Long-term functional outcomes of diffuse pigmented villonodular synovitis of knee: The role of adjuvant radiotherapy.

ABSTRACT: Diffuse pigmented villonodular synovitis (PVNS) of knee is a rare benign disease that has a destructive clinical course. Synovectomy and adjuvant radiotherapy (RT) have been reported as treatment options but literatures reporting functional outcomes were sparse. This study aimed to evaluate the long-term functional outcomes and disease control among treatment modalities through the 22 years of experience.A single-center database was searched for patients who received synovectomy of knee with the pathologic diagnosis of PVNS. General data, treatment modalities, and recurrent status were retrospectively collected from medical records. Functional outcomes were evaluated by Western Ontario and McMaster Universities Osteoarthritis Index through phone interviews by an independent orthopedist.From January 1995 to December 2017, 24 patients with diffuse PVNS of knee were identified, including 19 receiving open synovectomy (OP) and 5 undergoing arthroscopic surgery. Adjuvant RT was performed on 14 patients with a median dose of 35 Gy (range 20-40 Gy). After median follow up of 6 years, recurrences were recorded in 10 cases. The recurrence rate was significantly lower in the OP + RT group than the OP group (8.3% vs 57.1%, P = .038). Among those with preserved knee joints, there was no significant difference in the Western Ontario and McMaster Universities Osteoarthritis Index score and stiffness score between patients in the OP + RT and OP groups.For patients with diffuse PVNS of knee, the addition of moderate-dose adjuvant RT following OP provided excellent local control while maintaining good joint function with limited treatment-related morbidity. Our study emphasized the importance of moderate dose RT in diffuse PVNS of knee joint.

Identification of Patient Needs and Preferences in Pigmented Villonodular Synovitis (PVNS) Using a Qualitative Online Bulletin Board Study.

INTRODUCTION: Pigmented villonodular synovitis (PVNS), also known as giant-cell tumour of the tendon sheath (GCTT), is a rare, benign proliferative tumour affecting the inner lining of synovial joints and tendon sheets. Information on treatment needs of PVNS patients to inform drug development is currently scarce. We conducted an exploratory qualitative study with PVNS patients to generate insights into the objective and emotional aspects related to their medical journey and experiences of living with this disease. METHODS: A 4-day study using an online bulletin board (OBB), an asynchronous, online qualitative research platform, was conducted with patients recruited via physician referral who underwent screening questions to ensure eligibility for the study and willingness to participate. The discussion was moderated, was structured and allowed open answers in response to other participants' posts. RESULTS: Eleven patients (4 from the USA, 4 from the UK and 3 from Canada; 45% female), aged 28-57 years, suffering from PVNS for 2-27 years participated in the study. Key patient insights from the study were: (1) pain was the topmost, spontaneous thought that the participants associated with PVNS, constituting a significant emotional and psychological burden; (2) surgery (arthroscopy) did not completely ameliorate symptoms associated with PVNS, as the relapse rate was high in these patients; (3) PVNS has a substantial negative financial impact on patients, their families and the healthcare system; (4) orthopaedic specialists/surgeons predominantly managed PVNS, as surgery is currently the only therapeutic option. CONCLUSION: PVNS patients expressed an urgent need for a medical drug treatment, which can reduce pain, avoid relapses and provide an alternative to surgery, the current standard of care.

Lowering the recurrence rate in pigmented villonodular synovitis: A series of 120 resections.

OBJECTIVES: Tenosynovial giant-cell tumour or pigmented villonodular synovitis is an aggressive synovial proliferative disease, with the knee joint being the most commonly affected joint. The mainstay of therapy is surgical resection. The aim of this study was to evaluate the main patient characteristics, treatment and outcomes in a large single-centre retrospective study, focusing on meticulous aggressive open surgical procedures. METHODS: From 1996 through 2014, 122 surgical interventions were performed in 105 patients. All patients underwent open synovectomy and when the knee joint was affected, combined anterior and posterior synovectomy. Radiotherapy was applied in 2 patients, radiosynoviorthesis in 27 patients. RESULTS: In histopathology, the diffuse type was seen in 66 (54%) lesions. Two patients were lost during follow-up. At a median follow-up time of 71 months (range: 13-238), 22 (18%) lesions recurred within a median of 18 months, >90% in the first 3 years. Out of those 22 recurrences, 9 (11%) were seen in primary disease and 13 (34%) were a second recurrence. After renewed resection, 6 (5%) out of the 120 resections had persistent tumour at the end of follow-up. Based on the number of patients with complete follow-up (n = 103), this represents 5.8%. CONCLUSION: In diffuse-type pigmented villonodular synovitis, total synovectomy might be difficult to achieve. As shown in our results and also in the literature, meticulous open resection, especially in difficult to approach areas such as the popliteal space, reduces local recurrence rates. External beam radiation is an option in prevention of otherwise non-operable local recurrences or in non-operable disease.

