A severe headache due to pigmented villonodular synovitis in a rare location: facial nerve on temporomandibular joint. A case report.

BACKGROUND: Pigmented villonodular synovitis (PVNS) is an infrequent benign tumor-like proliferative lesion developing from the synovial membranes of the joint, tendon sheath, and bursa. CLINICAL PRESENTATION: A 44-year-old woman with numbness on the right side of her face, severe headaches, and swelling in temporomandibular region is presented. On head and neck magnetic resonance imaging, an encapsulated mass approximately 2 cm was detected. The fine-needle aspiration biopsy resulted as suspicion of mesenchymal tumor. A complete resection with the capsule was performed over the temporal branch by monitoring of the facial nerve. The final histopathologic examination resulted as a giant cell tendon sheath tumor. CONCLUSION: Headache is not the main symptom in PVNS, but in severe pain spreading from the temporomandibular region, physical examination should be done carefully for slight swelling, and the possibility of pigmented villonodular synovitis should be considered. Because of the high recurrence rate, en bloc resection is necessary.

Arthroscopic treatment of diffuse pigmented villonodular synovitis of the elbow.

INTRODUCTION: Pigmented villonodular synovitis (PVNS) is a rare pathology of the elbow, but presents a risk of progression with cartilage destruction. Surgical treatment consists in synovectomy, as complete as possible. Arthroscopy is an excellent tool for the exploration and treatment of intra-articular lesions in the elbow, but the results in PVNS of the elbow have never been evaluated. The aim of this study was to assess the recurrence rate of PVNS of the elbow after arthroscopic synovectomy, and secondarily to assess pain, joint range of motion, functional scores and complication rate. MATERIAL AND METHODS: We performed a retrospective study of a continuous series of 8 patients operated on between February 2012 and February 2019, with a mean age of 43.7 years. The operated side was the dominant side in 75% of cases. Surgery consisted in the most complete synovectomy possible, performed arthroscopically. Recurrence, clinical evaluation, with pain at rest and on mpvement on visual analogue scale (VAS) and joint range of motion, functional MEPS and DASH scores, and any complications were collected. RESULTS: At a mean 66.4 months' follow-up, 2 patients required revision surgery for recurrence. At the last follow-up, VAS for pain at rest was 0.9 and 1.9 for pain on movement. MEPS score was 85.6 and DASH score 82.2. No neurological, vascular or infectious complications of arthroscopic synovectomy were found in our series. DISCUSSION: Arthroscopic synovectomy in the treatment of PVNS of the elbow was a reliable and safe therapeutic alternative, with a low complications rate and 2 cases of recurrence (25%) in our study. This was the first study to report the results of arthroscopic surgical treatment of elbow PVNS. LEVEL OF EVIDENCE: IV Retrospective study without control group.

Two-stage TKA for tuberculosis septic arthritis of the knee masquerading as pigmented villonodular synovitis: A case report.

A 25-year-old female presented with left knee pain following arthroscopic synovectomy for presumed pigmented villonodular synovitis (PVNS). Radiographs and magnetic resonance imaging demonstrated tricompartmental arthritic changes. She underwent a two-stage procedure first involving antibiotic spacer implantation, followed 1 week later by spacer removal and definitive total knee arthroplasty (TKA) once initial intraoperative culture results were negative. Subsequent cultures confirmed tuberculosis septic arthritis. Repeat evaluation 1 year postoperatively showed no complications and patient satisfaction with left knee function. This is a unique case report in the United States describing 1-year outcomes following staged TKA for tuberculosis septic arthritis masquerading as PVNS.

An abrupt-onset shoulder joint subluxation and pseudoparalysis caused by intraarticular pigmented villonodular synovitis: A case report.

Pigmented villonodular synovitis (PVNS) occurs most frequently in the knee and hand joints and is extremely rare in the shoulder joints. A 27-year-old women was admitted to our outpatient clinic with an abrupt-onset pain and limited range of motion of the left shoulder. On arthroscopic examination, localized PVNS surrounding the reactive synovitis in the posterior aspect of the subscapularis within the shoulder joint cavity. The mass was removed using a grasper and shaver. Symptomatic relief was achieved following surgical removal. In conclusion, although rare, localized PVNS of the shoulder joint should be kept in mind in patients with a sudden-onset shoulder joint subluxation and pseudoparalysis.

[Rheumatoid arthritis combined with pigmented villonodular synovitis: A case report and literature review].

