[Diagnosis and differentiation diagnosis of primary temporomandibular joint cartilage and giant cell related tumor lesions].

Cartilage and giant cell-related neoplastic lesions originating in the temporomandibular joint region have similar clinical, imaging and pathological manifestations, making the diagnosis of these disorders challenging to varying degrees. Diagnostic findings can influence treatment procedures and a definitive pathological diagnosis is important for the prognosis of these conditions. In this article, we discuss the pathological diagnosis and differentiation of four benign cartilage and giant cell related tumors and tumor-like lesions that occur in the temporomandibular joint, namely synovial chondromatosis, tumoral calcium pyrophosphate deposition disease, pigmented villonodular synovitis and chondroblastoma, taking into account their clinical features and histological manifestations, with a view to providing a basis for clinical management.

A Systematic Approach to Diagnosing Arthritis Based on Radiological Imaging.

Radiology plays a key role in the diagnosis of arthritis. We herein suggest an algorithmic approach to diagnosing arthritis. First, the number of joint involvements is reviewed. Monoarticular arthritis includes septic arthritis, pigmented villonodular synovitis, and synovial chondromatosis. Second, polyarticular arthritis can be categorized by its characteristics: erosive, productive, and mixed. Erosive disease includes rheumatoid arthritis, hemophilia, and amyloidosis, while productive disease includes osteoarthritis and hemochromatosis. Third, mixed diseases are subcategorized by symmetricity. Ankylosing spondylitis and inflammatory bowel disease related arthritis affect joints symmetrically, while psoriatic arthritis, reactive arthritis, and crystalline arthropathy are asymmetric. Adjacent soft tissue density, periostitis, and bone density are ancillary findings that can be used as additional differential diagnostic clues. The final step in identifying the type of arthritis is to check whether the location is a site frequently affected by one particular disease over another. This systematic approach would be helpful for radiologists in diagnosing arthritis.

"Shoulder pain and limitation of motion in a young girl: think different".

BACKGROUND: Primary Synovial Chondromatosis (PSC) is a rare benign tumor of the synovial membrane in which cartilage metaplasia produces calcific loose bodies within the articular space. Only a few cases are reported in the pediatric population and its etiology remains unknown. This condition typically affects large weight-bearing joints with pain, swelling and decrease range of motion. Due to its slow progressions, delayed diagnosis is frequent and differential diagnosis should consider other chronic arthritis and malignancies. While arthroscopic removal of loose bodies is the current treatment up to now, the association of partial or complete synovectomy is debated. CASE PRESENTATION: We report about a 14-year-old girl with a long-lasting right shoulder pain, especially during movements or exercise, localized tenderness and hypotonia of the glenohumeral joint. No previous trauma was mentioned. Blood exams, Mantoux test and plain radiography of the right shoulder were unremarkable. Ultrasound imaging revealed echogenic and calcified bodies stretching the glenohumeral joint and dislocating the long head of biceps tendon. Magnetic resonance showed a "rice-grain" pattern of the right shoulder. From an arthroscopic surgery, multiple loose white bodies were removed within the synovial membrane, and synovial chondromatosis was confirmed by histological analysis. At one month follow up visit, the patient completely recovered without pain. CONCLUSION: Synovial chondromatosis is a very uncommon cause of mono articular pain in children, especially when it affects shoulder. Pediatricians should keep in mind this condition to avoid delayed diagnosis and treatment, even in consideration of the low risk of malignant transformation. Through this case, we would highlight common diagnostic pitfalls and treatment of synovial chondromatosis.

Synovial Chondromatosis of Hip Joint in a Patient with Ankylosing Spondylitis: A Case Report.

