Real-world drug utilization and treatment patterns in patients with tenosynovial giant cell tumors in the USA.

Aim: Real-world treatment patterns in tenosynovial giant cell tumor (TGCT) patients remain unknown. Pexidartinib is the only US FDA-approved treatment for TGCT associated with severe morbidity or functional limitations and not amenable to improvement with surgery. Objective: To characterize drug utilization and treatment patterns in TGCT patients. Methods: In a retrospective observational study using IQVIA's linked prescription and medical claims databases (2018-2021), TGCT patients were stratified by their earliest systemic therapy claim (pexidartinib [N = 82] or non-FDA-approved systemic therapy [N = 263]). Results: TGCT patients treated with pexidartinib versus non-FDA-approved systemic therapies were predominantly female (61 vs 50.6%) and their median age was 47 and 54 years, respectively. Pexidartinib-treated patients had the highest 12-month probability of remaining on treatment (54%); 34.1% of pexidartinib users had dose reduction after their first claim. Conclusion: This study provides new insights into the unmet need, utilization and treatment patterns of systemic therapies for the treatment of TGCT patients.

Treatment patterns in patients with tenosynovial giant cell tumors in the USAThis database study is the first investigation of how drugs are used to treat patients with tenosynovial giant cell tumor (TGCT) in the real world. We researched adult TGCT patients from IQVIA's prescription and medical claims databases who started treatment with pexidartinib (N = 82) or other non-US FDA-approved systemic therapies (N = 263). The patients included in this analysis were mostly women (61.0 and 50.6%) and their median age was 47 and 54 years for pexidartinib and other non-FDA-approved systemic therapies, respectively. The patients treated with pexidartinib were most likely to remain on treatment (54.0%) at the end of the first year. Most patients (79.3%) started pexidartinib treatment at a total daily dose of 800 mg/day, as per the product label. Only 34.1% of patients had reduced medication dose during follow-up. Of note, this study found that TGCT patients were treated with other systemic therapies which remain unproven to be safe and effective in medical studies of TGCT. Given the unmet need, and with pexidartinib being the only approved systemic treatment in USA, there is an opportunity for the larger population of adult TGCT patients to benefit from its use. Further research is needed to identify barriers for access to pexidartinib and treatment of TGCT patients.

Knee joint synovial hemangioma treated with arthroscopic resection without hemarthrosis: A case report.

INTRODUCTION: Intra-articular synovial hemangioma of the knee is a relatively rare benign tumor that if left undiagnosed and treated may be followed by degenerative cartilaginous changes and osteoarthritis. However, the non-specific symptoms of synovial hemangiomas limit its early diagnosis. We report our encounter with synovial hemangioma of the knee in which the diagnosis was based on a > 20-year history of chronic pain without joint swelling or hematoma. PRESENTATION OF CASE: A 34-year-old man with a localized vascular malformation on the upper edge of the left patella presented with pain and a restricted range of motion. CT and MRI revealed a tumorous lesion at this location. Upon excision of the lesion, the patient's symptoms disappeared, with no recurrence at the 1-year follow-up. DISCUSSION: Accurate diagnosis and appropriate early treatment are necessary for synovial hemangiomas to forestall articular cartilage degeneration due to recurrent intra-articular hemorrhages. CONCLUSION: Synovial hemangioma should be considered when a patient presents with recurrent knee pain, even in the absence of swelling or episodes of joint effusion.

[Causal relationship between gut microbiota and pigmented villonodular synovitis: a Mendelian randomization analysis].

