Radiofrequency ablation of atypical cartilaginous tumors in long bones: a retrospective study.

Purpose: To determine the size of the ablation zone after radiofrequency ablation (RFA) of atypical cartilaginous bone tumors (ACT) using temperature-controlled 20 and 30 mm RFA straight non-cooled electrodes.Materials and methods: Sixteen patients with ACT in their long bones, who had undergone a single-session single-application CT-guided temperature-controlled RFA, were included retrospectively in the study. Tumors with a diameter of 10-25 mm were treated with 20 mm electrodes (n = 10), and tumors of 25-35 mm, with 30 mm electrodes (n = 6). The ablated zone was measured after three months on MRI images.Results: All the tumors were within the ablated zone on the 3-month follow-up MRI scan. The mean ablation time with the electrode, at a target temperature of 90 °C, was 7.6 minutes (range 6-10). The median of the largest ablation diameters, on applying the 20 and 30 mm electrodes, were 42 mm (IQR 8.5, range 30-51 mm) and 44.5 mm (IQR 4.5, range 42-63 mm), respectively.Conclusions: All the retrospectively viewed tumors in the long bones of ACT patients treated with RFA were completely ablated. The ablation zone diameters in the bones were larger than expected, when compared to other tissues, such as the liver.

Chondroblastoma of the Foot: 40 Cases From a Single Institution.

Chondroblastoma (CB) of the foot is a rare lesion. The purpose of this report was to report a large series from a single institution to analyze clinical, radiographic, and histologic characteristics; treatment; and local recurrence. We present 40 patients (30 males, 10 females; mean age 25 years) diagnosed and treated for CB of the foot from 1975 to 2012. The mean follow-up visit was 55 months (range 7 months to 11 years). Clinical presentation, histology, imaging, surgical treatment, and local recurrence were evaluated. Males were more affected than females. The main symptom was pain (100%) accompanied by swelling (35%), with median duration of 12 months. The talus (50%) and calcaneus (37.5%) were the most affected bones. All patients underwent surgery: curettage (10 cases), curettage and bone graft (15 cases), curettage and cement (13 cases), wide resection (1 case), and Chopart amputation (1 case). Ten patients (25%) had secondary aneurysmal bone cyst. One patient had local recurrence after surgery. In conclusion, patients with CB of the foot are usually older than 20 years, and males are most affected. The hindfoot is the most affected area. Surgical treatment is required, and intralesional curettage and packing with cement or graft is curative in most cases. Local recurrence in foot is lower than in other locations.

Solitary osteochondromas in paleo-oncology: A case report from 4th-century BCE Pontecagnano (southern Italy).

OBJECTIVE: This study presents a new case of solitary osteochondroma (SOC) identified on the proximal tibia of a 4th-century BCE individual from Pontecagnano (Salerno, Italy) with an aim to contribute to differential diagnosis of bone tumors in archeological contexts. MATERIALS: Paleopathological assessment of a male individual with an estimated age-at death of 45.9-62.9 years brought to light during the archaeological excavations in the funerary sector of 'Sica de Concillis' within the necropolis of Pontecagnano. METHODS: Macroscopic and radiographic analyses were performed for diagnostic purposes. RESULTS: The proximal portion of the right tibia exhibited a large exophytic bone formation extending from the anteromedial to the posteromedial portion of the diaphysis. The x-ray confirmed the lesion being characterized by regular trabecular bone tissue with cortico-medullary continuity. CONCLUSIONS: The lesion observed is diagnostic of sessile SOC, a neoplasm that must have had aesthetic and, possibly, neurovascular complications due to its large size. SIGNIFICANCE: By offering a detailed description of a case of tibial osteochondroma and discussing the possible complications the individual might have experienced during life, this study highlights the role and importance of benign bone tumors in paleo-oncology. LIMITATIONS: Histological analysis was not carried out in order to preserve the integrity of the affected tibia. SUGGESTIONS FOR FURTHER RESEARCH: More attention should be devoted to benign tumors in paleopathology as their occurrence and manifestations in the past will lead to a better understanding of their impacts on the quality of life of affected individuals and to greater knowledge of their natural history.

Fibrocartilaginous Dysplasia - A Report of Five Cases with Review of Literature.

Introduction: Fibrocartilaginous dysplasia (FCD) is a variant of fibrous dysplasia (FD) with extensive cartilaginous differentiation. This has been reported in both monostotic and polyostotic types of FD, the proximal femur being the most common site involved. Case Report: We report five cases of fibrocartilaginous dysplasia with varying degrees of cartilaginous differentiation. The age of the patients ranged from 7 to 30 years, and there was a female predominance (M:F ratio of 1:4). The proximal femur was the site of involvement in all the cases. Imaging showed well demarcated radiolucent lesions with stippled calcifications. Histologically, cartilaginous areas were noted juxtaposed to typical areas of fibrous dysplasia. Four of the patients were treated with curettage and one with a marginal resection. None of the five cases had recurrences at the past follow-up. Conclusion: FCD is a rare variant of fibrous dysplasia which needs to diagnosed and treated early, as there is a high risk of pathological fracture.

