumors like enchondromas with nominal spatial resolutions of (1.9 mm)3 (23Na MRI) and (6 mm)3 (35Cl MRI).. · Measured median tumoral apparent Na+ and Cl- concentrations were nearly 13 times higher and 3 times higher than in normal muscle tissue, respectively.. · Enchondromas have a high contrast-to-noise-ratio when compared with the normal bone marrow in 23Na and 35Cl MRI, which may contribute to detection and differentiation in unclear or subtle cases.. CITATION FORMAT: · Weber M, Seyler L, Nagel AM. 7 Tesla Chlorine (35Cl) and Sodium (23Na) MR Imaging of an Enchondroma. Fortschr Röntgenstr 2021; 193: 1207 - 1211., Competing Interests: The authors declare that they have no conflict of interest., (Thieme. All rights reserved.)

Purpose: The aims of this study were to evaluate the degree of cortical thinning and the tumor area in simple radiographs in patients with enchondroma of the hand combined with pathologic fracture and to determine the surgical outcome of simultaneous tumor curettage and internal fixation of fracture., Methods: We enrolled 23 bone lesions of 21 patients diagnosed with enchondroma of the hand. Of them, 9 bone lesions of 8 patients had pathologic fracture. We evaluated the degree of cortical thinning and tumor area in simple radiographs and compared them between patients with and without pathologic fracture. For patients with pathologic fracture, we performed curettage of the tumor through the fracture site and fixed the fracture using K-wires. We then evaluated the clinical outcome of these patients using the visual analog scale to measure pain intensity and Takigawa's criteria 1 year after surgery., Results: There was a significant difference in the degree of cortical thinning between the 2 groups (P < 0.001); however, there was no significant difference in the percentage of tumor area (P = 0.259). The average time from surgery to bony union was 8.5 weeks in patients with pathologic fracture. The patients reported that they could return to their previous activities of daily living within 12 weeks of surgery. The average visual analog scale score was 0.6 at 1 year after surgery, and 6 patients were graded as excellent and 2 as good according to Takigawa's criteria., Conclusions: Cortical thinning seems to be related to pathologic fracture. Our scoring system for cortical thinning may be helpful in evaluating the risk for pathologic fracture. Early 1-stage surgical treatment for pathologic hand fracture with enchondroma is a very useful method with satisfactory outcomes that could shorten the treatment period., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Tumour-to-tumour metastasis is very unusual and has been defined as a tumour metastasis into another histologically different tumour. It is extremely rare in bone. We report a case of lung squamous cell carcinoma metastasized to an enchondroma in the femur of a patient with Ollier disease. A 60-year-old female had a history of a poorly differentiated squamous cell carcinoma of the lung. She underwent a video-assisted thoracoscopic lobectomy, and a follow-up MRI scan showed three lesions in the left distal femur and proximal tibia, which were initially interpreted as metastasis on radiology. Resection of the left proximal tibial lesion was performed, and the pathological findings were consistent with enchondroma with no evidence of metastasis. Subsequent curettage of lesions in the distal left femur revealed metastatic poorly differentiated carcinoma with foci of hyaline cartilage, which was most consistent with metastatic carcinoma in a pre-existing enchondroma. The MRI films were re-reviewed. Characteristic MRI features of enchondroma were found in the lesion in the left proximal tibia and one of the lesions in the left distal femur, while the features of the other lesion in the left distal femur included cortical destruction and extensive oedema in surrounding soft tissue, which were consistent with a malignant tumour. In addition, the enchondroma in the lateral condyle showed blurring and irregular inner margin and adjacent bone oedema, which likely represents a co-existing metastatic tumour and enchondroma. The difference in lineage was confirmed by immunohistochemistry. The final diagnosis was metastatic poorly differentiated carcinoma of the lung into a co-existent enchondroma. The diagnosis can be challenging and could be easily overlooked both radiologically and histologically. Thorough clinical and radiological information is critical for the diagnosis, and despite a very unusual event, awareness of the tumour-to-tumour metastasis phenomenon can avoid an inaccurate diagnosis by the pathologist, therefore preventing inappropriate clinical intervention., (© 2020. The Author(s).)