OBJECTIVE: To investigate the causal relationship between gut microbiota and pigmented villonodular synovitis using Mendelian randomization analysis. METHODS: We conducted a two-sample Mendelian randomization analysis to investigate the causal relationship between 211 gut microbiome taxa and pigmented villonodular synovitis based on GWAS summary data, with inverse variance weighted (IVW) analysis as the primary result and the other methods as supplementary analyses. The reliability of the results was tested using Cochran's Q test, MR-Egger regression, MR-PRESSO method and conditional Mendelian randomization analysis (cML-MA). RESULTS: The increased abundance of Barnesiella (OR=3.12, 95% CI: 1.15-8.41, P=0.025) and Rumatococcaceae UCG010 (OR=4.03, 95% CI: 1.19-13.68, P=0.025) may increase the risk of pigmented villous nodular synovitis, and elevated abundance of Lachnospiraceae (OR=0.33, 95% CI: 0.12-0.91, P=0.032), Alistipes (OR=0.16, 95% CI: 0.05-0.53, P=0.003), Blautia (OR=0.20, 95% CI: 0.06-0.61, P=0.005), and Lachnospiraceae FCS020 group (OR=0.38, 95% CI: 0.15-0.94, P=0.036) and Ruminococcaceae UCG014 (OR=0.36, 95% CI: 0.14-0.94, P=0.037) were all associated with a reduced risk of pigmented villonodular synovitis, which were supported by the results of sensitivity analyses. Reverse Mendelian randomization analysis did not reveal any inverse causal association. CONCLUSION: Increased abundance of specific intestinal microorganisms is associated with increased or decreased risks of developing hyperpigmented villonodular synovitis, and gut microbiota plays an important role in the pathogenesis of this disease.

Unusual presentation of talonavicular joint pigmented villonodular synovitis: a case report.

BACKGROUND: Pigmented villonodular synovitis is a rare yet locally invasive disorder impacting synovial tissues. This case report delineates the atypical manifestation of pigmented villonodular synovitis in the talonavicular joint, detailing its diagnostic complexity and successful management. CASE PRESENTATION: A 56-year-old Iranian patient with a 4-year history of chronic ankle pain, initially diagnosed with degenerative joint disease post-trauma based on imaging, underwent talonavicular fusion surgery. An unexpected pigmented villonodular synovitis mass was encountered during the procedure. Subsequent interventions encompassed tumor resection, talonavicular joint fusion, and allograft bone grafting. Despite the initial intervention, persistent pain and nonunion necessitated a secondary procedure, involving joint surface curettage and autograft bone grafting. At the 12-month follow-up, the patient remained pain-free without tumor recurrence. CONCLUSION: This case report highlights the significance of considering pigmented villonodular synovitis as a crucial differential diagnosis in chronic ankle pain, even when there is evidence of degenerative joint disease and a history of trauma. Magnetic resonance imaging serves a crucial role in accurate diagnosis. Treatment necessitates precise tumor removal, appropriate bone grafting techniques and secure fixation. LEVEL OF EVIDENCE: IV.

Pigmented villonodular synovitis of the flexor hallucis longus tendon: A rare cause of leg pain in a 12-year-old girl.

Pigmented villonodular synovitis is an uncommon benign neoplastic proliferation associated with the synovium, bursa, or tendon sheaths; most commonly occurring in the third to fourth decade of life. It is rare in children and may be painful or painless. Magnetic resonance imaging is the diagnostic study of choice. In this report, the radiologic, ultrasound, and magnetic resonance imaging findings of pigmented villonodular synovitis of the flexor hallucis longus in a 12-year-old girl are discussed. We briefly review the surgical findings as well. To our knowledge, this is the first case report that simultaneously synthesizes the imaging findings of 3 diagnostic imaging modalities for optimal visualization and is the youngest reported case of pigmented villonodular synovitis of the flexor hallucis longus tendon.

Invasion Patterns and Long-Term Clinical Outcomes of Diffuse Tenosynovial Giant Cell Tumor of the Ankle Joint.

