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PubMedDate not supplied

Synovial chondromatosis of the subtalar joint and tenosynovial chondromatosis of the posterior ankle.

Synovial chondromatosis is a rare, generally benign condition characterized by the formation of multiple cartilaginous nodules in the synovium of joints and occasionally on the tendon sheath or bursae. If the nodules are intra-articular, the condition is referred to as synovial chondromatosis; if extra-articular, tenosynovial chondromatosis. This space-occupying lesion can lead to chronic pain and limit the function of involved joints. We report a case of synovial chondromatosis involving the subtalar joint and tenosynovial chondromatosis involving the posterior ankle and review current definitions of this uncommon lower-extremity condition.

Ankle Joint↗
PubMed1996

[Synovial chondromatosis and chondrosarcoma. A study of the relationship between these two diseases].

PURPOSE OF THE STUDY: This retrospective study concerns six patients in whom chondrosarcoma was suspected to develop in synovial chondromatosis. We discuss for these cases different diagnosis. The authors expose the clinical signs and radiological aspects which suggest malignant transformation. We report the indispensable criteria for established diagnosis of malignant transformation of synovial chondromatosis and appropriated forms of therapeutic management were suggested. MATERIALS AND METHODS: Six patients, 3 males and 3 females from 36 to 58 years of age were included in this study. Three patients presented 6 months, 3 years and 25 years history of synovial chondromatosis of the knee joint. When the malignant transformation appeared, a surgical biopsy was performed and the pathologist diagnosed a chondrosarcoma in all cases. For the other three patients, the chondrosarcoma and synovial chondromatosis were diagnosed at the same time. The localization was shoulder, hip and knee. A surgical biopsy was performed and the pathologist diagnosed chondrosarcoma. Synovial chondromatosis was diagnosed by histologic examination of the resection or amputation specimen. TREATMENT AND RESULTS: Four patients had thigh amputation, one patient had "en bloc resection" of the hip-joint and the last patient had resection of the shoulder joint. In all cases, the histologic examination diagnosed chondrosarcoma and synovial chondromatosis. All patients were free of disease. DISCUSSION: The malignant transformation of synovial chondromatosis is rare but this diagnosis must be established to perform appropriate treatment. Other possible diagnosis are: low grade synovial chondrosarcoma initially diagnosed as a synovial chondromatosis. Bertoni believes that all cases of malignant transformation of synovial chondromatosis are initially low grade chondrosarcoma. We believe that his criteria are too strict for diagnosed chondrosarcoma. coexistence of synovial chondromatosis and synovial chondrosarcoma. These 2 diagnosis are extremely rare and their coexistence are unlikely. secondary synovial chondromatosis developed into chondrosarcoma. We don't have histologic criteria to confirm this diagnosis in all our cases. The symptoms that should suggest a malignant transformation of synovial chondromatosis were: rapid late deterioration of clinical conditions, bone invasion diagnosed by X-ray films and medullar invasion discovered by MRI. According to us, the indispensable criteria to diagnose malignant transformation were: 1.) histologic diagnosis of synovial chondromatosis established before diagnosis of chondrosarcoma, 2.) histologic diagnosis of chondrosarcoma on the same anatomic site as the synovial chondromatosis, 3.) diagnosis of chondrosarcoma and synovial chondromatosis on the same resection specimen. Only the three first cases were in accordance with these criteria. The treatment must be a "en bloc resection" of the joint or an amputation. CONCLUSION: Malignant transformation is rare, but this diagnosis should be established to perform adequate treatment. This diagnosis should be suspected when a rapid deterioration of the clinical status appeared and when bone involvement was detected by MRI. However, the danger still lies in the misinterpretation of the synovial chondromatosis as chondrosarcoma. This diagnosis can be made with clinical, radiological and pathological criteria. The treatment must be a wide resection or an amputation.

Adult↗
PubMed2005

Dysregulation of hedgehog signalling predisposes to synovial chondromatosis.