We reported a case of rheumatoid arthritis (RA) combined with pigmented villonodular synovitis (PVNS) from Peking University People's Hospital. The clinical data were introduced and the related literature was reviewed. The clinical features, treatment and prognosis of the disease were summarized to improve clinicians' understanding of this rare disease and avoid misdiagnosis and delayed diagnosis. A 45-year-old female, with 15 years of RA history and unregular treatment, was admitted to the hospital with the complaint of aggravating pain and swelling in the right knee for 3 months. The puncture of the right knee was performed and there was a large amount of bloody synovial fluid that could not be explained by her RA history. Moreover, the magnetic resonance imaging (MRI) of the right knee revealed hemosiderin deposition with low-intensity signals on both T1-weighted and T2-weighted images which suggested PVNS to us. Then, the patient underwent knee arthroscopy and biopsy to assist in diagnosis. The arthroscopic appearance and pathology were consistent with PVNS and the hyperplastic synovium was removed during arthroscopy. After the operation, she did active functional exercises and took disease-modifying antirheumatic drugs to control RA. She recovered well and remained asymptomatic after half a year of follow-up. Also, there was no recurrence of the right knee. As we all know, RA is a systemic autoimmune disease characterized by chronic synovitis and joint damage. And PVNS is characterized by synovial proliferation and infiltrative process. Both of them are synovial involvement and the clinical manifestations are quite similar. PVNS has occasionally been reported in association with RA. So it is difficult to make a clear diagnosis of RA combined with PVNS. Literature was searched with RA+PVNS in the WanFang Medical Network Database and China National Knowledge Infrastructure and there were no related Chinese cases. Then we searched literature from PubMed with RA+PVNS. The cases were still rare and eventually 2 related articles were yielded including 2 similar patients. It is necessary to fully understand the disease development, complicated MRI appearance and various pathological morpho-logy. They can contribute to making a correct diagnosis which is effective to guide the proper treatment.

The diabetic knee.

Diabetes is a multisystemic and multifaceted syndrome, and its clinical impact is wide ranging and varied. The musculoskeletal complications of diabetes, too cover a broad spectrum of joints and presentations. Relatively less attention is paid, however, to the diabetic knee. This communication describes the bidirectional relationship between diabetes and the knee, and the potential influence this may have on therapy.

Localized pigmented villonodular synovitis with hemorrhage arising from lateral meniscocapsular junction: A case report.

Pigmented villonodular synovitis (PVNS) is a benign tumorous condition commonly found in tendon sheathes, bursa, or joint synovium. Unlike the diffuse type which invades the entire synovium of the affected joint, synovium of localized PVNS shows relatively normal appearance. It presents nonspecific symptoms and typically progresses for a long time but acute locking phenomenon or internal derangement of knee symptoms suddenly commence in early stage. In this article, we present a 48-year-old female patient with well-capsulated localized PVNS with intra-capsular hemorrhage occurring from the junction of the mid-body of lateral meniscus and the lateral joint capsule in the knee. It expanded and then moved to the lateral joint space, which caused pain, limitation of knee flexion and locking spontaneously. Arthroscopic complete excision, biopsy, and focal synovectomy were performed with punch and motorized shaver. Full weight-bearing with full knee range of motion was allowed at one day post-surgery. The mechanical symptom of locking and severe pain disappeared. At the clinical follow-up one month post-surgery, the symptoms were no longer present.

Pigmented Villonodular Synovitis Presenting as Unilateral Hearing Loss: Review of the Literature and Case Report.

BACKGROUND/AIMS: To review the existing literature on pigmented villonodular synovitis (PVNS) of the temporomandibular joint (TMJ) and report a rare case of PVNS of the TMJ presenting with unilateral hearing loss. METHODS: Review of the existing literature and a description of personal experience with PVNS of the TMJ presenting with unilateral hearing loss. RESULTS: Review of the existing literature revealed 76 reported cases of PVNS of the TMJ. The most common presenting symptom was of a slowly enlarging mass or swelling of the preauricular area, with dysfunctional TMJ also frequently reported. All patients underwent surgical excision with some pursuing radiation as adjuvant therapy. Presented Patient: A 46-year-old man presented with several months of unilateral subjective hearing loss and aural fullness. Imaging revealed a mass centered along the superior TMJ with expansion through the squamous temporal bone and extra-axial intracranial extension into the middle cranial fossa. Imaging characteristics and fine-needle aspiration biopsy were consistent with PVNS. INTERVENTION: The patient underwent near-total excision of the mass via frontotemporal craniectomy and lateral temporal bone resection. FOLLOW-UP: At the 16-month follow-up there was no evidence of disease recurrence. CONCLUSION: PVNS of the TMJ represents a rare entity that can present with a variety of symptoms including unilateral hearing loss.