BACKGROUND: The invasion patterns and long-term outcomes of diffuse tenosynovial giant cell tumor (D-TSGCT) of the ankle joint remain unclear. METHODS: Seven patients who visited our department between 2011 and 2023 and were diagnosed with D-TSGCT of the ankle joint by contrast-enhanced MRI and a pathological diagnosis were included. The invasion patterns of ankle D-TSGCT on MRI were investigated. The recurrence rate and clinical symptoms were examined in five patients followed up for more than seven years after total resection. RESULTS: In seven patients (1 male/6 females, mean age 37.0±16.6 years, range 15-57 years) with D-TSGCT of the ankle joint, contrast-enhanced MRI at the initial presentation showed invasion within the ankle joint, extending along the tendon sheath, within the talocalcaneal joint, and in the tarsal sinus in 100% of cases, around the deltoid ligament in 86%, within the plantar surface in 43%, invasion of the interosseous membrane in 57%, around the Achilles tendon in 29%, and scalloping on the talocrural joint in 43%. The mean time from mass awareness to the first visit was 51.9±80.0 months (range 1-240 months). Gross total resection, defined as the removal of all tumors as gauged by MRI, was initially performed on 6/7 patients. One patient underwent partial resection of only the anterior part of the tumor. Of the six cases in which gross total resection was performed, 5 had long-term follow-up of more than seven years post-operatively, and one case is still only one year post-operatively. The long-term results of five patients followed for more than seven years after total resection were as follows: a mean follow-up period of 125 months (range 89-171 months), a 100% recurrence rate, a mean time to recurrence of 27.5±19.2 months (range 7-60 months), and a 16% reoperation rate. In the last follow-up, osteoarthritic changes were observed radiographically in 2/5 patients (40%), both of whom had scalloping of the talocrural joint on MRI at the time of the initial diagnosis. Four of the five patients (80%) had no clinical symptoms in the last follow-up. CONCLUSION: Ankle D-TSGCT presents with a strong local infiltrative pattern inside and outside the ankle joint along the tendon sheath, radical resection may be difficult, and the recurrence rate may be higher than previously reported. On the other hand, there are many cases that remain free of clinical symptoms in the long term after recurrence, and surgical indications for ankle D-TSGCT need to consider function preservation as well as recurrence rates.

Combination of manual lymphatic drainage and Kinesio taping for treating pigmented villonodular synovitis: A case report.

BACKGROUND: Pigmented villonodular synovitis (PVNS) is a benign proliferative disorder that affects the synovial joints, bursae, and tendon sheaths. To date, few studies have reported on the treatment of postoperative pain and edema in patients with PVNS. Herein, we present the case of a woman who developed pain and edema in the left lower limb 1 wk after synovectomy and arthroscopic partial meniscectomy and was unable to walk due to limited flexion and extension of the left knee. CASE SUMMARY: A 32-year-old woman underwent synovectomy and arthroscopic partial meniscectomy successively and was treated with a combination of manual lymphatic drainage (MLD) and kinesio taping (KT) in our hospital to alleviate postoperative pain and edema. The following parameters were assessed at 2 wk post-treatment and 1 wk post-discharge follow up: suprapatellar circumference, infrapatellar circumference, visual analog scale score, knee range of motion, pittsburgh sleep quality index score, hamilton anxiety rating scale (HAMA) score, and hamilton depression rating scale (HAMD) score. After treatment, the postoperative pain and edema in the patient's left knee were effectively relieved, resulting in improved sleep quality and remarkably attenuated HAMA and HAMD scores. CONCLUSION: Combined MLD and KT may be an effective approach for relieving postoperative pain and edema in patients with PVNS.

Digitalized diagnosis and treatment of pigmented villonodular synovitis of temporomandibular joint: a case report.

Pigmented villonodular synovitis (PVNS) is a rare locally aggressive benign tumor in the temporomandibular joint (TMJ). This paper presents a patient with TMJ-PVNS involving masseteric space, temporal bone, zygomatic process, and mandibular ramus. Digital technique was used to determine the boundary of the lesion and reconstruct the normal glenoid fossa. The temporalis myofascial flap was transplanted between titanium mesh and condyle to reconstruct the disk after the complete resection of the tumor. The patient's facial profile is symmetrical, with a mouth ope-ning of 43 mm. No local recurrence and complications, such as cerebrospinal fluid fistula and encephaloceles, were detected during 30-month follow-up period.

Case Report: Cytodiagnosis of pigmented villonodular synovitis involving carpal bones of right wrist.

Background: Pigmented Villonodular Synovitis (PVNS) is a rare disease of osteoskeletal tissue. Cytodiagnosis of PVNS on fine needle aspiration (FNA) smears is therefore rarely reported. The PVNS usually affects the larger joints. The involvement of the smaller joints and bones are uncommon. Case presentation: The reported case is one such rarity wherein the diagnosis of PVNS was carried out on the FNAC. The case showed the involvement of all carpal bones except for the pisiform. The 2 nd- 5 th metacarpal bases were also found to be involved in the disease process. The presence of sheets of synoviocytes with brown altered hue to the cytoplasm along with multinucleate giant cells and pigmented macrophages were characteristically present in the smears of FNA. The diagnosis was confirmed on the tissue biopsy. The present case is reported for its unusual multiosteotic involvement of wrist joint bones and the metacarpal bones simultaneously with radiological evidence. The cytomorphology of the lesion in the present case were noteworthy as a learning experience in reporting of PVNS of wrist joint on FNA smears.