Synovial chondromatosis is a condition affecting joints in which metaplastic cartilage nodules arise from the synovium, causing pain, joint dysfunction, and ultimately joint destruction. Because dysregulation of hedgehog signalling is a feature of several benign cartilaginous tumours, expression of the hedgehog target genes PTC1 and GLI1 was examined in this study in samples from human synovial chondromatosis. Significantly higher expression levels were found in synovial chondromatosis than in the synovium, from which it arises. To determine if hedgehog-mediated transcription predisposes to synovial chondromatosis, the extra-toes mutant mouse, which harbours a heterozygous mutation in the hedgehog transcriptional repressor, Gli3, resulting in decreased expression of Gli3 protein, was studied. The extra-toes mutant mouse has a phenotype consistent with overactive hedgehog signalling, suggesting that Gli3 acts as a transcriptional repressor of limb development. Eighty-five per cent of Gli3 mutant mice developed synovial chondromatosis at 18 months of age, compared with 30% of wild-type littermates (p < 0.05). Three of the ten Gli3 mutant mice treated with triparanol, which blocks hedgehog signalling upstream of the Gli transcription factors, developed synovial chondromatosis, compared with eight of ten control mice. These data demonstrate that hedgehog signalling plays an important role in the development of synovial chondromatosis and suggest that blockade of hedgehog signalling may be a potential treatment for this disorder.

Animals↗
PubMed2000

Synovial chondromatosis in a facet joint of a cervical vertebra.

STUDY DESIGN: A case report of a cervical facet joint synovial chondromatosis. OBJECTIVES: To correlate the radiologic and histologic features of vertebral synovial chondromatosis with review of the literature. SUMMARY OF BACKGROUND DATA: Only two previous cases of vertebral facet joint synovial chondromatosis were found in a review of the English language medical literature. METHODS: A 39-year-old woman had severe cervical pain associated with neurologic signs and symptoms in the left upper extremity. Computed tomographic and magnetic resonance imaging studies were performed. RESULTS: Imaging studies showed lytic defects in the laminae of C3 and C4, with intermediate T1 and high T2 signal intensities. The diagnostic impression was that of a lymphangioma or synovial cyst. A laminectomy showed synovial tissue in both the C3-C4 facet joint and the lamina bone. Histologic examination disclosed synovial chondromatosis. CONCLUSIONS: Synovial chondromatosis of the vertebral spine is quite rare, this being only the third reported example. Direct invasion of the cancellous bone, as in this case, also is a very uncommon feature of chondromatosis. It is emphasized that when radiologic studies demonstrate a lesion with cartilaginous characteristics within or juxtaposed to a joint, synovial chondromatosis, despite its rarity, should be included in the differential diagnosis, regardless of the anatomic site.

Adult↗
PubMed2004

Vertebral synovial chondromatosis. Report of two cases and review of the literature.

Synovial chondromatosis is an uncommon disorder characterized by the formation of multiple cartilaginous nodules within the synovium, most commonly affecting large joints. Its involvement with the spine is rare; only six cases have been reported. The authors describe two patients with synovial chondromatosis involving the cervical spine. In the first case, synovial chondromatosis arose from the left C1-2 facet joint. This patient underwent a two-stage procedure including a posterior approach for tumor resection and occipitocervical fusion as well as a transmandibular circumglossal approach to the anterior craniocervical junction to complete the tumor removal. Interestingly, on histopathological examination, scattered foci of low-grade chondrosarcoma were intermixed within the synovial chondromatosis. To the authors' knowledge, this is the first report of secondary low-grade chondrosarcoma arising in vertebral synovial chondromatosis. In the second case, synovial chondromatosis involved the left C4-5 facet joint. Tumor resection and cervical fusion were performed via a posterior approach. In this report, the authors describe the clinical presentation, radiographic findings, operative details, histopathological features, and clinicoradiological follow-up data obtained in these two patients and review the literature pertaining to this rare entity.