Synovial chondromatosis in association with ankylosing spondylitis is extremely rare and has been reported only once before and this case report is presenting a similar case. The knee is the preferential site of involvement with involvement of the hip being reported sparsely. We herein report a case of a 52-year-old male who came with complaints of the lower back pain for 5 years and left hip pain for 1.5 years who was diagnosed with synovial chondromatosis of the hip joint with axial ankylosing spondylitis and was managed operatively. We here review briefly the clinical manifestations, pathogenesis, diagnosis, previously reported cases as well as treatment of synovial chondromatosis in patients with immune-mediated inflammatory arthritides. There should be a high index of suspicion to diagnose synovial chondromatosis in association with inflammatory arthritides. We also believe that surgical management is an effective method of treatment of an established synovial chondromatosis of the hip joint. Keywords: ankylosing spondylitis; case reports; hip joint; synovial chondromatosis.

Evaluation and Management of Intra-Articular Tumors of the Knee.

Intra-articular tumors of the knee are most commonly benign. Overall, this is a relatively rare clinical presentation. The differential diagnosis includes pigmented villonodular synovitis, synovial chondromatosis, lipoma arborescens, synovial hemangioma, and very rarely primary sarcoma (synovial sarcoma being the most common). The clinical presentation for these conditions is usually non-specific, but radiographic and advanced imaging findings are able to differentiate some of these tumors. It is essential to obtain and send tissue specimens to pathology for histologic analysis to rule out a primary malignancy as a missed diagnosis can have grave implications on patient outcomes. This review summarized key aspects of diagnosis and treatment for these conditions.

Avoiding anchoring bias in unexplained chronic pain: an unexpected diagnosis of synovial osteochondromatosis.

Unconscious biases may influence clinical decision making, leading to diagnostic error. Anchoring bias occurs when a physician relies too heavily on the initial data received. We present a 57-year-old man with a 3-year history of unexplained right thigh pain who was referred to a physiatry clinic for suggestions on managing presumed non-organic pain. The patient had previously been assessed by numerous specialists and had undergone several imaging investigations, with no identifiable cause for his pain. Physical examination was challenging and there were several 'yellow flags' on history. A thorough reconsideration of the possible diagnoses led to the discovery of hip synovial osteochondromatosis as the cause for his symptoms. Over-reliance on the referral information may have led to this diagnosis being missed. In patients with unexplained pain, it is important to be aware of anchoring bias in order to avoid missing rare diagnoses.

[Clinical analysis of arthroscopy used in the diagnosis and treatment of synovial chondromatosis of the temporomandibular joint].

OBJECTIVES: To evaluate the value of arthroscopy in the diagnosis and treatment of synovial chondromatosis of the temporomandibular joint (TMJSC). METHODS: The cases of 16 patients preliminarily diagnosed with TMJSC by magnetic resonance imaging (MRI) from July 2011 to December 2018 were analyzed retrospectively. If the diagnosis was confirmed by arthroscopy, the opening operation was performed. The preoperative MRI, arthroscopy and opening operation, postoperative pathology and postoperative MRI of confirmed cases were analyzed, and clinical follow-up was performed to evaluate the curative effect of open surgery. The degree of mouth opening and visual analogue scale (VAS) scores for pain pre-operation and during follow-up of the confirmed cases were analyzed by t-test. RESULTS: Fourteen cases of TMJSC were diagnosed by arthroscopy, consistent with the postoperative pathological diagnosis. Postoperative MRI examination showed that articular cavity lesions basically disappeared. Ten patients with synovial chondromatosis were followed-up (follow-up rate, 71.4%) from 6 months to 7 years and 8 months (average follow-up time, 17.6 months); no recurrence was found, and clinical symptoms improved by varying degrees. Before operation and at follow-up, t-test results of opening degree difference were t=7.757, P<0.05; t-test results of VAS were t=-3.274, P<0.05. CONCLUSIONS: Arthroscopy is essential in the diagnosis and treatment of TMJ synovial chondromatosis.

Delayed-onset progressive pseudorheumatoid dysplasia with secondary synovial chondromatosis.