Sinovite vilonodular pigmentada da articulação temporomandibular: uma revisão integrativa / Pigmented villonodular synovitis of the temporomandibular joint: an integrative review / Sinovitis villonodular pigmentada de la articulación temporomandibular: una revisión integradora

Objetivo: Avaliar os casos nos quais os pacientes apresentassem SVNP na ATM, incluindo aspectos clínicos, imaginológicos, histopatológicos e tratamento. Metodologia: Trata-se de uma revisão integrativa com dados obtidos nas bases de dados SciELO, PubMed, Medline e Lillacs entre 1982 e 2021, através dos descritores: "Case report", "Temporomandibular Joint", "Pigmented Villonodular Synovitis". Critérios de inclusão: estudos de relato de caso, textos completos disponíveis, idiomas de publicação em inglês, português ou espanhol. Critérios de exclusão: estudos sem presença de aspectos clínicos, relatos não localizados na ATM, artigos de metanálise, revisão sistemática e de literatura, ensaios clínicos, capítulos de livro, dissertações e teses. Dos 156 resultados, apenas 23 compuseram a revisão. Resultados: Como tratamento, a ressecção total através da cirurgia aberta é recomendada. Os sintomas mais comuns foram: dor na mastigação, trismo, dor pré auricular, dormência, parestesia, perca auditiva e inchaço da glândula parótida. Conclusão: Os aspectos imaginológicos revelam erosão de fossa glenóide e côndilo, histopatologicamente, células gigantes com depósito de hemossiderina, e o tratamento recomendado, ressecção via cirurgia aberta com posterior curetagem... (AU)

Objective: To evaluate the cases in which patients presented PVNS in the TMJ, including clinical, imaging, histopathological and treatment aspects. Methodology: This is an integrative review with data obtained from the SciELO, PubMed, Medline and Lillacs databases between 1982 and 2021, using the descriptors: "Case report", "Temporomandibular Joint", "Pigmented Villonodular Synovitis". Inclusion criteria: case report studies, full texts available, languages of publication in English, Portuguese or Spanish. Exclusion criteria: studies without the presence of clinical aspects, reports not located in the TMJ, meta analysis articles, systematic and literature reviews, clinical trials, book chapters, dissertations and theses. Of the 156 results, only 23 made up the review. Results: As a treatment, total resection through open surgery is recommended. The most common symptoms were: chewing pain, trismus, pre-auricular pain, numbness, paresthesia, hearing loss and parotid gland swelling. Conclusion: The imaging findings reveal erosion of the glenoid fossa and condyle, histopathologically, giant cells with hemosiderin deposits, and the recommended treatment, resection via open surgery with subsequent curettage... (AU)

Objetivo: Evaluar los casos en que los pacientes presentaron SVNP en la ATM, incluyendo aspectos clínicos, imagenológicos, histopatológicos y tratamiento. Metodología: Se trata de una revisión integradora con datos obtenidos de las bases de datos SciELO, PubMed, Medline y Lillacs entre 1982 y 2021, utilizando los descriptores: "Caso clínico", "Articulación temporomandibular", "Sinovitis villonodular pigmentada". Criterios de inclusión: estudios de casos clínicos, textos completos disponibles, idiomas de publicación en inglés, portugués o español. Criterios de exclusión: estudios sin aspectos clínicos, informes no localizados en la ATM, artículos de metanálisis, revisiones sistemáticas y de literatura, ensayos clínicos, capítulos de libros, disertaciones y tesis. De los 156 resultados, 23 conformaron la revisión. Resultados: Como tratamiento se recomienda la resección total mediante cirugía abierta. Los síntomas más frecuentes fueron: dolor masticatorio, trismus, dolor preauricular, entumecimiento, parestesia, hipoacusia e inflamación de glándula parótida. Conclusión: Los hallazgos imagenológicos revelan erosión de fosa glenoidea y cóndilo, histopatológicamente células gigantes con depósitos de hemosiderina y el tratamiento recomendado, resección abierta con posterior curetaje... (AU)