[Pigmented villonodular synovitis. A rare differential diagnosis of synovial joint swelling]. / Pigmentierte villonoduläre Synovialitis. Eine seltene Differenzialdiagnose der synovialen Gelenkschwellung.

BACKGROUND: Pigmented villonodular synovitis (PVNS) describes a rare disease caused by an abnormal proliferation of the synovial membrane in large and small joints. In order to achieve an optimal result of treatment it is necessary to carry out specific diagnostics and a targeted therapy approach. OBJECTIVE: This article gives a review of the epidemiology, etiopathogenesis and diagnostic management of PVNS as well as presenting the current therapy and treatment recommendations. MATERIAL AND METHODS: A systematic search of the literature was performed in the databank of the National Center for Biotechnology Information ( http://www.ncbi.nlm.nih.gov/pubmed ). The search targeted randomized clinical and experimental studies, systematic and non-systematic review articles, expert opinions and case reports related to PVNS, independent of the level of evidence attained by each study. RESULTS: The differential diagnosis of PVNS should be considered in cases of recurrent hemorrhagic joint effusions. The cause of the disease has not yet been exactly clarified. The final diagnosis can ultimately only be confirmed by histological investigations. In order to obtain representative histological tissue samples for the diagnosis, magnetic resonance imaging (MRI) with the appropriate heme sequences should be carried out prior to taking samples. The management of PVNS is often difficult due to the high risk of recurrence depending on the various forms. In view of the high rate of recurrence, therapy should include a complete synovectomy. CONCLUSION: For the surgical approach arthroscopic and open procedures have been described, which are currently controversially discussed with respect to the complication and recurrence rates. Adjuvant interventional therapy forms, such as radiosynoviorthesis are recommended to reduce the recurrence rate.

Pigmented villonodular synovitis: a retrospective multicenter study of 237 cases.

PURPOSE: To review clinical characteristics of pigmented villonodular synovitis (PVNS) in China. METHODS: Electronic medical records (EMR) of four Chinese institutes were queried for patients with histologically proven PVNS between January 2005 and February 2014. Their data were collected including gender, age at diagnosis, clinical presentation, affected site, symptom duration, comorbidities, treatment strategy, recurrence and routine laboratories. RESULTS: A total of 237 patients with biopsy-proven PVNS were investigated. The gender ratio was 1.35 for a female predominance (101 males and 136 females). The average age was 36 years (range, 2 to 83 years). The median delay from initial clinical symptom to diagnosis was 18 months. Main affected areas were the knee (73.84%) and the hip (18.14%). Forty patients had a clear history of joint trauma. Six patients were concurrently diagnosed with PVNS and avascular necrosis (AVN). Five patients suffered from PVNS following implantation of orthopaedic devices including artificial prosthesis, plate and wire. One hundred and twenty-nine patients underwent arthroscopic synovectomy and 108 open synovectomy. Altogether 48 patients (26 males and 22 females) had recurrence of disease. The relapse rate was 24% (knee) and 6.98% (hip), 20.93% (open surgery) and 19.44% (arthroscopy), respectively. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) rate were elevated in 45.83% and 38.41% of the patients respectively. CONCLUSIONS: To our knowledge, this study is the largest sample size of PVNS patients reported as well as the largest sample of PVNS with concurrent AVN reported to date. Our outcomes suggest that PVNS shows a female predominance, occurs mostly between 20-40 years and favors the knee and hip. Recurrence is frequent, particularly in the knee. Serum ESR and CRP may be elevated in some patients. Additionally, the present study supports the theory of an association between PVNS and orthopedic surgery, which is not limited to joint replacement.

Surgical outcomes after excision of pigmented villonodular synovitis localized to the ankle and hindfoot without adjuvant therapy.