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

Objective: 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. Methods: 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. Results: 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. Conclusion: 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. Innovation: 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.

Diagnostic Value of CT- and MRI-Based Texture Analysis and Imaging Findings for Grading Cartilaginous Tumors in Long Bones.

OBJECTIVE: To confirm the diagnostic performance of computed tomography (CT)-based texture analysis (CTTA) and magnetic resonance imaging (MRI)-based texture analysis for grading cartilaginous tumors in long bones and to compare these findings to radiological features. MATERIALS AND METHODS: Twenty-nine patients with enchondromas, 20 with low-grade chondrosarcomas and 16 with high-grade chondrosarcomas were included retrospectively. Clinical and radiological information and 9 histogram features extracted from CT, T1WI, and T2WI were evaluated. Binary logistic regression analysis was performed to determine predictive factors for grading cartilaginous tumors and to establish diagnostic models. Another 26 patients were included to validate each model. Receiver operating characteristic (ROC) curves were generated, and accuracy rate, sensitivity, specificity and positive/negative predictive values (PPV/NPV) were calculated. RESULTS: On imaging, endosteal scalloping, cortical destruction and calcification shape were predictive for grading cartilaginous tumors. For texture analysis, variance, mean, perc.01%, perc.10%, perc.99% and kurtosis were extracted after multivariate analysis. To differentiate benign cartilaginous tumors from low-grade chondrosarcomas, the imaging features model reached the highest accuracy rate (83.7%) and AUC (0.841), with a sensitivity of 75% and specificity of 93.1%. The CTTA feature model best distinguished low-grade and high-grade chondrosarcomas, with accuracies of 71.9%, and 80% in the training and validation groups, respectively; T1-TA and T2-TA could not distinguish them well. We found that the imaging feature model best differentiated benign and malignant cartilaginous tumors, with an accuracy rate of 89.2%, followed by the T1-TA feature model (80.4%). CONCLUSIONS: The imaging feature model and CTTA- or MRI-based texture analysis have the potential to differentiate cartilaginous tumors in long bones by grade. MRI-based texture analysis failed to grade chondrosarcomas.

Imaging features of cartilaginous tumors of the head and neck.

There is a wide spectrum of head and neck cartilaginous lesions which include both neoplastic and nonneoplastic processes. Cartilaginous tumors of the head and neck are uncommon, posing a diagnostic challenge. Benign cartilaginous tumors that may occur in the head and neck include chondroma, chondroblastoma, chondromyxoid fibroma, osteochondroma, and synovial chondromatosis. Chondromesenchymal hamartoma is a rare non-neoplastic cartilaginous lesion that is included for the 1first time in the new WHO classification and radiologically can mimic a tumor. Malignant cartilaginous tumors include chondrosarcoma and chondroid variant of chordoma. Characteristic tumor locations, internal chondroid matrix calcification, and typical T2 hyperintense signal secondary to high-water content within the extracellular matrix of the hyaline cartilage are useful imaging features that narrow the differential diagnosis and help in diagnosing these diseases. This article presents a narrative review of the anatomy of the head and neck cartilaginous structures, discusses the current knowledge and imaging spectrum of benign and malignant cartilaginous tumors and tumor-like lesions of the head and neck.

A Radiological Scoring System for Differentiation between Enchondroma and Chondrosarcoma.

BACKGROUND: It is challenging to differentiate between enchondromas and atypical cartilaginous tumors (ACTs)/chondrosarcomas. In this study, correlations between radiological findings and final diagnosis were investigated in patients with central cartilaginous tumors. METHODS: To evaluate the diagnostic usefulness of radiological findings, correlations between various radiological findings and final diagnoses were investigated in a cohort of 81 patients. Furthermore, a new radiological scoring system was developed by combining radiological findings. RESULTS: Periosteal reaction on X-ray (p = 0.025), endosteal scalloping (p = 0.010) and cortical defect (p = 0.002) on CT, extraskeletal mass (p < 0.001), multilobular lesion (p < 0.001), abnormal signal in adjacent tissue (p = 0.004) on MRI, and increased uptake in bone scan (p = 0.002) and thallium scan (p = 0.027) was significantly correlated with final diagnoses. Based on the correlations between each radiological finding and postoperative histological diagnosis, a radiological scoring system combining these findings was developed. In another cohort of 17 patients, the sensitivity, specificity, and accuracy of the radiological score rates for differentiation between enchondromas and ACTs/chondrosarcomas were 88%, 89%, and 88%, respectively (p = 0.003). CONCLUSION: Radiological assessment with combined radiological findings is recommended to differentiate between enchondromas and ACT/chondrosarcomas.