Tumor de células gigantes de la bursa de la pata de ganso (bursitis villonodular pigmentada extraarticular): a propósito de un caso / Giant cell tumor of the pes anserine bursa (extraarticular pigmented villonodular bursitis): a case report

Resumen: La sinovitis villonodular pigmentada extraarticular, también llamada tumor de células gigantes de la vaina tendinosa, es frecuente en la mano, siendo extremadamente rara su localización en la rodilla. Se presenta el caso de un paciente con una bursitis villonodular pigmentada de la bursa de la pata de ganso sin afectación intraarticular. Extra-articular pigmented villonodular synovitis, also called giant cell tumor of the tendon sheath, is common in the hand, being extremely rare in the knee. We present the case of a patient with a pigmented villonodular bursitis of the pes anserine bursa without intraarticular involvement.

Abstract: Extra-articular pigmented villonodular synovitis, also called giant cell tumor of the tendon seath, is common in the hand, being extremely rare in the knee. We present the case of a patient with a pigmented villonodular bursitis of the pes anserine bursa without intraarticular involvement.

Bilateral localized pigmented villonodular synovitis of the knee: case report and review / Sinovite vilonodular pigmentada localizada e bilateral dos joelhos. relato de caso e revisão

ABSTRACT Objectives Several cases of bilateral diffuse pigmented villonodular synovitis (PVNS) or tenosynovial giant cell tumor have been described in the literature. Nevertheless, some presentations are rare and differential diagnoses are necessary. Methods The purpose of this study was to perform a systematic review of the literature related to PVNS and to report a rare supra-patellar bilateral and focal presentation. We performed a systematic data review in the Pubmed Clinical Queries database using MeSH and keywords related to PVNS and tenosynovial giant cell tumor. Results Two cases of bilateral and local PVNS had been previously described, but neither was localized in the supra-patellar compartment. To our knowledge, this case report is the first to describe supra-patellar bilateral and localized PVNS of the knee. This case involves a 28 -year-old woman with bilateral localized PVNS of the supra-patellar recess of the knee. MRI showed a low-signal intensity nodule in T1- and T2-weighted images. These were associated with hemosiderin pigmentation. Conclusion The most important finding of the case reported is related to rarity and location. Histopathology analysis confirmed a rare case of hemosiderin pigmentation in the capsular nodule with internal non-pigmented villous content. Lipoma arborescens in the supra-patellar form must be ruled out as a differential diagnosis since it occurs in the same site. Level of Evidence IV; Case series.

RESUMO Objetivos Diversos casos de sinovite vilonodular pigmentada difusa bilateral (SVNP) ou tumor de células gigantes tenossinoviais foram descritos na literatura. Entretanto, algumas apresentações são raras e o diagnóstico diferencial é necessário. Métodos O objetivo do estudo foi realizar uma revisão da literatura relacionada à SVNP e relatar uma apresentação de forma bilateral e localizada rara na região supra-patelar. Foi realizada uma revisão dos bancos de dados do Pubmed Clinical Queries, MeSH e unitermos relacionados com SVNP e tumor de células gigantes tenossinoviais. Resultados Dois casos de SVNP bilateral e local foram descritos anteriormente. No entanto, nenhum deles foi localizado no compartimento supra-patelar. Até onde sabemos, este relato é o primeiro caso descrito de SVNP bilateral localizada supra-patelar. Apresentamos uma mulher de 28 anos com SVNP bilateral no recesso supra-patelar do joelho. A RM mostrou baixo sinal dos nódulos nas imagens ponderadas em T1 e T2, associados ao pigmento hemossiderina. Conclusão O achado mais importante está relacionado à raridade e localização. A histopatologia confirmou um caso raro de pigmento de hemossiderina no nódulo da cápsula com conteúdo viloso não pigmentado internamente. O diagnóstico diferencial com lipoma arborescens na forma supra-patelar é necessário devido à localização comum. Nível de Evidência IV; Série de casos.

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.

