Case Of Pigmented Villonodular Synovitis Research Articles

E56 A 16-year diagnosis delay of a patient with Pigmented Villonodular Synovitis

Abstract Introduction Pigmented villonodular synovitis (PVNS), a subtype of tenosynovial giant cell tumours, is a rare non-cancerous proliferative synovial disorder usually affecting the knee in adults(1). The incidence rate is 1.8 patients per million people of the general population, with few reports amongst the paediatric population, hence it is frequently misdiagnosed(2). It has a slow onset of symptoms and has been noted to take ∼18 months to make a definitive diagnosis(3). Objective To describe a case of pigmented villonodular synovitis in a seventeen-year-old African female. Methods A retrospective chart review was done to ascertain the clinical presentation and management of the patient. Case description We present a case of a 17-year-old African female who presented with bilateral knee pain and swelling from 10 months of age. She had been on follow-up at a peripheral facility for 14 years, where she was being managed with methotrexate 12.5 mg weekly and folic acid 5 mg OD for Juvenile Idiopathic Arthritis with no marked improvement in her symptoms. In view of her poor response to therapy, the mother stopped her treatment 3 months prior to presentation in our facility. She initially had intermittent bilateral swelling of the small joints of the hand that were tender which eventually evolved to involve both knee joints. The pain was worse in the morning and was associated with the inability to ambulate and occasional night awakening. The systemic inquiry was otherwise unremarkable. She had an unremarkable birth history, and normal developmental milestones. She was however shorter than her peers and had not attained menarche. The patient had been admitted 8–10 times per year during the span of her illness for similar complaints and had undergone multiple knee joint fluid aspirations to relieve the swelling and several laboratory tests and radiological studies were done. On assessment, she was noted to be of fair general condition with gross asymmetric swelling of both knees and a surgical scar over the right knee with no limitation in the range of movement. There was minimal effusion of bilateral elbows and ankles, and the MCP, ICP and wrist joints weren’t affected. The patient had thoracic scoliosis, a grade 3/6 systolic murmur on the left lower sternal border, no radiation, and hepatomegaly 4 cm below the costal margin. She was prepubertal on sexual maturation rating. There was no central nervous system, abdominal, or respiratory abnormalities. A series of lab investigations to exclude the possibility of juvenile idiopathic arthritis were conducted. This panel included a complete blood count, which showed hypochromic, microcytic anaemia, c-reactive protein was raised at 61 mg/l but normal biochemistry and unremarkable autoantibody profile. Imaging done included a bilateral knee X-Ray followed by a bilateral knee MRI which showed large volume bilateral knee joint effusions associated with synovial-based masses causing extrinsic periarticular bony erosions and intra-articular synovial proliferation forming a villonodular appearance. The patient was referred to orthopaedic surgery and bilateral arthrotomy, drainage and synovectomy of both knees were done. She continues to be followed up in our paediatric rheumatology and orthopaedic clinic with good mobility and no other co-morbidities. She is currently undergoing work up for a delayed puberty. Conclusion Pigmented villonodular synovitis is a rare disease in the paediatric population. In the absence of awareness of its existence and expertise on how to diagnose it, patients may be misdiagnosed and mismanaged over a long duration of time as seen in the presented case.

