Intra-articular Pigmented Villonodular Synovitis Research Articles

E69 Localised intraarticular pigmented villonodular synovitis in an adolescent patient: a case-based review of the literature

Abstract Introduction Pigmented villonodular synovitis (PVNS) is a rare, locally aggressive subtype of tenosynovial giant cell tumours that affect the synovium of the joints and tendons, occurring either focally or diffusely associated with the deposition of hemosiderin into the involved joint(s) and soft tissues. Although PVNS is historically considered an inflammatory process, recent evidence suggests the possibility of a neoplastic transformation. In addition, despite being typically found as a monoarticular knee disorder, other large and small joints can also be affected. The disease is rarely reported in African children, with most reported cases not histologically confirmed. We, therefore, present the first histologically proven case of localised intraarticular PVNS in an African teenager who presented with unilateral left knee swelling and pain. Method/Setting We report the case of a 16-year-old male teenager who presented to the rheumatology clinic with a nine-month history of non-traumatic anterior left knee swelling and pain that increased over time with associated difficulty walking and standing without aid. After clinical examination and relevant investigations, Magnetic Resonant Imaging (MRI) was done to confirm our suspicion of PVNS. Findings/Treatment Examination revealed a tender, swollen knee with a fluctuant palpable infrapatellar mass. Knee arthrocentesis yielded about 200mls of non-clotted hemorrhagic effusions. The patient had an open synovectomy following MRI, which was highly suggestive of pigmented villonodular synovitis with extensive suprapatellar bursitis. Specific histological findings (below) further confirmed the diagnosis. He commenced early physiotherapy, and symptoms resolved without recurrence of swelling eleven months after surgery. Conclusion This case highlights the visual presentation of an uncommon joint disorder and suggests it is considered a differential diagnosis of non-traumatic monoarticular pain and swelling in our rheumatology clinic set-up and among teenagers in whom juvenile rheumatoid arthritis would have been the first differential. Key words: Pigmented villonodular synovitis, Synovectomy, Multinucleated giant cells Contribution statement: AA conceived the idea to write the case report, draft the first manuscript, and manage the patient. YA, HO and AF edited the manuscript, while AIL interpreted the histological findings. All the authors approved the final version of the manuscript. Disclosure statement: None declared. Ethics and patient consent: This case report adheres to the Helsinki declaration, and the patient’s parents gave written consent to publish the details of the information. Additional information: Any institution did not fund this publication. Acknowledgements: We thank Dr Isaac Akanbi and Dr Bukar for interpreting the MRI and performing the surgery.

Pigmented villonodular synovitis

Pigmented villonodular synovitis

Pigmented Villonodular Synovitis: Current Concepts About Diagnosis and Management

At present, the treatment strategies in patients with localized and diffuse forms of pigmented villonodular synovitis have more or less been standardized. However, these strategies are not optimal because high recurrence rates persist and studies with a sufficient level of evidence are lacking. This systematic review article describes all known treatment options for intra-articular pigmented villonodular synovitis and their clinical results. Based on this research, we provide guidelines to support physicians in making the optimal treatment decisions. Given the rarity of the disease, randomized studies are not to be expected, but an international registry through existing networks would offer the benefit of getting a better insight into the outcome of this disease. Therefore, we propose a basic set of data to be investigated and ideally to be reported on in such a registry.

Pigmented villo-nodular synovitis of joints and tendon sheaths

Abstract Pigmented villo-nodular synovitis (PVNS) is a rare, benign and aggressive disorder of synovial joints and tendon sheaths which is capable of eroding articular structures and bone. It represents a spectrum of pathological disorders ranging from the most common giant-cell tumour of tendon sheath to localized or diffuse intra-articular PVNS lesions and may be associated with joint erosions. It is best treated surgically but recurs if inadequately excised. Adjunctive therapies include injection of intra-articular radio-isotopic materials and external beam radiotherapy.

Pigmented villonodular synovitis of the knee: A case report

Pigmented villonodular synovitis (PVNS) is characterized by hyperplasia of the synovial tissue in joints, of tendon sheaths, and of the mucous membranes, or fibrous tissue adjacent to the tendons. Its etiology is unknown. We report a case of diffuse intra-articular PVNS of the right knee in a 38-year-old man. Ultrasound and magnetic resonance imaging (MRI) features of the disease are described.