Pigmented villonodular synovitis occurring in the temporomandibular joint.

OBJECTIVE: Pigmented villonodular synovitis occurring in the region of the temporomandibular joint is a rare disease, requiring a review of the treatment method, follow-up period. METHOD: Refer to the past literature, along with a retrospective search. RESULTS: An excision, including the skull base bone, was performed in all cases; however, recurrence was found in one case on which fractional excision was performed. Past reports have also indicated that en bloc resection was considered desirable. CONCLUSION: It is necessary to perform en bloc resection on patients with pigmented villonodular synovitis occurring in the region of the temporomandibular joint. Furthermore, due to reported cases of recurrence after a long period of time, follow-up observations of about 10 years are considered necessary.

Complications After Total Knee Arthroplasty in Patients With Pigmented Villonodular Synovitis.

BACKGROUND: Pigmented villonodular synovitis (PVNS) is a locally destructive histiocytic proliferation most commonly occurring in the knee. Extensive local joint destruction can indicate the need for a total knee arthroplasty (TKA). The objective of this study is to evaluate PVNS of the knee as a risk factor for complication after TKA. METHODS: Patients who underwent TKA with a diagnosis of PVNS of the knee from 2007 to 2016 were identified in a national private payer insurance database. Complication rates for emergency room visits, readmission, revision, stiffness, infection, and death were calculated and compared to a control population of patients who received TKA for osteoarthritis (OA). RESULTS: Four hundred fifty-three patients were diagnosed with PVNS of the knee and underwent TKA during the time period and compared with a matched control cohort of 1812 patients who underwent TKA for OA. The rate of revision TKA at 2 years, emergency room visits, readmission, and death did not differ between the PVNS group and the control cohort. The PVNS group had stiffness at 1 year compared to the OA group (6.84% vs 4.69%, odds ratio 1.48, P = .023). The infection rate at 2 years was 3.31% in the PVNS group and 1.55% in the OA group (odds ratio 1.73, P = .011). CONCLUSION: The complication rates for TKA in patients with a diagnosis of PVNS of the knee have not been previously demonstrated. These patients have a higher rate of stiffness and infection when compared to a control cohort, so they may have a more complicated postoperative course.

Pigmented Villonodular Synovitis of the Knee: An Underappreciated Source of Pain in Children and Adolescents.

BACKGROUND: Pigmented villonodular synovitis (PVNS) is a benign proliferative synovial disorder most commonly described in adults. The purpose of this study is to describe the presentation and management of a large single-center series of pediatric patients with PVNS of the knee. METHODS: A retrospective review was performed of consecutive pediatric patients treated for PVNS at a single institution from 2001 to 2016. Inclusion criteria were patients below 20 years of age with surgical treatment of histologically proven PVNS of the knee. Review of the electronic medical record was utilized to gather demographic data, clinical presentation, affected joint, imaging findings, treatment, and disease persistence. RESULTS: Over the 16-year study period, 17 pediatric patients with an average age of 11 years (range, 3 to 19 y) were treated for PVNS of the knee. The average duration of symptoms before orthopaedic evaluation was 16 months. More than half of the children were misdiagnosed with a variety of rheumatologic or orthopaedic conditions before their diagnosis of PVNS. On the basis of magnetic resonance imaging (MRI), PVNS was included as a potential diagnosis in 12 of 17 patients (71%). A total of 27 surgeries were performed in 17 patients. Ten patients were treated with a single surgery and 2 patients were treated with staged biopsy followed by synovectomy. Five patients required ≥2 therapeutic surgeries. The majority of procedures were arthroscopic synovectomies (89%). One patient required open resection of a mass in the posterior compartment of the knee and 1 patient had a manipulation under anesthesia for arthrofibrosis. Ten patients were noted to have nodular disease, 4 diffuse, and 3 mixed. At an average 15-month follow-up, 15 patients (88%) were considered to be disease-free based on clinical examination and/or follow-up MRI. Two patients (12%) had MRI studies following their last surgery with findings consistent with persistent PVNS, but only a single patient had persistent symptoms. CONCLUSIONS: This case series of pediatric patients with PVNS of the knee represents the largest single-center cohort in the literature. Although previously considered a disease of young adult patients, PVNS should be considered in pediatric patients with an insidious onset of joint swelling with or without pain. Symptoms may be present for months to years before diagnosis. Patients are frequently misdiagnosed, most commonly with rheumatologic diseases, bleeding disorders, or septic arthritis. MRI with gradient echo sequences is the diagnostic imaging study of choice and arthroscopic synovectomy produces good outcomes with low rates of symptomatic disease persistence. LEVEL OF EVIDENCE: Level IV.