Chondroid Tenosynovial Giant Cell Tumor of the Temporomandibular Joint: A Rare Case Report

Abstract Tenosynovial giant cell tumor of diffuse type (TGCT-d) or pigmented villonodular synovitis (PVNS) is a locally aggressive lesion that mostly affects the joints of long bones. Chondroid tenosynovial giant cell tumor (CTGCT) or PVNS with chondroid metaplasia is a rare distinct subset of synovial tumors that has a predilection for the TMJ. We report a rare case of CTGCT in the TMJ, initially misdiagnosed as temporomandibular disorder (TMD). A 51-year-old woman was referred to the surgeon with the chief complaint of TMJ pain for 5 years and a past history of an unsuccessful TMD treatment. Extraoral examination revealed discrete preauricular swelling and restricted mandibular range of motion. Panoramic radiograph and computerized tomography showed destruction of the mandibular fossa and condyle. Histologically, the tumor was composed by large mononuclear cells with prominent eosinophilic cytoplasm and grooved nuclei, small histiocytoid cells, osteoclast-like multinucleated cells, brown pigmentation and areas of chondroid metaplasia. Morphological and immunohistochemical characteristics lead to the final diagnosis of CTGCT. The rarity of CTGCT could be attributed to the lack of recognition of this lesion, with cases diagnosed as chondroblastomas, synovial chodromatosis and chondrosarcoma. The patient received immediate reconstruction and recurrence was found 22 months after initial intervention. TGCT-d and CTGCT of the TMJ can present similar symptoms to TMD, but clinicians must distinguish both lesions by complete examination, imaging and, when necessary, histopathologic evaluation.

Resumo Tumor de células gigantes tenossinovial do tipo difuso (TCGT-d) ou sinovite vilonodular pigmentada (SVP) é uma lesão localmente agressiva que afeta principalmente as articulações dos ossos longos. Tumor de células gigantes tenossinovial condroide (TCGTC) ou SVP com metaplasia condroide é um tipo distinto e raro de tumor sinovial que tem a predileção pela articulação temporomandibular (ATM). Nós relatamos um caso raro de TCGTC da ATM, inicialmente diagnosticado, equivocadamente, como disfunção temporomandibular (DTM). Uma mulher de 51 anos foi encaminhada ao cirurgião com a queixa principal de dor na ATM por 5 anos, e uma história de tratamento de DTM sem sucesso. O exame extrabucal revelou discreto aumento de volume preauricular e movimentação mandibular restrita. A radiografia panorâmica e a tomografia computadorizada evidenciaram destruição da fossa mandibular e côndilo. Histologicamente, o tumor era composto por células mononucleares grandes, com amplo citoplasma eosinofílico e núcleo sulcado, pequenas células histiocitoides, células multinucleadas semelhantes a osteoclastos, pigmentação acastanhada e áreas de metaplasia condroide. As características morfológicas e imuno-histoquímicas levaram ao diagnóstico final de TCGTC. A raridade desta lesão pode estar associada ao seu não reconhecimento, sendo casos diagnosticados como condroblastoma, condromatose sinovial ou condrossarcoma. A paciente recebeu reconstrução imediata e recorrência foi observada 22 meses após a intervenção inicial. TCGT-d e TCGTC da ATM podem apresentar sintomas similares à DTM, mas os clínicos devem diferenciar ambas as lesões por meio do exame clínico completo, exames de imagem e, quando necessário, avaliação histopatológica.

Arthroscopic surgical treatment of pigmented villonodular synovitis of the elbow: case report / Tratamento cirúrgico artroscópico de sinovite vilonodular pigmentada de cotovelo: relato de caso

ABSTRACT This case concerns a male patient complaining of pain and discomfort in his right elbow, associated with decreased range of motion. Elbow radiography showed an osteolytic lesion in the metaphyseal region of the distal humerus and magnetic resonance imaging showed intra-articular tumor formation with swelling that suggested pigmented villonodular synovitis. Arthroscopic treatment was performed in order to obtain a synovial biopsy and then to carry out total synovectomy. Anatomopathological examination confirmed the diagnosis. The patient presented excellent progress through the physiotherapeutic rehabilitation proposed and continued to be asymptomatic 12 months after the operation.