Adult↗
PubMed2003

Chromosome 6 abnormalities are recurrent in synovial chondromatosis.

Synovial chondromatosis, a lesion composed of multiple nodules of cartilage involving articular or tendon sheath synovial membranes, has traditionally been considered a metaplastic condition. A specific or characteristic chromosomal anomaly has not yet been identified in synovial chondromatosis. Cytogenetic and molecular cytogenetic analyses of three cases of synovial chondromatosis revealed clonal karyotypic abnormalities in all three cases including structural abnormalities of chromosome 6 in two. Anomalies of chromosome 6 have been observed in three of five previously reported synovial chondromatosis cases. These findings support a neoplastic origin for synovial chondromatosis and suggest that chromosome 6 aberrations are recurrent in this lesion.

Adult↗
PubMed2003

Synovial chondromatosis presenting with cervical radiculopathy: a case report.

STUDY DESIGN: A case report is presented. OBJECTIVES: To report a case of synovial chondromatosis of a cervical facet joint and describe the appearance with magnetic resonance imaging and computed tomography myelography. SUMMARY OF BACKGROUND DATA: Synovial chondromatosis is an uncommon disorder characterized by the presence of multiple cartilaginous or osteocartilaginous nodules in the synovium of a joint space. Synovial chondromatosis in the cervical facet joint is rare. METHOD: A 52-year-old woman experienced the sudden onset of severe pain in the dorsal shoulder girdle and in the ulnar side of her right arm and forearm. This refractory pain only responded to an epidural nerve root block. Neurologic examination showed right nerve root signs that ranged from the C7 to Th1 segments of the spinal cord. Radiologic and electrophysiological examinations were carried out. RESULT: A mass was found in the right facet joint between C7 and Th1 with magnetic resonance imaging and computed tomography myelography. These investigations clearly indicated the location, size, and extent of the lesion accompanying the irregularity of the joint and osteolytic change. Somatosensory-evoked potentials with right ulnar nerve stimulation indicated a significant conduction block in the lower right cervical nerve roots. After surgical removal of this lesion, the neurologic symptoms markedly improved. The histopathology diagnosed synovial chondromatosis. CONCLUSION: Synovial chondromatosis should be included in the differential diagnosis of radiculopathies of unknown etiology.

Cervical Vertebrae↗
PubMedDate not supplied

[Intraarticular chondromatosis].

Intraarticular chondromatosis is subdivided into primary (synovial) and secondary one. Synovial chondromatosis is a benign, focal proliferation of cartilaginous tissue within the synovial membrane of a joint, articular bursa and tendons. More often synovial chondromatosis is present in the synovial membrane. Some nodules loose a bond to the synovial membrane and form chondromal intraarticular bodies that are freely located within the joint cavity. Secondary intraarticular chondromatosis develops in the presence of a pre-existing process, such as injury, degenerative joint diseases including osteoarthrosis. In secondary intraarticular chondromatosis, loose intraarticular bodies are most commonly formed on the basis of a small disconnected fragment of hyaline articular cartilage.

Adult↗
PubMed2000

Fine-needle aspiration diagnosis of synovial chondromatosis of the tibiofibular joint.

We report a case of synovial chondromatosis of the tibiofibular joint in a 25-year-old woman that was diagnosed by fine-needle aspiration (FNA). The patient presented with pain in the left knee and a mass in the popliteal fossa. Synovial chondromatosis usually presents with joint symptoms and is often associated with intra-articular loose bodies, whereas presentation as a soft tissue mass is unusual and may raise the clinical suspicion of malignant neoplasm. The diagnosis is commonly confirmed by histopathologic examination of biopsy or excision of the specimen. To the best of our knowledge, this is the first case of synovial chondromatosis of a large joint successfully diagnosed by FNA. Two cases of synovial chondromatosis of the temporomandibular joint have been reported in which the diagnosis was suspected on the basis of FNA. In both these cases, the final diagnosis was established by histopathology of the excised specimens.