Anterior Capsule Reconstruction in the Setting of PVNS

Category:Ankle; OtherIntroduction/Purpose:Pigmented villonodular synovitis (PVNS) is a rare benign proliferative disease affecting tendon sheaths and synovial tissue. Most cases of PVNS arise in the hip and knee joints, while < 5% of total cases occur in the foot and ankle. PVNS of the ankle has a high rate of recurrence and can be destructive to the affected joints if incompletely removed. This case series analyzes functional outcomes after PVNS surgical resection and anterior ankle capsular reconstruction, using a novel technique.Methods:This case series included three patients who underwent surgery between 2010 and 2020 for their ankle pain and swelling caused by PVNS. The surgical technique involved a posterior, midline approach for PVNS resection of the affected ankle joint, followed by a standard anterior approach for capsular excision. Subsequent anterior capsular reconstruction was performed with a regenerative tissue matrix and a bioresorbable anchoring system. Preoperative and postoperative range of motion testing for the ankle and subtalar joints were assessed along with appropriate radiographs and MRI imaging.Results:All three surgical procedures for PVNS of the ankle resulted in successful recovery and promising clinical outcomes. The mean cohort age was 43.7 (R:36-58) years and follow-up period was 52.3 (R:4-123) months. Preoperative ankle dorsiflexion and plantarflexion along with subtalar inversion and eversion range of motion were all normal. Postoperative ankle motion and subtalar motion were recorded at final follow up and found to be unchanged. No complications or recurrence of PVNS occurred.Conclusion:This case series demonstrates that PVNS surgical resection and anterior ankle capsular reconstruction is a safe and effective treatment modality that allows for maintenance of pre-operative motion. Further investigation is warranted to better understand the clinical outcomes of this surgical technique on PVNS recurrence.

The Role of Early Temporomandibular Joint Arthroscopy in the Detection, Diagnosis, and Surveillance of Synovial Neoplasms: A Massachusetts General Hospital Experience and Algorithm of Care

PurposeSynovial neoplasms that affect the small joints, such as the temporomandibular joint (TMJ), are uncommon.1 Of the neoplasms that affect the TMJ, pigmented villonodular synovitis (PVNS), synovial chondromatosis (SC), and juxta-articular myxoma are the most prevalent. To date, there have been only 115 and 360 reported cases of PVNS and SC of the TMJ in the literature, respectively.2 These lesions are locally destructive with the capacity to invade the cranial base and often demonstrate an inconspicuous growth pattern, a high degree of recurrence, and presentation that is confounded with other forms of temporomandibular joint disorder. TMJ arthroscopy has been established as a minimally invasive surgical modality to establish diagnosis and reduce patient morbidity. The purpose of the present study was to evaluate the role and impact of TMJ arthroscopy in the early detection, diagnosis, treatment, and surveillance of PVNS and SC of the TMJ. Patients and methodsThis is a retrospective case series. Inclusion criteria were patients with synovial neoplasms: specifically, PVNS and SC at Massachusetts General Hospital. Exclusion criteria were patients with malignant tumors involving the TMJ and cranial base and tumors arising from extra-articular, non-synovial origin. Patients were categorized into 2 groups: Group I (who did not undergo early TMJ arthroscopy prior to lesion excision) and Group II (who did undergo early TMJ Level II [multi-port] arthroscopy prior to lesion excision). Pre-operative time to definitive lesion excision surgery, pre-operative biopsy status, extent of surgery (arthroscopic removal vs. open arthrotomy with or without craniotomy), and lesion recurrence were evaluated. ResultsA total of 6 patients were included in the present study (3 men and 3 women), all unilateral involvement for a total of 6 sides. The mean age was 31 (15-61). Patients were categorized into 2 groups: Group I-3 PVNS patients identified. All patients did not have a pre-operative biopsy prior to lesion excision. Mean pre-operative time duration to definitive lesion removal from initial presentation was 12 months. One isolated open arthrotomy and 2 open arthrotomy with craniotomy were required due to tumor growth and invasion of the middle cranial fossa. Recurrence of the lesion was present in 2 cases requiring repeat excision. Group II consisted of 3 patients: 2 PVNS (Figure 1) and 1 SC (Figure 2). All patients had pre-ablative biopsy diagnosis yielded from arthroscopic synovial biopsy. Mean pre-operative time duration to definitive lesion removal from initial presentation was 7 months. One PVNS case was excised arthroscopically. One isolated open arthrotomy and 1 open arthrotomy with craniotomy were required for definitive lesion excision. No lesion recurrence was noted, with 1 second-look arthroscopy conducted. ConclusionThe results from the present study demonstrated that the early TMJ arthroscopy has efficacy in the early detection, diagnosis, and surveillance of synovial neoplasms PVNS and SC. Visualization and synovial biopsy of the lesion provide diagnostic and surgical planning benefit in conjunction with standard pre-operative workup. One lesion was arthroscopically removed. No recurrence has occurred, and TMJ arthroscopy proffers a minimally invasive means for disease surveillance if recurrence is suspected. Larger studies with continued follow-up are needed to further establish the algorithm established by this work to continue to minimize patient morbidity with lesion excision and reduce recurrence rates.