Objective: To determine the rate of chondrosarcoma in incidentally discovered painless long bone cartilage lesions and to determine if any further imaging is needed., Materials and Methods: A cartilage lesion was said to be an enchondroma when it had characteristic matrix mineralization and no aggressive features. Search of all imaging reports and tumor board files for keywords enchondroma, cartilage lesion, chondroid, and chondrosarcoma. Retrospective review of medical records and imaging studies from 4.5-year period. Data points collected included patient age, sex, lesion site, size, symptoms, type of imaging, imaging appearance, and length of follow-up. Only patients with no pain were included as enchondroma. Patients with final diagnosis of chondrosarcoma were included for comparison of all features., Results: Only 1/73 (1.4%) patients with an initial incidentally discovered painless lesion was later diagnosed, with new symptoms, as atypical cartilage tumor. Average age was 59.4 years. Bones involved were the femur (n = 33), humerus (n = 30), tibia (n = 7), fibula (n = 2), and ulna (n = 1). Average enchondroma size was 3.9 cm (range 1.4-11.5). Average follow-up was 47 months (range 2-196 months). Eleven long bone chondrosarcomas were identified. All chondrosarcoma patients had pain and aggressive imaging findings., Conclusion: Our study reveals that the rate of chondrosarcoma in incidentally found painless chondroid lesions without aggressive features in long bones is low. Imaging follow-up may be needed only in the setting of new symptoms.

Background: We compared the curettage/bone grafting and the curettage/bone graft substitutes surgical techniques in their relation to functional outcomes, oncologic outcome (recurrence, malignant transformation), the rate of postsurgical complications, durations of surgery and of postsurgical immobilization for hand-localized cases of solitary and multiple enchondromas., Methods: The current prospective randomized trial analyzed 200 adult patients (2012-2017) with enchondroma who underwent surgical intervention. The cases were randomly divided into Group 1 (n = 100; F 56, M 44) for surgeries with curettage and autogenous bone grafting, and Group 2 (n = 100; F 55, M 45) for surgeries with curettage and bone graft substitutes. The placebo control Group 3 consisted of cases operated by curettage only (n = 56; F 31, M 25). The follow-up period was set at 30 months., Results: The duration of surgery was 51 ± 4 min in Group 1 and 27 ± 1 min in Group 2 (p = 0.008). In Group 1, the rate of recurrence was 6% against 1% in Group 2 (p = 0.005). No other statistically significant differences in postsurgical outcomes between three involved groups were noted., Conclusion: In cases of enchondroma of the hand, postsurgical functional outcomes, the rate of postsurgical complications, the duration of immobilization, and the time to complete recovery are not influenced by the type of chosen grafting material. The implementation of HAp-collagen bone substitutes in granules instead of autogenous bone grafting reduces the duration of surgery. The implementation of autogenous bone grafting may increase the rate of tumor recurrence., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

»: Enchondromas are benign cartilaginous lesions that rarely require surgical intervention., »: Atypical cartilaginous tumors (ACTs), also referred to as grade-1 chondrosarcomas, may be managed without any intervention or with extended intralesional curettage and bone-void filling., »: High-grade chondrosarcomas, or grade-2 and 3 chondrosarcomas, should be managed aggressively with wide resection., »: Chemotherapy and radiation do not currently play a role in the treatment of chondrosarcomas., »: Differentiating an enchondroma from an ACT and an ACT from a high-grade chondrosarcoma can be difficult and requires clinical experience, radiographic and advanced imaging, and possibly a biopsy. Ultimately, a multidisciplinary team that includes a musculoskeletal oncologist, a radiologist, and a pathologist is needed to make the most appropriate diagnosis and treatment plan for each patient., Competing Interests: Disclosure: The authors indicated that no external funding was received for any aspect of this work. The Disclosure of Potential Conflicts of Interest forms are provided with the online version of the article (http://links.lww.com/JBJSREV/A691)., (Copyright © 2021 Written work prepared by employees of the Federal Government as part of their official duties is, under the United States Copyright Act, a ‘work of the United States Government’ for which copyright protection under that Act is not available. As such, copyright protection does not extend to the contributions of employees of the Federal Government prepared as part of their employment.)