Pigmented villonodular synovitis in pediatric population: review of literature and a case report.

BACKGROUND: Pigmented villonodular synovitis (PVNS) is a rare proliferative process in children that mostly affects the knee joint. CASE PRESENTATION: The study follows the case of a 3-year-old boy presenting recurrent patellar dislocation and PVNS. Due to symptoms such as chronic arthritis, he had been taking prednisolone and methotrexate for 6 months before receiving a definitive diagnosis. After a period of showing no improvements from his treatment, he was referred to our center and was diagnosed with local PVNS using magnetic resonance imaging (MRI). The patient was treated for his patellar dislocation by way of open synovectomy, lateral retinacular release, and a proximal realignment procedure, with no recurrence after a 24-month follow-up. CONCLUSION: PVNS may appear with symptoms resembling juvenile idiopathic arthritis, thus the disease should be considered in differential diagnosis of any inflammatory arthritis in children. PVNS may also cause mechanical symptoms such as patellar dislocation. In addition to synovectomy, a realignment procedure can be a useful method of treatment.

Diffuse Pigmented Villonodular Synovitis as a Rare Cause of Graft Failure Following Anterior Cruciate Ligament Reconstruction.

This case report describes a 42-year-old woman who was diagnosed with pigmented villonodular synovitis (PVNS) in the knee. The patient had received a bone-patella tendon-bone autograft reconstruction of her anterior cruciate ligament (ACL) 22 years prior to her diagnosis of PVNS. After a traumatic event that tore her ACL graft, she underwent a second surgery to repair the initial reconstruction. However, her pain and joint instability remained unresolved. When radiolucent lesions in her tibia and femur were identified through a radiographic image, the patient was referred to the authors' orthopedic oncology clinic. Additional imaging, including magnetic resonance imaging, revealed PVNS, and she was scheduled for debridement and a complete synovectomy of the knee. After surgery, the patient's pain decreased dramatically. She continues to maintain an active lifestyle despite a relatively minor decrease in range of motion. In this case, PVNS proved to be an unlikely complication after ACL reconstruction. The patient remains at risk for the development of degenerative arthritis. [Orthopedics. 2018; 41(1):e142-e144.].

Presentación atípica de sinovitis villonodular pigmentada de la articulación coxofemoral. Reporte de un caso y revisión de la literatura / Unusual presentation of pigmented villonodular synovitis of the hip joint: case report and review of the literature

Resumen: Introducción: La sinovitis villonodular pigmentada (SNVP) es una entidad clínica poco frecuente, con un incidencia mayor en las mujeres (3:1) y una edad de presentación entre los 20 y 40 años. La cadera es un sitio poco común de ocurrencia, estando involucrada en tan solo 15% de todos los casos. Reporte de caso: Masculino de 47 años con cuadro de 10 años de evolución caracterizado por limitación de los arcos de movilidad, dolor asociado a aumento de volumen y acortamiento del miembro pélvico; los estudios de imagen mostraron destrucción de la articulación coxofemoral y extensión a la pelvis; el reporte histopatológico describió sinovitis villonodular pigmentada. Fue manejado con hemipelvectomía externa izquierda. Discusión: La presentación de la sinovitis villonodular pigmentada difusa en la cadera tiene una incidencia baja y comportamiento localmente agresivo. Es necesario tener en cuenta diagnósticos diferenciales que incluyan neoplasias malignas. El estándar de tratamiento es quirúrgico.

Abstract: Introduction: Pigmented villonodular synovitis (PVNS) is a rare clinical entity with higher incidence in women (3:1) and an age of presentation between 20 and 40 years. The hip is a rare site of appearance, being involved in only 15% of all cases. Case report: 47-year-old male with 10 years of evolution characterized by a limited range of motion, pain associated with an increase in volume and shortening of the pelvic limb. Imaging studies showed destruction of the hip joint and extension to the pelvis. The histopathology report described pigmented villonodular synovitis. His final treatment was left hemipelvectomy. Discussion: The presentation of the diffuse pigmented villonodular synovitis of the hip has a lower incidence and a locally aggressive behavior. It is necessary to consider differential diagnoses that include malignant neoplasms. The standard treatment is surgical.