Although a benign disorder, pigmented villonodular synovitis (PVNS) has a high rate of recurrence. Because of the high incidence of recurrence and concern about destruction of the affected joint, several adjuvant therapies have been promoted without a clear standard treatment strategy. We reviewed cases of PVNS affecting the ankle and hindfoot joints (ankle and/or subtalar joints) treated with surgical resection without adjuvant therapy in an effort to identify the incidence of PVNS recurrence after excision without adjuvant therapy. Of the 10 cases with a mean follow-up duration of 33.2 ± 19.8 months, 4 (40%) developed a recurrence, with a mean interval of 6 (range 3 to 14) months. At the final follow-up visit, the mean American Orthopaedic Foot and Ankle Society ankle-hindfoot score was 86.6 ± 12. The clinical outcomes of PVNS affecting the ankle and hindfoot joints are associated with a relatively high incidence of recurrence, and additional clinical investigation comparing the incidence of recurrence in patients undergoing excision versus excision with adjuvant therapy is needed for us to better understand this condition and provide more informed recommendations to our patients.

[Pigmented villonodular synovitis]. / La synovite villonodulaire.

Pigmented villonodular synovitis (PVNS), also known as tenosynovial giant cell tumour is an articular pathology that occurs predominantly in young adults and is caused by an abnormal proliferation of the synovial membrane. The clinical presentation includes pain and joint swelling. MRI represents the best imaging modality to investigate this disease but the histopathology of synovial tissue provides the definitive diagnosis. The management of PVNS is often difficult due to the high risk of relapse after treatment. The objective of this article is to review the literature regarding the diagnosis and therapy of this poorly understood condition.

[Temporomandibular joint primitive tumors and pseudo tumors]. / Pseudotumeurs et tumeurs primitives de l'articulation temporomandibulaire.

The temporomandibular joint (TMJ) can be the site of bone, cartilaginous, or synovial tumors. There is no well-defined histological classification. We listed all benign tumors, malignant primitive tumors, and rare pseudo tumors of the TMJ. We provide a list to help for the diagnosis and the differential diagnosis of non-tumoral lesions by far the most frequent.

[The role of external beam radiation therapy in the adjuvant treatment of pigmented villonodular synovitis]. / Stellenwert der externen Radiotherapie bei der adjuvanten Behandlung der pigmentierten villonodulären Synovitis.

AIM: Pigmented villonodular synovitis (PVNS) is a rare proliferative disorder arising from synovial cells of the tendon sheets and joint capsules. The potential value of external beam radiation therapy in the interdisciplinary management of PVNS is demonstrated by a comprehensive literature review on the clinical use of radiotherapy and the results of national patterns of care study (PCS) which was conducted by the German Cooperative Group on Radiotherapy in Benign Diseases (GCG-BD) in 2008-2009. MATERIAL AND METHOD: A structured questionnaire was mailed to all 227 RT institutions in Germany to assess all previous treatments, the RT indication and techniques, the rate of local control, the functional outcome and the possible adverse effects related to the use of external beam radiation therapy (RT). For comparison of the clinical outcome data, a systematic literature research in several international electronic databases and a conventional library search were performed to identify publications addressing the use of RT for PVNS. RESULTS: Based on an overall response rate of 83.2%, the PCS was nationally representative. Ten percent of institutions presented clinical experience with the use of RT for PVNS; from this database a total of 41 treated sites from 14 institutions were evaluable for long-term analysis. The primary therapeutic approach was cytoreductive surgery in all cases. In cases of residual tissue or complete resection of extensive local recurrences, RT was applied in 39 cases (95.1%). An excellent or good functional outcome was noted in 34 cases (82.9%). The use of RT was not associated with early or late toxicity larger than RTOG grade II. The literature review identified 19 published studies (1940-2009) which represented a total of 140 cases or patients, respectively. After follow-up periods ranging from 1-250 months and administration of total doses in the range of 16-50 Gy the overall rate of local control was 84.5%. CONCLUSION: Both the results of the national PCS and the literature review demonstrate that RT is a very safe and effective treatment option for the prevention of disease progression or recurrence in PVNS after primary surgical interventions. The planned treatment volume should include the whole synovial space and eventually all invasive components of the disease. Currently, total doses in the range of 30-36 Gy are recommended.

A case of co-existing pigmented villonodular synovitis and tuberculosis infection of the foot and ankle.