Arthroscopic management of intra-articular pigmented villonodular synovitis of temporomandibular joint

This retrospective study reports on four patients with intra-articular pigmented villonodular synovitis (PVNS) of the temporomandibular joint (TMJ) who were managed with arthroscopy between 2002 and 2009. There were three females and one male, with a mean age of 46 years at diagnosis. The common symptoms were trismus and pain. No pre-auricular swelling or mass was detected. Magnetic resonance imaging (MRI) and arthrography showed an anteriorly displaced disc, disc perforation, osteophyte of the condyle, or increased joint effusion. No neoplasm was suspected radiologically. Under arthroscopy, a yellow nodule and loose bodies were found in one patient, and a yellow or brown hyperplasia of the synovial membrane was noted in the other three patients. Degeneration of the articular cartilage was detected in two patients. The arthroscopic procedures used for every patient were partial synovectomy and debridement of articular surfaces with electric shaving and coblation. Arthroscopic disc repositioning was performed for the two young patients. Postoperative histological examination verified the diagnosis of PVNS of the TMJ. The average follow-up period was 57.4 months, and no recurrence was found. Arthroscopy has proved to be a useful method for the management of intra-articular PVNS of the TMJ.

Diffuse Pigmented Villonodular Synovitis

Because of the high local recurrence rate associated with surgical resection alone, patients with diffuse intraarticular pigmented villonodular synovitis were treated with surgical resection followed by colloidal chromic P32 synoviorthesis. The medical records of nine consecutive patients treated in this manner were reviewed retrospectively to determine the recurrence rate of pigmented villonodular synovitis. All patients had either one or two surgical resections (arthroscopy in one patient, open resection in seven, arthroscopy and open resection in one). The involved joints included six knees and one each, ankle, elbow, and hip. Eight of the nine patients remained recurrence free at a mean followup of 38 months (range, 19-60 months) after surgery. One patient had a suspected asymptomatic recurrence documented by magnetic resonance imaging 29 months after surgery. Seven patients reported their normal activities as unrestricted. Five reported improved activity levels, one reported the activity level remained the same, and one reported activity as the same or better. None reported reduced activity levels. In these patients synoviorthesis with colloidal chromic P32 following gross resection of all obvious pigmented villonodular synovitis provided local disease control in all but one.

Localized pigmented villonodular synovitis of the patellar fat pad: A report of two cases

Pigmented villonodular synovitis (PVNS) of the knee is a well-recognized entity. We report on two cases of localized PVNS that involved the patellar fat pad, an area of involvement not previously reported. Diagnostic and therapeutic arthroscopies were performed, and typical findings of localized PVNS were found. Complete resection of the lesions were performed arthroscopically. At short-term follow-up, all symptoms had resolved. Arthroscopy can be used as an effective diagnostic tool for identification of intraarticular PVNS of the knee. In the case of localized involvement, it also can be considered as definitive treatment.

Malignant giant cell tumor of synovium and locally destructive pigmented villonodular synovitis: Ultrastructural and immunohistochemical study and review of the literature

The first reported case of an intraarticular malignant giant cell tumor of synovium studied with electron microscopic and immunohistochemical examination is presented, together with a case of diffuse intraarticular pigmented villonodular synovitis with extensive bone destruction. The malignant case was dominated by uniform cells positive for histiocytic markers, the fine structure showing a gradual change from cells dominated by organelles serving a secretory function to cells with phagocytic activity. The reported cases of giant cell tumor of the tendon sheath indicate that the pertinent histologic changes regarding malignancy are an increase in cell polymorphism and in the number of mitoses, and a decrease in the number of multinucleated giant cells.

Pigmented Villonodular Synovitis of Joints

In a series of 38 young adults with intra-articular pigmented villonodular synovitis, the joint predominantly affected was the knee. For early diagnosis of intra-articular pigmented villonodular synovitis, a high level of suspicion is necessary for patients with complaints of persistent pain or swelling in the knee, or erosive osteoarthritis of the hip and shoulder of unknown etiology in young individuals. Arthrography of the knee was not found to be a very helpful test in the diagnosis of pigmented villonodular synovitis. Localized intra-articular pigmented villonodular synovitis should be treated by local excision. Diffuse intra-articular pigmented villonodular synovitis of the knee should be treated by extensive synovectomy followed by manipulation under general anesthesia two to three weeks postsurgery. Intra-articular pigmented villonodular synovitis of the shoulder and hip should be treated by extensive synovectomy or by total joint arthroplasty in cases with marked bone destruction and cyst formation.