Cartilage diseases.

Hyaline cartilages, fibrocartilages and elastic cartilages play multiple roles in the human body including bearing loads in articular joints and intervertebral discs, providing joint lubrication, forming the external ears and nose, supporting the trachea, and forming the long bones during development and growth. The structure and organization of cartilage's extracellular matrix (ECM) are the primary determinants of normal function. Most diseases involving cartilage lead to dramatic changes in the ECM which can govern disease progression (e.g., in osteoarthritis), cause the main symptoms of the disease (e.g., dwarfism caused by genetically inherited mutations) or occur as collateral damage in pathological processes occurring in other nearby tissues (e.g., osteochondritis dissecans and inflammatory arthropathies). Challenges associated with cartilage diseases include poor understanding of the etiology and pathogenesis, delayed diagnoses due to the aneural nature of the tissue and drug delivery challenges due to the avascular nature of adult cartilages. This narrative review provides an overview of the clinical and pathological features as well as current treatment options available for various cartilage diseases. Late breaking advances are also described in the quest for development and delivery of effective disease modifying drugs for cartilage diseases including osteoarthritis, the most common form of arthritis that affects hundreds of millions of people worldwide.

Integrating Morphology and Genetics in the Diagnosis of Cartilage Tumors.

Cartilage-forming tumors of bone are a heterogeneous group of tumors with different molecular mechanisms involved. Enchondromas are benign hyaline cartilage-forming tumors of medullary bone caused by mutations in IDH1 or IDH2. Osteochondromas are benign cartilage-capped bony projections at the surface of bone. IDH mutations are also found in dedifferentiated and periosteal chondrosarcoma. A recurrent HEY1-NCOA2 fusion characterizes mesenchymal chondrosarcoma. Molecular changes are increasingly used to improve diagnostic accuracy in chondrosarcomas. Detection of IDH mutations or HEY1-NCOA2 fusions has already proved their immense value, especially on small biopsy specimens or in case of unusual presentation.

Extraskeletal Mesenchymal Chondrosarcoma of Shoulder: An Extremely Rare Case.

INTRODUCTION: Extraskeletal chondrosarcoma (EMC) is a rare, aggressive neoplasm which has been seen in the soft tissue area. This soft tissue sarcoma is classified to myxoid and mesenchymal based on histologic criteria. The mesenchymal subtype has a poor prognosis. In approximately 50% of patient with EMC, we could observe soft tissue lesion and stippled calcification in the conventional radiography. CASE REPORT: In the current paper, we introduced a 47-year-old Iranian male patient having painless, mobile, nontender, and firm mass in left shoulder. We did not find neurovascular disturbance at the upper extremity, and the patient had a full range of motion in the left shoulder. The tumor was treated with wide resection and followed by radiation therapy. CONCLUSION: Complete wide resection of mesenchymal chondrosarcoma could be enough as an initial treatment and chemotherapy reserved for patients that have unresectable masses. Apparently, the main key in the treatment is the surgical resection, and this process is the most important method in their management.

Laryngeal chondroma.

Cartilaginous tumors of laryngeal skeleton are a rarity: Laryngeal chondroma is an unusual cause of upper airway obstruction. The present report illustrates a case of a 13 year old male presenting with mass in the subglottis. Histopathology of the mass revealed laryngeal chondroma.

Enfermedad de Ollier: reporte de un caso / Ollier's Disease: a case report

La enfermedad de Ollier es una encondromatosis múltiple querepresenta el 3 % de los tumores óseos benignos. Las encodromatosisson tumores cartilaginosos que pueden ensanchar el hueso yproducir la clásica característica de la calcifi cación moteada dentrode las lesiones. Se presenta el caso de un escolar masculino de6 años de edad con presencia de múltiples encondromas en lasextremidades torácicas y pélvicas derechas (húmero- radio y cubito,falanges de la mano, fémur, tibia, peroné, y falanges del pie), quehan producido deformidades angulares, rotacionales y asimetría deextremidades pélvicas, que han sido tratadas con osteotomías yfi jación externa tipo Ilizarow a nivel del defecto tumoral en la Unidadde Ortopedia pediátrica del Hospital del Niño de La Paz-Bolivia.

Ollier’s disease is a multiple enchondromatose which constitutes3% of benign bone tumors. Enchondromatoses can thicken thebone and produce the classical characteristics of the cartilaginoustumors: spotted calcifi cations within the lesion. I present the caseof a six year-old school boy who had multiple enchondromes in theright thoracic and pelvic extremities (humero-radius and cubitus,phalanges of the hand, femur, tibia, fi bula, and phalanges of the foot).that had produced angular, rotational deformities and asymmetry ofthe pelvic extremities which had been treated with osteotomy andexternal fi xation according to IIizarow at the level of the tumorousdefect, in the Unit of Pediatric Orthopedics of the Children’s Hospitalin La Paz, Bolivia.