A woman presented with chronic left ankle pain and swelling with no systemic upset. Magnetic resonance imaging suggested pigmented villonodular synovitis (PVNS) of her left ankle joint and flexor hallucis longus tendon. Combined arthroscopic and open synovectomy was performed. Histological examination of the synovium confirmed the diagnosis of PVNS, but culture of the diseased synovium yielded mycobacterium tuberculosis. This case illustrates the possibility of double pathology and the importance in excluding tuberculosis infection.

Sinovitis vellonodular pigmentada de rodilla en pacientes esqueleticamente inmaduros / Pigmented villonodular synovitis of the knee in skeletally inmature patients

Introducción: La sinovitis vellonodular pigmentada(SVNP) es un trastorno proliferativo idiopático benignoque ocasiona vellosidades o formaciones nodulares en articulaciones, tendones y bolsas serosas. Es una patología infrecuente en el grupo etario pediátrico, por lo que su diagnóstico suele ser tardío. En este trabajo se analizan los métodos de tratamiento utilizados y los resultados funcionales obtenidos en el tratamiento de esta patología. Materiales y métodos: Se analizaron todas las SVNP de rodilla tratadas entre enero de 1988 y junio de 2006. Se estudió la forma de presentación, la demora diagnóstica, el diagnóstico previo, el tipo de tratamiento, las recidivas y la necesidad de tratamientos posteriores. Los hallazgos radiográficos se clasificaron de acuerdo con el sistema propuesto por Flandry y para la evaluación clínica se utilizóel Oxford Knee Score. Resultados: Se evaluaron 9 pacientes con un promedio de edad de 9,8 años. El seguimiento promedio fue de 8,5 años. Cuatro se presentaron en forma difusa, 4 en forma intraarticular y 1 extraarticular. Sólo en 3 casos se observaronhallazgos radiográficos preoperatorios. La demoradiagnóstica fue de 18 meses promedio. La resección serealizó a cielo abierto en 7 casos y artroscópica en 2 casos. El 78 por ciento de los pacientes evidenciaron una función articular satisfactoria en el último control. No hubo recidivas luego de la resección. Conclusiones: La resonancia magnética (RM) es de gran utilidad para la orientación diagnóstica y para determinar la extensión de la lesión. La cirugía es el tratamiento de elección. Los peores resultados funcionales se relacionaron directamente con el tiempo de demora diagnóstica

Sinovitis vellonodular pigmentada de rodilla en pacientes esqueleticamente inmaduros / Pigmented villonodular synovitis of the knee in skeletally inmature patients

Introducción: La sinovitis vellonodular pigmentada(SVNP) es un trastorno proliferativo idiopático benignoque ocasiona vellosidades o formaciones nodulares en articulaciones, tendones y bolsas serosas. Es una patología infrecuente en el grupo etario pediátrico, por lo que su diagnóstico suele ser tardío. En este trabajo se analizan los métodos de tratamiento utilizados y los resultados funcionales obtenidos en el tratamiento de esta patología. Materiales y métodos: Se analizaron todas las SVNP de rodilla tratadas entre enero de 1988 y junio de 2006. Se estudió la forma de presentación, la demora diagnóstica, el diagnóstico previo, el tipo de tratamiento, las recidivas y la necesidad de tratamientos posteriores. Los hallazgos radiográficos se clasificaron de acuerdo con el sistema propuesto por Flandry y para la evaluación clínica se utilizóel Oxford Knee Score. Resultados: Se evaluaron 9 pacientes con un promedio de edad de 9,8 años. El seguimiento promedio fue de 8,5 años. Cuatro se presentaron en forma difusa, 4 en forma intraarticular y 1 extraarticular. Sólo en 3 casos se observaronhallazgos radiográficos preoperatorios. La demoradiagnóstica fue de 18 meses promedio. La resección serealizó a cielo abierto en 7 casos y artroscópica en 2 casos. El 78 por ciento de los pacientes evidenciaron una función articular satisfactoria en el último control. No hubo recidivas luego de la resección. Conclusiones: La resonancia magnética (RM) es de gran utilidad para la orientación diagnóstica y para determinar la extensión de la lesión. La cirugía es el tratamiento de elección. Los peores resultados funcionales se relacionaron directamente con el tiempo de demora diagnóstica.

[Pigmented villonodular synovitis]. / Synovite villonodulaire.