A 25-year-old man presented to us with progressive multiple joint pain, enlargement, and restricted movements. X-rays showed platyspondyly, multiple epiphyseal widening, synovial chondromatosis, and decreased bone stock and cortical thickness. Genetic testing showed biallelic pathogenic variants in CCN6 which confirmed the diagnosis of progressive pseudorheumatoid dysplasia. Supportive care, physical therapy, genetic and psychological counselling were provided to the patient.

A molecular study of synovial chondromatosis.

Synovial chondromatosis (SC) is a rare benign cartilaginous neoplasm in which recurrent fibronectin 1 (FN1) and activin receptor 2A (ACVR2A) gene rearrangements have been recently reported. Triggered by a case of malignant transformation in SC (synovial chondrosarcoma) showing a novel KMT2A-BCOR gene fusion by targeted RNA sequencing, we sought to evaluate the molecular abnormalities in a cohort of 27 SC cases using a combined methodology of fluorescence in situ hybridization (FISH) and/or targeted RNA sequencing. Results showed that FN1 and /or ACVR2A gene rearrangements were noted in 18 cases (67%), with an FN1-ACVR2A fusion being confirmed in 15 (56%) cases. Two cases showed only FN1 gene rearrangement, without other abnormalities. A novel FN1-NFATc2 gene fusion was noted in one case by RNA sequencing. The remaining nine cases showed no abnormalities in FN1 and ACVR2A genes. No additional cases showed BCOR gene alterations. In conclusion, this study confirms that FN1-ACVR2A fusion is the leading pathogenetic event in SC, at even higher frequency than previously reported. FISH methodology emerges as an appropriate tool in the identification of FN1 and ACVR2A gene abnormalities, which can be used in challenging cases. Further studies are needed to determine the recurrent potential of BCOR abnormalities in this disease.

Potential utility of anti-TNF drugs in synovial chondromatosis associated with ankylosing spondylitis.

We describe a previously unreported association of ankylosing spondylitis with synovial chondromatosis, and briefly review previously reported cases and treatment of synovial chondromatosis in patients with other immune-mediated inflammatory arthritides. A 20-year-old man with ankylosing spondylitis whose axial disease was in remission with nonsteroidal anti-inflammatory drugs and oral disease-modifying anti-rheumatic drugs developed recurrent right knee pain and swelling. Magnetic resonance imaging of his right knee revealed calcified loose bodies, suggestive of synovial chondromatosis. While waiting for the surgical intervention and other invasive therapy previously reported in patients with synovial chondromatosis, a trial of etanercept eliminated the pain and swelling of the knee; however, the loose bodies have persisted during the 2-year follow-up. Thus, synovial chondromatosis should be considered in the differential diagnoses of a refractory monoarticular pain and swelling in patients with otherwise controlled inflammatory arthritis. Our report advocates a trial of anti-tumor necrosis factor drugs, which might delay the need for invasive therapy in patients with synovial chondromatosis.

A Rare Case of Distal Interphalangeal Chondromatosis in the Middle Finger: Case Report With Review of Literature.

Primary synovial chondromatosis is a benign condition and consists of cartilaginous metaplasia of the synovium, which leads to the production of intra-articular and periarticular osteocartilaginous bodies. The entity usually involves the synovium of large joints (knee, hip, elbow, and shoulder). It has also been reported in the hand, especially involving interphalangeal or metacarpal joints of digits. The most common symptoms are pain, swelling, loss of range of motion and muscle atrophy. Because of its low prevalence and non-specific symptoms, synovial chondromatosis can present a diagnostic challenge for the hand surgeon and may lead to a delay in treatment. To date, only three cases involving distal interphalangeal joint have been reported. This article describes a rare case of primary articular synovial chondromatosis in the distal interphalangeal joint of a 65-year-old female, giving a deep insight into this pathology, and reviews the literature.

Huge Bursitis and Bursal Synovial Osteochondromatosis Associated With Scapular Osteochondroma Mimicking a Giant Calcific Mass of the Chest Wall.