Localized pigmented villonodular synovitis in a child knee.

Pigmented villonodular synovitis is a benign proliferative tumor of the synovium. It is very rare, and most cases occur in the knee joint. In this article, we report a case of localized pigmented villonodular synovitis in the knee joint of a 14-year-old boy. This condition is rare in the knees of the children. We preferred to remove the tumor with arthrotomy instead of arthroscopy for two reasons: (i) the patient was obese, (ii) we thought that recurrence risk was high after arthrotomy.

Osteonecrosis of the femoral head associated with pigmented villonodular synovitis.

A 23-year-old Japanese woman with no history of corticosteroid intake or alcohol abuse presented with a 10-month history of left hip pain without any antecedent trauma. An anteroposterior radiograph performed 10 months after the onset of pain showed slight joint space narrowing and bone erosions surrounded by sclerotic lesions in both the acetabular roof and femoral neck. Magnetic resonance images of the left hip showed a feature of osteonecrosis of the femoral head and a mass with villus proliferation extending from the posterior intertrochanteric area to the anteromedial aspect of the femoral neck. In addition, the left quadratus femoris muscle, which is generally located just above the nutrient vessels of the femoral head, was not detected. Based on these findings, the patient was diagnosed with osteonecrosis of the femoral head caused by impairment of the nutrient vessels from invasion of the pigmented villonodular synovitis. She underwent radical synovectomy of the left hip 16 months after the onset of pain, and her hip pain improved after the surgery.

Multifocal tenosynovial giant cell tumors in a child with Noonan syndrome.

Noonan syndrome is a genetic disorder with variable expression of distinctive facial features, webbed neck, chest deformity, short stature, cryptorchidism and congenital heart disease. The association of Noonan syndrome and giant cell granulomas of the mandible is widely reported. However, Noonan syndrome may also be associated with single or multifocal tenosynovial giant cell tumors, also referred to as pigmented villonodular synovitis. We report a child with Noonan syndrome, giant cell granulomas of the mandible and synovial and tenosynovial giant cell tumors involving multiple joints and tendon sheaths who was initially misdiagnosed with juvenile idiopathic arthritis. It is important for radiologists to be aware of the association of Noonan syndrome and multifocal giant cell lesions, which can range from the more commonly described giant cell granulomas of the mandible to isolated or multifocal intra- or extra-articular tenosynovial giant cell tumors or a combination of all of these lesions.

[Unusual presentation of pigmented villonodular synovitis of the hip joint: case report and review of the literature]. / Presentación atípica de sinovitis villonodular pigmentada de la articulación coxofemoral. Reporte de un caso y revisión de la literatura.

INTRODUCTION: Pigmented villonodular synovitis (PVNS) is a rare clinical entity with higher incidence in women (3:1) and an age of presentation between 20 and 40 years. The hip is a rare site of appearance, being involved in only 15% of all cases. CASE REPORT: 47-year-old male with 10 years of evolution characterized by a limited range of motion, pain associated with an increase in volume and shortening of the pelvic limb. Imaging studies showed destruction of the hip joint and extension to the pelvis. The histopathology report described pigmented villonodular synovitis. His final treatment was left hemipelvectomy. DISCUSSION: The presentation of the diffuse pigmented villonodular synovitis of the hip has a lower incidence and a locally aggressive behavior. It is necessary to consider differential diagnoses that include malignant neoplasms. The standard treatment is surgical.

INTRODUCCIÓN: La sinovitis villonodular pigmentada (SNVP) es una entidad clínica poco frecuente, con un incidencia mayor en las mujeres (3:1) y una edad de presentación entre los 20 y 40 años. La cadera es un sitio poco común de ocurrencia, estando involucrada en tan solo 15% de todos los casos. REPORTE DE CASO: Masculino de 47 años con cuadro de 10 años de evolución caracterizado por limitación de los arcos de movilidad, dolor asociado a aumento de volumen y acortamiento del miembro pélvico; los estudios de imagen mostraron destrucción de la articulación coxofemoral y extensión a la pelvis; el reporte histopatológico describió sinovitis villonodular pigmentada. Fue manejado con hemipelvectomía externa izquierda. DISCUSIÓN: La presentación de la sinovitis villonodular pigmentada difusa en la cadera tiene una incidencia baja y comportamiento localmente agresivo. Es necesario tener en cuenta diagnósticos diferenciales que incluyan neoplasias malignas. El estándar de tratamiento es quirúrgico.