Intracranial intradural chondroma is a rare disorder,the imaging findings of which have been rarely reported.The current study reported a case of intracranial extra-cerebral chondroma and described the detailed CT and magnetic resonance imaging findings,which would provide valuable imaging evidence for the diagnosis of intracranial extra-cerebral chondroma.

Recent scholarship on enchondroma, chondrosarcoma, and chondroblastoma is presented. The focus of this article is on the imaging appearance of these tumors and the means by which they can be distinguished from one another by both clinical and imaging criteria., (Copyright © 2020 Elsevier Inc. All rights reserved.)

"Acral FibroChondroMyxoid tumor" (AFCMT) is a recently described distinctive subtype of acral soft tissue tumor that typically arises on the fingers and toes. We herein present the unreported imaging features of AFCMT in a 44-year-old woman. This otherwise healthy patient was referred for a painful, slow-growing, soft tissue mass in the middle finger of her right hand. Initial radiographs and computed tomography showed a small lesion centered in the soft tissue of the ulnar aspect of the proximal phalanx, associated with scalloping of the underlying bone. Magnetic resonance imaging confirmed the presence of a well-circumscribed soft tissue tumor that exhibited relatively high T2-weighted signal intensity and marked enhancement after contrast administration. Subsequent excisional biopsy was performed. Histologically, the tumor was characterized by an abundant stroma displaying fibrous, chondroid, and myxoid areas. By immunohistochemistry, tumor cells stained for CD34, ERG, and focally S100 protein. RNA-sequencing allowed detection of THBS1-ADGFR5 gene fusion which confirmed the diagnosis of AFCMT. At 2-year follow-up, the patient remains free of recurrence. AFCMT is a previously unrecognized entity that may mimic chondroma and should be considered in the differential diagnosis of soft tissue tumors with cartilaginous or myxoid stroma in the extremities.

Background:  Chondrogenic tumors are the most frequent primary bone tumors. Malignant chondrogenic tumors represent about one quarter of malignant bone tumors. Benign chondrogenic bone tumors are frequent incidental findings at imaging. Radiological parameters may be helpful for identification, characterization, and differential diagnosis., Methods:  Systematic PubMed literature research. Identification and review of studies analyzing and describing imaging characteristics of chondrogenic bone tumors., Results and Conclusions:  The 2020 World Health Organization (WHO) classification system differentiates between benign, intermediate (locally aggressive or rarely metastasizing), and malignant chondrogenic tumors. On imaging, typical findings of differentiated chondrogenic tumors are lobulated patterns with a high signal on T2-weighted magnetic resonance imaging (MRI) and ring- and arc-like calcifications on conventional radiography and computed tomography (CT). Depending on the entity, the prevalence of this chondrogenic pattern differs. While high grade tumors may be identified due to aggressive imaging patterns, the differentiation between benign and intermediate grade chondrogenic tumors is challenging, even in an interdisciplinary approach., Key Points:   · The WHO defines benign, intermediate, and malignant chondrogenic bone tumors. · Frequent benign tumors: osteochondroma and enchondroma; Frequent malignant tumor: conventional chondrosarcoma. · Differentiation between enchondroma versus low-grade chondrosarcoma is challenging for radiologists and pathologists. · Pain, deep scalloping, cortical destruction, bone expansion, soft tissue component: favor chondrosarcoma. · Potential malignant transformation of osteochondroma: progression after skeletal maturity, cartilage cap thickness (> 2 cm adult; > 3 cm child). · Potentially helpful advanced imaging methods: Dynamic MRI, texture analysis, FDG-PET/CT., Citation Format: · Engel H, Herget GW, Füllgraf H et al. Chondrogenic Bone Tumors: The Importance of Imaging Characteristics. Fortschr Röntgenstr 2021; 193: 262 - 274., Competing Interests: The authors declare that they have no conflict of interest., (Thieme. All rights reserved.)