Postoperative radiotherapy for pigmented villonodular synovitis (PVNS): case series and literature review

Abstract Purpose: Pigmented villonodular synovitis (PVNS) is a rare benign proliferative disease of the synovium with locally aggressive behaviour. We reviewed our experience using external beam radiotherapy (RT) in the treatment of PVNS. Method: We report five cases of PVNS who underwent Arthroscopic Synovectomy followed by postoperative RT in National Oncology centre in Oman. The total dose RT ranges between 30 and 36 Gray (Gy) Three-dimensional radiotherapy technique. Conclusion: Postoperative RT is effective in preventing disease recurrence and should be offered following maximal cytoreduction to enhance local control in PVNS.

Pediatric solid intra-articular masses of the knee: prevalence, imaging features and etiologies.

The etiologies of pediatric solid intra-articular soft-tissue masses are not well described and can present diagnostic dilemmas. Our hypothesis was that these entities have a limited differential diagnosis and are mostly benign. We reviewed knee magnetic resonance imaging (MRI) scans performed at our tertiary care institution between 2001 and 2019 (n=3,915). Our inclusion criterion was knee MRI with a solid intra-articular soft-tissue mass. Our exclusion criteria were cases with no masses or non-solid intra-articular lesions with clear radiologic diagnoses. Multiple radiologic characteristics were evaluated. Radiologic and histological diagnoses were collected when available. Cases without histology were assigned a suspected diagnosis. Twenty-five out of 3,915 (<1%) cases met the inclusion/exclusion criteria. Twenty patients underwent biopsy. Patient age ranged from 1 to 19years with a mean age of 12years. Lesion size ranged from 1 to 7cm with a mean of 3.3cm. The top three histological diagnoses were pigmented villonodular synovitis (PVNS) (7/20, 35%), vascular malformations (2/20, 10%) and inflammatory arthritis (2/20, 10%). There was one malignant case of synovial sarcoma (1/20, 5%). Of the five cases without biopsies, PVNS was the most common diagnosis (3/5, 60%). Five out of 10 (50%) PVNS cases were focal and 5/10 (50%) were multifocal. All PVNS cases (10/10, 100%) had hypointense signal on T2. Solid intra-articular soft-tissue masses of the knee in children are rare, with a prevalence of <1% in our study. The vast majority are benign with PVNS being the most common diagnosis.

99mTc-MDP 3-Phase Bone Imaging in 3 Cases of Pigmented Villonodular Synovitis.

Pigmented villonodular synovitis (PVNS) is a rare proliferative synovial benign disorder, which is characterized by villonodular hyperplasia of joints, tendon sheath, and synovium; invasion of adjacent tissue; and sometimes visible hemosiderin deposition. Studies regarding bone scan findings of PVNS were relatively limited. Here, we report our findings on 99mTc-MDP 3-phase bone scan with SPECT/CT images on delayed phase in 3 patients with joint PVNS.