Adult↗
PubMed2003

Synovial chondromatosis of the temporomandibular joint: clinical, surgical and histological aspects.

Nine patients with histologically confirmed unilateral synovial chondromatosis of the temporomandibular joint were treated surgically with extirpation of loose bodies and partial synovectomy. In six of them the histological material was available for a systematic examination. The results of treatment were evaluated clinically and with MRI after a follow-up ranging between 1 and 17 years. Our findings suggest that synovial chondromatosis of the temporomandibular affects only the synovial lining of the upper compartment. The histological appearance is that of a benign chronic inflammation varying in severity and with metaplastic activity. The most specific clinical sign of synovial chondromatosis is swelling over the joint. Distension of the lateral capsule and fluid in the joint on the MRI are very suggestive of this diagnosis. Loose bodies also indicate synovial chondromatosis, but they are not always detected on the preoperative MRI. The surgical treatment should be conservative and include thorough removal of the loose bodies and partial synovectomy in areas with marked inflammation.

Adult↗
PubMed2002

Synovial chondromatosis of the temporomandibular joint: clinical, diagnostic, and histomorphologic findings.

Objective. The primary diagnosis of synovial chondromatosis of the temporomandibular joint is extremely rare. Often many months, or even years pass before the diagnosis is confirmed. Study Design. Eight patients with synovial chondromatosis were treated by arthrotomy. In addition to the definitive diagnosis, histologic classification according to the stages proposed by Milgram was undertaken and confirmed with the literature. Results. The predominant symptoms of synovial chondromatosis, including pain, swelling, restricted movement of the mandible, and crepitation, were evaluated. Nevertheless, more than 80% of the patients were previously primarily treated on the basis of other tentative diagnoses. Conclusion. Synovial chondromatosis of the temporomandibular joint should be included in the differential diagnosis of chronic swelling and pain in the preauricular region, and the patient should be sent for appropriate diagnostics and therapy at an early stage.

Adult↗
PubMed2004

A case of tenosynovial chondromatosis with tophus-like deposits.

Tenosynovial chondromatosis has not been well recognized because of its rarity, but it is clinically important because of its high rate of recurrence. We report here a case of tenosynovial chondromatosis with deposits of crystalline material that appeared to be sodium urate (gouty tophi). A 37-year-old Japanese man was admitted because of a hard mass in his left third finger. He had undergone surgery at the same anatomical site four and seven years previously. The roentgenogram revealed a soft tissue mass in the flexor aspect of the proximal phalanx. At operation, the tumor was found to have arisen in the tendon sheath. Histopathological examination showed that the tumor was composed of well-defined, multiple, cartilaginous nodules that were surrounded by tenosynovial tissue. A few of the nodules were calcified. The chondrocytes had mild atypia, and were immunopositive for S-100 protein. A diagnosis of tenosynovial chondromatosis was made. The nodules also contained crystalline deposits, which bore a histological resemblance to gouty tophi. We were unable to define the exact nature of these deposits even by transmission electron microscopy and electron roentgenographic microanalysis. Crystalline deposits in chondromas of soft tissue have been reported but not in tenosynovial chondromatosis.

Adult↗
PubMedDate not supplied

[Synovial chondromatosis of the temporomandibular joint].

Synovial chondromatosis is an uncommon monoarticular proliferative disease of the synovium characterized by loose bodies developed in the synovial membrane. The literature is reviewed and two cases of synovial chondromatosis of the temporomandibular joint characterized by swelling, pain and limitation of mandibular movement are reported. Radiographic evidence of loose bodies may or may not be present. Computed tomography (CT) or magnetic resonance (MR) might be helpful in the diagnosis of synovial chondromatosis. The treatment of choice of synovial chondromatosis is surgical and the loose bodies should be removed by arthrotomy with examination of the joint cavity. In our patients, no recurrence had occurred according to other authors' experience.