Chondroma of the dural convexity (CDC) is a benign and extremely rare type of intracranial chondroma. In this study, we reported five CDCs in a single center and reviewed the available literature to determine the clinical characteristics and surgical outcomes and possible origins of the disease. The clinical data of five patients (4 females) who confirmed to be CDC between 2000 and 2019 in our single center was collected together with 22 cases from literatures. The clinical characteristics and surgical outcomes were reviewed and analyzed. Among all the available CDC cases, the mean age was 31 ± 13.7 years; the mean tumor volume was 42.3 ± 40.9 cm 3 , showing a female predominance (63% vs. 37%). The tumors showed calcification in 88.2% cases (15/17) on CT scans and hypointense on T1WI (15/19, 78.9%), mixed intense on T2WI (10/18, 55.6%), and inhomogeneous enhancement without dural tail sign after administration of gadolinium (20/21, 95.2%). Almost all the tumors were misdiagnosed as meningiomas preoperatively. In addition, almost all image available CDC lesions (24/25, 96%) located across the cranial sutures indicating that the tumor originated from ectopic chondrocytes from adjacent skull sutures. No tumors recurred after total resection in follow-up. CDCs are characterized with female predominance and may originate from ectopic chondrocytes from adjacent skull sutures. The lesion with inhomogeneous contrast enhancement without dural tail sign and avascular in cerebral angiography are key points to be differentiated from meningioma. The most effective treatment is total resection., Competing Interests: No potential conflicts of interest were disclosed., (Copyright © 2020 Jing Guo et al.)

Benign and malign tumors can affect the temporomandibular joint (TMJ) as any other articulation. Nevertheless, TMJ tumors are rare and mostly benign. Their clinical expression is varied including symptomatology similar to TMJ dysfunctional disorders, otologic or neurologic pathologies. In some cases, they remain totally asymptomatic. Hence, diagnosis is difficult since the symptomatology can be misleading with TMJ dysfunctional disorders or otologic disorders wrongly diagnosed. There is thus frequently a long delay between symptoms onset and diagnosis. The great variety of TMJ lesions explains the wide range of possible treatment modalities, mostly based on surgery. We provide here a review of the lesions originating from the TMJ. Tumoral or cystic mandibular lesion affecting the TMJ through local extension will not be discussed. Osteoma, osteoid osteoma, osteoblastoma, chondroma, osteochondroma, chondroblastoma, tenosynovial giant cell tumors, giant cell lesions, non-ossifying fibroma, hemangioma, lipoma or Langerhans cell histiocytosis are all possible diagnosis among the benign tumors found in the TMJ. Pseudotumors include synovial chondromatosis and aneurysmal bone cyst. Finally, malign tumors of the TMJ include mainly sarcomas (osteosarcoma, chondrosarcoma, synovial sarcoma, Ewing sarcoma, and fibrosarcoma), but also multiple myeloma and secondary metastases. We will review the clinical, radiological and histological aspects of each of these lesions. The treatment and the recurrence risk will also be discussed., (Copyright © 2020 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

Enchondromas (EC) are frequent incidental findings on magnetic resonance imaging (MRI) performed for the diagnosis of joint pathology, especially observed on MRI examinations of the knee and shoulder. Enchondroma has potential for malignant transformation to chondrosarcoma (CS), and it may be difficult to distinguish EC from low-malignant CS on the basis of imaging or histopathology studies. Therefore, EC is mostly followed up to monitor any growth and/or changes indicating aggressive tumor.There is no consensual evidence on when and how to follow up patients with EC with regard to potential malignant transformation. Therefore, the Danish Multidisciplinary Cancer Groups initiated and supported the elaboration of Danish guidelines in 2020 based on a literature review. The guidelines are presented here, in addition to a summary of the background literature.