Pigmented Villonodular Synovitis Mimicking an Acute Septic Hip in an Eight-Year-Old Male

Pigmented villonodular synovitis (PVNS) is a less common but known cause of joint pain in the adult population. PVNS in pediatric patients is even more rare, with only case reports of occurrence in persons under the age of 18 years. Presentation is typically that of more insidious pain and limited range of motion, and is primarily seen in the knee joint. Diagnosis can be suspected with imaging, but ultimately surgical intervention is needed for tissue confirmation. We present a case of PVNS in a pediatric patient with acute symptoms concerning for a septic joint. The patient’s workup revealed a large effusion on hip ultrasound, with operative intervention pursued and further imaging deferred given the patient's symptom burden. A 4 × 1 × 1.5 cm intra-articular pigmented mass excised from the synovium in the operating room. The patient’s symptoms improved after the procedure, with pathology showing sheets of plump mononuclear cells in a collagenized stroma with hemosiderin deposits, confirming the diagnosis. This case highlights the importance of keeping non-infectious etiologies in the differential diagnosis of acute onset joint pain.

Effectiveness of tibio-talo-calcaneal arthrodesis with full thread headless compression screws via combined minimal incisions

To investigate the effectiveness of tibio-talo-calcaneal arthrodesis with full thread headless compression screws via combined minimal incisions. Between January 2012 and December 2016, 36 patients (36 feet) with ankle diseases underwent tibio-talo-calcaneal arthrodesis with parallel double thread headless compression screws via minimal anterior and lateral oblique incisions. There were 14 males and 22 females with an average age of 53.8 years (range, 18-76 years). There were 19 cases of left feet and 17 cases of right feet. There were 21 cases of talar necrosis, 7 cases of post-traumatic arthritis, 3 cases of rheumatoid arthritis, 2 cases of tuberculosis infection (inactive), 1 case of talar absence, 1 case of Charcot’s disease, and 1 case of pigmented villonodular synovitis of ankle and subtalar joints. Preoperative American Orthopaedic Foot and Ankle Society (AOFAS) score and visual analogue scale (VAS) score were 53.7±2.5 and 5.9±0.2, respectively. The operation time was recorded and the wound healing and complications were observed. The bone healing was assessed by X-ray film and CT scanning. The function and pain of joint were evaluated by AOFAS and VAS scores. The mean operation time was 49.8 minutes (range, 33-82 minutes). Incision infection occurred in 1 patient (2.8%) at 3 weeks after operation, and recovered after debridement. The other incisions healed by first intention without complications. Thirty-five patients were followed up with an average of 18.5 months (range, 12-29 months). Imaging examination showed fusion of the ankle and subtalar joints with an average fusion time of 10.9 weeks (range, 8-15 weeks). After 1 year, the AOFAS score (84.7±0.6) and VAS score (0.3±0.1) were significantly higher than preoperative scores ( t=12.596, P=0.000; t=30.393, P=0.000). It is an effective surgical method of tibio-talo-calcaneal arthrodesis with full thread headless compression screws via combined minimal incisions for end-stage ankle disease because of the less complications incidence and the higher postoperative fusion rate.

The predictive value of preoperative neutrophil-lymphocyte ratio (NLR) on the recurrence of the local pigmented villonodular synovitis of the knee joint

BackgroundTo explore and evaluate the predictive value of preoperative Neutrophil-lymphocyte ratio (NLR) on the recurrence of pigmented villonodular synovitis (PVNS) of the knee joint treated by arthroscopic surgery combining local radiotherapy.MethodsSixty pathological-proven PVNS cases of the knee joint in our department from April 2006 to March 2017 were included. All of them are treated by arthroscopic synovectomy combined with adjuvant radiotherapy. The pre-operative hematological indexes such as c-reactive protein (CRP), erythrocyte sedimentation rate (ESR), NLR, Platelet-lymphocyte ratio (PLR) and Lymphocyte-monocyte ratio (LMR) were collected retrospectively and their relationship with postoperative recurrence was analyzed by using univariate and multivariate analysis, the receiver operating characteristic curves (ROC curve), the Kappa correspondence test and the Mc Nemar Chi-square test.ResultsAll 60 patients were followed up for a median of 52.8 months (7–138 months) and the recurrence rate is about 23.3% (14/60). There is a significant difference in NLR between the recurrent and non-recurrent group (P = 0.002). It had a certain correlation with postoperative recurrence (correlation coefficient r = 0.438, P = 0.001). The optimal thresholds in ROC curve were 2.42 (sensitivity 71.4%, specificity 78.3% respectively). which had predictive ability for recurrence after arthroscopic treatment.ConclusionThe preoperative NLR is an easy and cost-effective predictor for relapse in PVNS of the knee joint after the arthroscopic surgery combined with local radiotherapy, which is of profound significance to guide clinical work.