Osteochondroma is the most common benign bone tumor, but it rarely arises from the scapula. Scapulothoracic bursitis is quite rare and osteochondroma is one of the unusual causes of this condition. Synovial chondromatosis may occur extremely uncommonly in this bursa. We reported an unusual case of scapulothoracic bursitis with synovial chondromatosis, which is caused by osteochondroma. To the best of our knowledge, there is no defined chondromatosis in the scapulothoracic bursa secondary to scapular osteochondroma in the literature.

Presentación inusual de condromatosis sinovial de la rodilla: caso reportado / Unusual presentation of synovial chondromatosis of the knee. Case report

La condromatosis sinovial se caracteriza por la formación de múltiples nódulos cartilaginosos intraarticulares del tejido subsinovial, que puede ser diagnosticada erróneamente debido a la ausencia de un examen físico, o de hallazgos en RM característicos en etapas iniciales. Se presenta un caso de una mujer de 33 años que consulta por dolor, pseudobloqueo y contractura en flexión de rodilla izquierda sin antecedente traumático previo. Debido a la sospecha de condromatosis sinovial de rodilla, se indicó la exploración artroscópica. Intraoperatoriamente se identificaron múltiples cuerpos sueltos intra y extraarticulares cartilaginosos: se realizó una sinovectomía radical y extracción de cuerpos libres. Al año de seguimiento, el paciente recuperó el rango completo de movimiento y retornó íntegramente a la actividad deportiva. Los resultados del presente informe indican que, en casos en los que existe una sospecha de condromatosis sinovial, la exploración artroscópica es un método eficaz para el diagnóstico y tratamiento con resultados satisfactorios. Tipo de estudio: Reporte de caso. Nivel de evidencia: IV

We report an unusual presentation of synovial chondromatosis of the knee in a 33-year-old women. At this unusual age and location, there is a greater probability of missed diagnosis, due to the lack of definite informative incidence, and difficulty in finding the lesions. In this case, intra and extraarticular multiple loose bodies were hidden. After careful arthroscopic inspection, we found numerous cartilaginous loose bodies and removed them with arthroscopy in association with sinovectomy. Synovial chondromatosis, is a rare, benign condition characterized by the formation of multiple intraarticular cartilaginous nodules from the subsynovial tissue. Synovial chondromatosis can be often misdiagnosed due to the lack of a specific physical exam or MRI findings in early stages. The present report examines a 33-year-old female with symptoms of left knee flexion contracture, pain mechanical locking without previous traumatic event. Following admittance, an arthroscopic exploration was conducted. Multiple cartilage-like intra and extraarticular loose bodies were identified in the patient's joints during surgery. Radical synovectomy and removal of loose bodies were performed. Following one year follow-up, the patient recovered full range of motion and returned to sports activities. The results of the present report indicate that, in cases where synovial chondromatosis is suspected, arthroscopic exploration is an effective method for diagnosis and treatment with satisfactory results. Type of Study: Case report. Level of evidence: IV

Synovial chondromatosis of the hip joint in childhood: A case report and literature review.

RATIONALE: Pain in the hip joint is a common symptom in children. The common diseases leading to pain in the hip joint in children include transient synovitis of the hip, septic arthritis of the hip, and Legg-Calve-Perthes disease. PATIENT CONCERNS: A 7-year-old boy was admitted due to pain in the right hip joint and limping for more than 1 month. DIAGNOSIS: Synovial chondromatosis. INTERVENTIONS: The patient underwent a hip open surgery, all the loose bodies in articular capsule were removed. OUTCOMES: At the 6-month follow-up, pain and limping disappeared, and the range of activity of the hip joint was restored to a normal level. CONCLUSIONS: Synovial chondromatosis is an uncommon disease which can cause pain of hip joint in children. LESSONS: When the pediatric orthopedic surgeon treats the children suffered with hip pain the surgeon should be aware of this rare disease.