OBJECTIVE: To evaluate clinical, radiological and histological characteristics as well as outcome of pigmented villonodular synovitis (PVNS) in 14 patients. METHODS: This retrospective study examined data from 14 cases of pathologically-confirmed PVNS over a 10-year period (1990-1999). RESULTS: All 14 cases (9 women and 5 men) of PVNS involved the knee. Mean age at diagnosis was 32.7 years. The principal functional signs were mechanical pain and disability. All patients had radiographs (normal for 8 patients); 3 had arthrography, which found diffuse cyst formations, 2 had MRI, which showed images characteristic of PVNS, and 4 had arthroscopy. All patients underwent complete surgical excision, and 4 also had osmic acid synoviorthesis. Recurrence occurred in 4 patients, within 10 months on average. CONCLUSION: MRI and arthroscopy improve the early management of pigmented villonodular synovitis.

[Pigmented villonodular synovitis of the hip. Results of a national survey apropos of 58 cases]. / La synovite villo-nodulaire pigmentée de la hanche. Résultats d'une enquête nationale à propos de 58 observations.

Pigmented villonodular synovitis is an uncommon synovial disease which only rarely involves the hip. In a multicenter retrospective study, we identified 58 histologically-proven cases. There were 33 females and 25 males. Mean age at diagnosis was 38 years. In all but two cases, only one hip was involved; the right hip was affected somewhat more often (33 cases) than the left. Two patients probably had bilateral hip disease. Mean delay to diagnosis was four years. Pain was the presenting symptom in most cases. A palpable mass in the groin was found in six patients. Plain roentgenograms were considered normal in only three patients. Bony cysts were seen in 39 patients and kissing cysts in 19. Joint space narrowing was found in 40 patients and was diffuse in half the cases. Roentgenograms suggested pigmented villonodular synovitis in 63% of cases, osteoarthritis of the hip in 16%, and inflammatory hip disease in 14%. Additional imaging studies included opaque arthrography in 21 subjects, computed tomography in 23, magnetic resonance imaging in 11, and arthroscopy in 9. Initial treatment was osmic acid synoviorthesis in 14 patients, partial synovectomy in 9, and total synovectomy in 21; in addition, eight patients had insertion of a cup prosthesis and 13 had total arthroplasty of the hip. Treatment was successful in 65% of cases after a mean follow-up of three years; among the 35% of failures, there were seven recurrences (14%). Total hip arthroplasty was performed secondarily in nine patients. This study illustrates the diversity of roentgenological changes in pigmented villonodular synovitis of the hip and the high frequency of osteoarticular lesions precluding conservative treatment. Magnetic resonance imaging and/or arthroscopy should be used to establish the diagnosis at an early stage when conservative treatment with total synovectomy and synoviorthesis is most likely to be successful.

Pigmented villonodular synovitis in a child.

The authors present a case of pigmented villonodular synovitis (PVNS) in an adolescent with monarticular involvement of the ankle and without congenital anomalies or sibling involvement. Its rarity in the ankle and in childhood is discussed; a review of the clinical, radiological, and pathological features of PVNS is presented.

[Pigmented villonodular synovitis of the shoulder joint in childhood]. / Pigmentierte villonoduläre Synovitis des Schultergelenkes im Kindesalter.

A case of a 5-year-old boy with a pigmented villonodular synovitis of the shoulder is described in this article for the first time. The clinical features, preoperative diagnostic imaging including MRI and treatment were explained and compared with the literature.

[Hemopigmented villonodular synovitis]. / La synovite villonodulaire hémopigmentée.

Pigmented Villonodular Synovitis (PVNS) in an infrequent tumoral like disease and there are only a few MRI studies published. Concerning our series of five cases compared with the literature, the readers attention is drawn to the etiologies still under debate and to the respective contribution of the different imaging methods. MRI known for its high sensibility, but also for its low specificity in tumoral pathology, has turned out to be, in the case of PVNS, quite remarkable in both regards. The RMI aspect is quite well correlated to the histological structure of this synovial hyperplasia and to its evolution: highly vascular mass at the beginning and then low cellular density stroma, fibrous, with deposition of hemosiderin. In our five cases, as in almost all those previously reported in the literature, MR imaging shows heterogeneous areas of decreased signal intensity on T2 weighted sequences and on two of our cases after administration of gadolinium. Still the same MR aspects can be found in rheumatoid, hemophilic arthritis, as well as synovial chondromatosis, and therefore the clinical background and findings as well as plain films become essential. MR imaging should be the first examination to be undertaken after plain films, leading in most cases to a precise local preoperative assessment.