Pigmented Villonodular Synovitis of the Foot and Ankle

Category: Other Introduction/Purpose: Pigmented Villonodular Synovitis (PVNS) is a benign but potentially locally aggressive condition, that results in an increased proliferation of synovium, causing villous or nodular changes of synovial-lined joints and tendon sheaths. The estimated incidence of PVNS is 1.8 new cases annually per million people, of which 2.5% occur in the foot and ankle, reflected by the limited reports of PVNS in the foot and ankle literature. We present our clinical experience of 76 patients presenting with PVNS about the foot and ankle. Methods: Patients with PVNS of the foot and ankle were identified from our tertiary referral hospital patient database from 2007 to 2017. The case notes were carefully reviewed to analyse the clinical, radiological, and histopathological features and to investigate their clinical behaviour, management and the factors influencing recurrence. Results: 76 patients were identified (32 male, 44 female) with a mean age of 39 years and mean follow-up of 5 years. The majority of cases were of localised, nodular PVNS (52%, 40/76) with the diffuse PVNS cases (48%, 36/76) presenting most commonly in the hindfoot. The most common presentation was of a swelling/lump (78%- 62/79), of which pain was an associated feature in 11% (7/62) and restricted range of motion a feature in 10% (6/62). Osteoarthritic change and cortical erosions were more commonly associated with diffuse PVNS. All patients underwent open synovectomy with a recurrence rate of 11% (7/76), all of which were the diffuse type. In those cases where synovectomy was paired with arthrodesis of the joint, there were no cases of recurrence (12%,9/76). Conclusion: We present our tertiary referral experience of PVNS in the foot and ankle as the largest series in the foot ankle ankle literature to date. A high index of suspicion is required in diagnosing PVNS. Nodular PVNS appears more common in the foot whereas diffuse PVNS more common in the ankle and hindfoot. Open synovectomy yields low recurrence rates. Although theorised, there is insufficient evidence to establish whether concurrent arthrodesis of the affected joint reduces risk of recurrence.

Histopathological Audit of Arthroscopic Synovial Biopsies Done for Persistent Knee Effusions