Adult↗
PubMed2001

Synovial chondromatosis of the elbow.

Synovial chondromatosis is an uncommon disorder with rare occurrence in the elbow. Case reports in the literature for elbow synovial chondromatosis have described presenting symptoms secondary to peripheral nerve compressions or localized bursitis. We discuss a case of synovial chondromatosis of the elbow that presented as an isolated soft-tissue mass over the radial head-more suggestive of a soft-tissue tumor than of synovial chondromatosis.

Chondromatosis, Synovial↗
PubMedDate not supplied

Synovial chondromatosis of the temporomandibular joint with extension to the middle cranial fossa.

A rare case of synovial chondromatosis with extension to the middle cranial fossa is reported. Synovial chondromatosis, a benign disorder characterized by multiple cartilaginous, free-floating nodules that originate from the synovial membrane is not exclusive to the temporomandibular joint (TMJ). This condition is commonly seen in the axial skeleton and can involve multiple joints. In this case, synovial chondromatosis of the TMJ led to complete bony erosion of the glenoid fossa extending into the middle cranial fossa. Although plain radiographs showed the involvement of the joint, Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) provided more detailed information about the lesion in all three dimensions. This case demonstrates the value of CT and MRI in both the diagnosis and treatment planning. A review of previously reported cases of synovial chondromatosis with cranial extensions is included.

Aged↗
PubMed1996

Synovial chondromatosis of the metacarpophalangeal joint: case report and review of the literature.

Intra-articular synovial chondromatosis in the hand is rare but should be considered in the differential diagnosis of a swollen, stiff or painful joint. Other possible diagnoses include osteoarthritis, rheumatoid arthritis, gout, trauma and chronic infection, and unless enchondral ossification of loose bodies is seen the diagnosis of synovial chondromatosis may not be made preoperatively. A 69-year-old man with synovial chondromatosis of the metacarpophalangeal joint is reported. The joint was swollen and tender. He had not sustained trauma and there was no evidence of arthritis, involvement of other joints or infection. Complete synovectomy with removal of all loose bodies was successful and his symptoms resolved. Intra-articular synovial chondromatosis is a benign condition, but spontaneous resolution is the exception and surgical synovectomy remains the most effective treatment.

Aged↗
PubMed2003

Tenosynovial (extraarticular) chondromatosis: an analysis of 37 cases of an underrecognized clinicopathologic entity with a strong predilection for the hands and feet and a high local recurrence rate.

Tenosynovial chondromatosis is a multinodular cartilaginous proliferation that arises from the tenosynovial membranes. This report describes the clinical, radiologic, and histopathologic findings in 37 cases of this uncommon entity. There were 17 males and 20 females, ranging in age from 20 to 86 years (mean and median age, 46 years). The process involved tenosynovium of the fingers (n = 19), feet (n = 8), wrists (n = 4), ankles (n = 2), hand, not otherwise specified, or palm (n = 2), knee (n = 1), and forearm (n = 1). Signs of disease or symptoms were present for 5 weeks to 18 years (median duration, approximately 2 years) before surgical excision. The two most common complaints were a painless mass and a mass that was mildly tender with pressure. None of the tumors had clinical, radiologic, or histopathologic evidence of articular or bone involvement. Histologically, all tumors consisted of a multinodular cartilaginous proliferation involving tenosynovium and/or subsynovial connective tissue. Mild or moderate atypia, as encountered in chondroma of soft parts and synovial chondromatosis, was a frequent finding. Follow-up information was available for 16 patients (43%). Only two patients with follow-up information remained disease free after their initial surgical procedure. Seven patients had one recurrence and seven patients had two or more recurrences. Tenosynovial chondromatosis appears to be an extraarticular counterpart of synovial (intraarticular) chondromatosis. Our review indicates this process is often confused with chondroma of soft parts, in part, because both entities have a predilection for the hands and feet. Diagnosis of this underrecognized entity is of clinical importance because of the high local recurrence rate.

Adult↗