The patient underwent then the surgical excision of the mass via a conservative and innovative trans-oral approach, to facilitate the enucleation of the numerous components of the neoformation (Fig. 3).
Finally, the histological examination revealed that the lesion was compatible with a SC (Fig. 4); the biopsy specimen resulted negative for biomarkers of soft tissue malignancies, such as S100, Actin 1A4, and CD34.18–20
The mass involved the pterygo-maxillary fossa with involvement of the parotid lodge and of the right TMJ; this multifocal extension suggested for a trans-oral surgical procedure, in the light of the suspicion of a possible malignant nature of the neoplasm. By means of CT and MRI images it was possible to identify a clear cleavage plane between the mass and the surrounding tissues: due to this approach, we were able to detach the neoformation from the lateral wall of the maxillary sinus and single it out from the parotid lodge. Sigmoid incisures allowed separating the neoplasm from the TMJ capsule.
Synovial chondromatosis is a rare, benign process that typically affects large joints in young adults. Although it commonly involves the knee,5 it may occur in the TMJ. The disease is characterized by the development of cartilaginous nodules within the synovial space from the synovial connective tissue matrix;21 the nodules subsequently degraded, detached, and become fluctuating intrarticular bodies tending to calcify in the joint space. The involved joint space may also become widened with possible articular erosions, eventually leading to secondary osteoarthritis.
Synovial chondromatosis of other joints has been reported to occur twice as often in males as in females, with a mean age of onset in the fifth decade.22 In the patient of TMJ, it occurs more often in females by a ratio of 4:1, and is usually located on the right side (right to left ratio of 4:1).23
In 1933, Axhausen24 described the first patient of SC affecting the TMJ. The etiology remains unknown, but generally a history of trauma and recent inflammations is often found in patients.24
Histologically, SC may be divided into 3 stages of development: metaplasia found in the synovial membrane without the presence of detached particles, metaplasia found in the synovial membrane with the presence of detached particles, and presence of detached particles, which may vary in size from less than 1 mm to greater than 10 mm.25
In the patient of larger joints, the classical triad of signs and symptoms is represented by restricted joint range of motion (65% of the patients), pain (57% of the patients), swelling (46.5% of the patients),16 which are also the main signs and symptoms identified in patients of SC of the TMJ.26 These features are often nonspecific, overlapping those of other TMJ diseases.27 In case of absence of physical signs, the development of nonspecific pain and headaches can lead to a delay in diagnosis or to a misdiagnosis of other more common causes of headaches.28 Data on the duration of the symptoms before diagnosis and intervention show that in the 80% of the patients reported in the literature, symptoms lasted for more than 2 years.16
Advanced imaging techniques, such as MRI and CT, are now being used with great success to evaluate TMJ disorders,27 helping to depict joint changes and the presence of loose bodies, suggesting the diagnosis of SC.29 Progresses in CT and MRI have improved the ability to delineate TMJ disease markedly, particularly with the use of sagittal and coronal section imaging. Magnetic resonance imaging may be useful for depicting the nodules in the early stages of formation, before ossification,24 and for planning surgery in the early stages. Noyek et al22 pointed out the radiologic features of SC in the TMJ: widening of the joint space, limitation of motion, irregularity of the joint surface, presence of calcified loose bodies (cartilage), and sclerosis or hyperostosis (overgrowth) of the glenoid fossa and mandibular condyle. These radiologic features, however, are also commonly seen in osteoarthritis involving the TMJ, except for the presence of calcified loose bodies.30 For this reason, differential diagnosis is also very important. Intrarticular temporomandibular pain most commonly is due to degenerative osteoarthritis, a typical progressive disease, osteosarcoma and chondrosarcoma, the most frequent, which are malignant diseases that can arise within the TMJ.31 Other causes of loose bodies within a joint include osteochondritis dissecans, the most common one,32 intracapsular fracture, avascular necrosis, tuberculous or pyogenic arthritis, rheumatoid arthritis, and neutrophic arthritis.32,33 The surgery has always been recommended as therapy of choice,3,28 but some authors advocate less invasive techniques, such as arthroscopy and 2-needle arthrocentesis, to remove the loose bodies from the joint space.31 Many authors suggested that CT, MRI imaging, and arthroscopy can facilitate the clinical diagnosis of synovial chondromatosis.25,34,35 Nevertheless, a definitive diagnosis can be made only by histological examination.27 In the reported case, the immunohistochemical analysis was negative for the following markers of malignancy: CD34, S100, and Actin 1A4;18–20 this laboratory analysis was leading to confirm the suspect of the diagnosis of chondromatosis, erasing in this way the initial doubts derived from the analysis of the radiological findings.
According to the latest suggestions, TMJ arthroscopy has no advantages over arthrocentesis in terms of efficacy, and none over open surgery in terms of postsurgical course.36,37 In many patients, additional procedures, such as total synovectomy, diskectomy and condylectomy, were performed for loose bodies’ removal alone; the choice of the surgical procedure should be based on the stage of the disease.25,28 In patients of an advanced stage of the disease, characterized by degeneration and calcification of the loose bodies and by a nonproliferative inactive synovial membrane, aggressive surgery with synovectomy might not be necessary.16 Since literature data do not provide useful information on the correlation between the stage of the disease and the type of surgical intervention, the experience of the surgeon is the main determinant;16 moreover, the intraoral condition should be carefully evaluated so to avoid intraoral infections or diseases.38
Once the loose bodies have been removed, the recurrence rate of SC appears to be very low; only 1 patient has been documented in the literature.39 This may suggest a spontaneous inactivation of the metaplastic process in most patients and, at the same time, that surgery can be considered a radical and definitive treatment.40,41
In conclusion, a correct clinical and instrumental approach, together with a multidisciplinary evaluation (pathological, oncological, and surgical), allowed performing a radical and clean surgical procedure. The marked cellular atypia emerged from the first biopsy, closely linked to hypothetical doubts of pulmonary metastases, has been clearly investigated by means of the reported postsurgery histological examinations. Three months after the surgery, lung and maxillofacial TC control showed a reduction of the lesion and the absence of residual local disease.
Due to our intraoral approach, the patient will avoid unaesthetic scars42 in the preauricular and facial regions without otherwise reducing the effectiveness of surgery; we can obtain that the patient regains a correct mouth opening with relative mandibular protrusion and lateral movements, close to normality. Our technique could represent a safe alternative approach43 aimed to treat this rare oncological pathology.
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Keywords:© 2016 by Mutaz B. Habal, MD.
Conservative surgery; oncological surgery; synovial chondromatosis; temporomandibular joint neoplasm