This study aims to educate medical practitioners on possible synovial disoders that may be the cause of persistent knee pain and recurrent effusions. Histopathological audit of arthroscopic synovial biopsies done for persistent knee effusions. Monoarticular knee pain and swelling has a plethora of differential diagnoses. In South Africa tuberculosis is a common treatable cause that needs to be excluded. The presence of multiple comorbidities and unremarkable septic markers may pose a diagnostic challenge. This necessitates the need for synovial biopsy. We review the clinical presentation and histopathological diagnoses of patients who had arthroscopic knee synovial biopsies for persistent undiagnosed knee effusions. A retrospective review of patients who had knee arthroscopy was done. Those who had diagnostic arthroscopy and synovial biopsy done were included in the study. Comorbidities, blood results (white cell count, C-reactive protein and erythrocyte sedimentation rate) and histopathological diagnoses were recorded. Descriptive statistical analysis of continuous and categorical variables was perfomed. Out of 194 arthroscopies, 19 patients had biopsy done. Only 10 histopathology results were found. Mean age was 40.6 SD 10.3 years (range 24-55 years). All cases presented with knee effusion and pain. Duration of symptoms ranged from 1-8years with a median of 3.25 years (IQR 1.25-6.5). Two patients were documented HIV positive with one having co-existing systemic lupus erythematosus. One had previous tuberculosis treatment and there was one rheumatoid arthritis patient on treatment. Mean white cell count was 6.13x10?/L (SD 1.57x10?) with range 4.56-8.60x10?. Median ESR was 17mm/hr (IQR 15-19) and CRP range was 3-20mg/L. Histopathology revealed 3 cases of pigmented villonodular synovitis (30%), 2 cases of synovial lipomatosis (20%) and 1 each of mycobacterium tuberculosis, rheumatoid arthritis, connective tissue disease, chronic synovitis and combined necrotising and non-necrotising granulomatous inflammation. Diagnostic arthroscopies are overshadowed by the need for therapeutic intervention for ligament or meniscal repair. However it has a role in clinching a diagnosis in cases of long standing knee effusions that pose a diagnostic dilemma. The majority of patients had biopsy done mainly to rule out tuberculous infection. These were patients with persistent knee pain and effusions despite treatment with DMARDs in the setting of coexistent immunosuppression. This study reveals a wide range of synovial disorders that may be the underlying cause of chronic knee pain and effusions. PVNS and synovial lipomatosis were the leading histopathological diagnoses. Arthroscopic synovial biopsy is reliable in reaching a histopathological diagnosis in cases of persistent undiagnosed painful knee effusions.

Results and recurrence of pigmented villonodular synovitis of the ankle: does diffuse PVNS with extra-articular extension tend to recur more often?

The aim of this study was to provide the outcomes and to analyze the recurrence of Pigmented Villonodular Synovitis (PVNS) of the ankle joint treated by surgical synovectomy. Thirty-one PVNS cases of the ankle, including 5 localized PVNS cases and 26 diffuse PVNS cases, were treated in our institute between 2004 and 2015. The median age was 35 (range 18-63) years. The 5 localized PVNS cases were treated with partial synovectomy (group I); 10 diffuse PVNS cases limited to intra-articular synovium received arthroscopic comprehensive synovectomy (group II); and 16 cases with diffuse PVNS spreading to extra-articular tendon sheaths underwent combined arthroscopic and open synovectomy (group III). Adjuvant radiotherapy was provided in groups II and III. The American Orthopaedic Foot and Ankle Society (AOFAS) score and subjective grading of procedure were used to evaluate the results. Twenty-seven patients were followed with a median of 54 (range 15-108) months. In the three groups, the average AOFAS score improved from 75 (in all three groups) points preoperatively to 100 (group I), 97 (group II), and 90 points (group III) postoperatively. The rate of good-to-excellent result was 100% in group I and II and 73.3% in group III. Only in group III, recurrence occurred in five cases. Diffuse PVNS of the ankle can be successfully managed with surgical synovectomy and adjuvant radiotherapy. Radiotherapy is not needed for localized PVNS. The recurrence rate in PVNS patients with extra-articular extension is higher. IV.

Villonodular synovitis of the knee: about 20 cases

La synovite villonodulaire pigmentée (SVNP) est une prolifération bénigne rare de la synoviale des articulations, des bourses séreuses et des gaines tendineuses, d'étiopathogénie inconnue. Notre travail porte sur 20 cas de SVN du genou colligés à l'hôpital militaire Avicenne de Marrakech sur une période de 9 ans allant de janvier 2000 au décembre 2009 Il vise à identifier les spécificités de cette lésion, et à étudier ses principaux aspects anatomocliniques et pronostiques. L'incidence annuelle était de 2,2 cas par an. Ils étaient 15 hommes et 5 femmes, d'âge moyen de 32,5 ans, atteints du coté droit dans 55%des cas sous un mode mono articulaire chez 18 patients et bi articulaire chez un seul. La douleur et la tuméfaction étaient présentes dans 80% des cas, une masse palpable dans un cas, un syndrome méniscal a été retenu dans un cas, une mono arthrite septique dans 3 circonstances de même qu'un kyste poplité dans 2 autres. L'atteinte était diffuse dans 14 cas (70%), localisée dans 6 cas. L'imagerie par résonnance magnétique(IRM) pratiquée chez 5 patients était évocatrice chez 3, l'arthroscopie diagnostique a été utilisée chez 2 malades. La confirmation s'est faite à chaque fois à l'examen anatomopathlogique. Le traitement a consisté en une synovectomie subtotale dans 15 cas et en l'exérèse de la tumeur dans les autres formes localisées, 2 cas présentant une destruction ostéocartilagineuse ont nécessité une arthroplastie. L'évolution a été marquée par la survenue de 2 récidives sous la forme diffuse avec un recul de 3, 7 ans. On a noté une raideur avec atrophie quadricipitale chez 3 patients et une arthrolyse a été réalisée. Un cas de SVN confirmé par l'histologie s'est révélé être 5 mois après la synovectomie totale un Synovialosarcome monophasique envahissant l'os d'où l'indication de l'amputation.

Radiotherapy inducing total knee prosthetic component loosening for recurrent pigmented villonodular synovitis following joint replacement

Pigmented villonodular synovitis (PVNS) following total knee arthroplasty (TKA) is a rare condition. We reported a case of PVNS following TKA; initially, arthroscopic synovectomy was performed; however, recurrence of PVNS happened. We performed second arthroscopic synovectomy and moderate dose radiotherapy (total dose 44 cGy/22 fractions to posterior fossa, 36 cGy/18 fractions to knee) for the patient. Unfortunately, distal femur osteonecrosis with femoral and tibial component loosening happened 2 months after completing the radiotherapy procedure. Revision TKA with Legacy Constrained Condylar Knee was done. Radiotherapy is not recommended for recurrent PVNS after TKA.

A case of Pigmented Villonodular Synovitis (PVNS) of the Knee joint in a young female

Pigmented villonodular synovitis (PVNS) is a rare, benign, but potentially locally aggressive and recurrent condition characterized by synovial proliferation and hemosiderin deposition inside the joints, tendon sheaths, and bursae. It usually affects the large joints such as hip, knee, and ankle. The present report was a case of PVNS of the knee joint in a young female which was treated by subtotal synovectomy alone without the use of adjuvants. At the 20-month follow-up, the patient was pain free and had no signs of disease recurrence.

MRI and thallium features of pigmented villonodular synovitis and giant cell tumours of tendon sheaths: a retrospective single centre study of imaging and literature review.

The purpose of this study was to characterize the MRI and thallium-201 ((201)TI) scintigraphy attributes of pigmented villonodular synovitis (PVNS) and giant cell tumours of tendon sheaths (GCTTS). The epidemiology of these uncommon lesions was also assessed and less commonly encountered pathology reported on including multifocality, necrosis and concurrent malignancy. A retrospective single centre review of MRI and (201)TI scintigraphy findings for 83 surgically proven or biopsy-proven consecutive cases of PVNS was undertaken. Radiological findings including lesion size, (201)TI uptake (as a marker of metabolic activity), location, extent and patient demographics were correlated with biopsy and surgical specimen histology. Typical appearances are described, as well as less common imaging manifestations. The study period encompassed all patients presenting or referred to a tertiary bone and soft-tissue tumour referral centre with PVNS or GCTTS between 1 January 2007 and the 1 December 2013. Lesions occur most commonly around the knee joint in the fourth decade of life, with younger patients showing a tendency to occur in the hip. Features of PVNS and GTTS include bone erosion, ligamentous and cartilage replacement, muscle infiltration and multifocality. MR signal characteristics were variable but post-contrast enhancement was near-universal. 14 of 83 cases showed no uptake of (201)TI and revealed a statistically significant smaller average axial dimension of 19.8 mm than lesions displaying active (201)TI uptake of 36.4 mm, p = 0.016. Four lesions demonstrated central necrosis on gross histology, two of each from both the (201)TI-avid and (201)TI-non-avid groups. MR is the imaging modality of choice when considering the diagnosis of these uncommon tumours. (201)TI scintigraphy as a marker of metabolic activity further adds minimal value although small lesions can appear to lack (201)TI avidity. This article depicts typical imaging findings of PVNS/GCTTS and also a subset of lesions that demonstrate no uptake on metabolic functional imaging, namely smaller sized lesions irrespective of anatomical location. This represents an important departure from previously documented imaging manifestations, whereby an absence of isotope accumulation suggested exclusion of these lesions from the differential diagnosis. These findings have important implications when considering the diagnosis of these uncommon lesions and may be important when interpreting post-treatment response. We suggest that further investigation, for example, with MRI is valuable in order to clarify potential post-treatment response, as well as the use of alternate functional imaging modalities such as positron emission tomography (PET), to further corroborate these findings.

Expression of colony-stimulating factor 1 is associated with occurrence of osteochondral change in pigmented villonodular synovitis.

Pigmented villonodular synovitis (PVNS) is a benign, translocation-derived neoplasm. Because of its high local recurrence rate after surgery and occurrence of osteochondral destruction, a novel therapeutic target is required. The present study aimed to evaluate the significance of protein expression possibly associated with the pathogenesis during the clinical course of PVNS. In 40 cases of PVNS, positivity of colony-stimulated factor 1 (CSF1), its receptor (CSF1R), and receptor activator of nuclear factor kappa-B ligand (RANKL) were immunohistochemically determined. The relationship between the positivity and clinical outcomes was investigated. High positivity of CSF1 staining intensity was associated with an increased incidence of osteochondral lesions (bone erosion and osteoarthritis) (p = 0.009), but not with the rate of local recurrence. Positivity of CSF1R and RANKL staining was not associated with any clinical variables. The number of giant cells was not correlated with positivity of any of the three proteins, or with the clinical outcome. Focusing on knee cases, CSF1 positivity was also associated with the incidence of osteochondal change (p = 0.02). CSF1R positivity was high in cases which had local recurrence, but not significantly so (p = 0.129). Determination of CSF1 and CSF1R expression may be useful as a prognosticator of the clinical course and/or outcomes of PVNS.

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 Presenting as a Popliteal Cyst.

Introduction:Pigmented villonodular synovitis is an uncommon disease characterized by hyperplastic synovium, large effusions and bone erosions. It commonly mimics other conditions. As a result, the diagnosis may remain elusive, as the classic signs of tenderness and effusion are not specific. Occasionally, PVNS presents as a popliteal cyst, which may divert attention from intra-articular pathology. In this article, we describe a case of pigmented villonodular synovitis that presented with a popliteal cyst as the chief problem.Case Report:A twelve year old boy presented to us with a history of a gradually enlarging mass in the left popliteal fossa since eighteen months. He complained of restriction of terminal knee flexion, but he had no pain. After clinical examination and imaging the mass was interpreted as being either a popliteal cyst or chronic hematoma without ruling out the possibility of soft tissue sarcoma. An open excisional biopsy of the lesion was performed which revealed a reddish-brown cystic-nodular mass communicating with the knee joint. The histology was consistent with that of pigmented villonodular synovitis. We performed an arthroscopic synovectomy 3 weeks later. Abnormal synovium found in all compartments of the knee was removed. Histology from the intra-articular lesion was also consistent with a diagnosis of pigmented villonodular synovitis.Conclusion:A swelling in the popliteal region may be due to various etiologies. The cause should be thoroughly investigated. Along with clinical examination and imaging the tissue must be subjected to histopathological examination. Pigmented villonodular synovitis can rarely present as a popliteal cyst. In such a situation, treatment consists of dealing with intra-articular and extra-articular pathology.

Pigmented villonodular synovitis

Pigmented villonodular synovitis