RESUMO O caso diz respeito a um paciente do sexo masculino com queixa de dor e desconforto no cotovelo direito associados a diminuição da amplitude de movimento. Apresentava radiografia do cotovelo com lesão osteolítica da região metafisária do úmero distal e ressonância magnética que mostrava tumoração intra-articular com aumento de volume que sugeria sinovite vilonodular pigmentada. Foi feito tratamento artroscópico para biópsia sinovial e sinovectomia total. O estudo anatomopatológico confirmou o diagnóstico. O paciente apresentou ótima evolução com reabilitação fisioterápica proposta, até 12 meses de pós-operatorio apresentava-se assintomático.

Caracterización imagenológica de la sinovitis vellonodular pigmentada y el tumor de células gigantes de la vaina sinovial / Radiological features of pigmented villonodular synovitis and giant cell tumor of tendon sheath

OBJETIVO: Mostrar los hallazgos imagenológicos en la resonancia magnética (RM) de la sinovitis vellonodular pigmentada (SVP) y el tumor de células gigantes de la vaina sinovial (TCGVS), dado que son entidades que representan un diverso grupo de alteraciones en la proliferación de la sinovial. MATERIALES Y MÉTODOS: Entre mayo de 2011 y junio de 2013, se estudiaron en nuestra institución 25 casos con diagnóstico histológico de proliferación de la sinovial. Se destacaron los distintos tipos de presentación en imágenes a través de una RM 1.5 Tesla. Los resultados fueron analizados y comparados con la literatura. RESULTADOS: La RM mostró características similares para esta patología en todos los pacientes. No obstante, se distinguieron 4 patrones principales de presentación, dependiendo de la morfología, la localización de la lesión y las características radiológicas diferenciales. Estos fueron: como dominante, el tumor de células gigantes de la vaina sinovial (n = 10), todos de localización extraarticular; la sinovitis vellonodular pigmentada de localización bursal (n = 2); la sinovitis vellonodular pigmentada de forma intraarticular focal (n = 5); y la sinovitis vellonodular pigmentada difusa (n = 8). CONCLUSIÓN: La sinovitis vellonodular pigmentada y el tumor de células gigantes de la vaina sinovial se consideran entidades similares desde el punto de vista anatomopatológico. La RM fue de gran utilidad para objetivar tanto las características radiológicas comunes como las diferenciales. Estas últimas, junto con la localización, nos permitieron clasificar 4 patrones de presentación. Su reconocimiento posibilita un adecuado seguimiento de la patología y un óptimo manejo terapéutico.

PURPOSE: To show the resonance magnetic imaging (MRI) findings of pigmented villonodular synovitis (PVNS) and giant cell tumor of the tendon sheath (PVNTS), entities with similar histology but differences in clinical and some radiological manifestations. MATERIALS AND METHODS: We studied 25 cases with histologically benign synovial proliferation in intra and extraarticular location of the extremities. It highlighted with a 1.5T MRI unit the different types of images presentation. The results were analyzed and compared with the literature. RESULTS: MRI displayed very specific imaging features in all patients. However, we were able to distinguish 4 main patterns of presentation depending on the morphology, location of the lesion and radiological differential. These were: as dominant presentation, pigmented villonodular synovitis localized form (n=10); pigmented villonodular synovitis bursal form (n=2); pigmented villonodular synovitis focal (n =5); and pigmented villonodular synovitis diffuse (n = 8). CONCLUSION: Both pigmented villonodular synovitis as well as giant cell tumor of the tendon sheath are considered similar from the point of view of the histological findings. MRI was useful to objectify both radiological features in common, such as the differential, which along with the location, allow us to classify patterns into 4 individual presentations. This recognition involves adequate radiological evaluation and is important for optimal management.

Sinovitis villonodular pigmentada focal: reporte de un caso / Focal pigmented villonodular synovitis: a case report

Pigmented villonodular synovitis represents a benign synovial proliferation associated to hemosiderin deposits, which originates in bone joints, bursae or tendinous sheaths. The knee articulation is the most commonly involved joint (80 percent of the cases), followed by the hip joint. Normally, this pathology has a diffuse presentation, and a small percentage shows a localized form. PVNS displays very specific imaging features, which allows a good diagnostic approach when aided by different radiological techniques, particularly magnetic resonance imaging (MRI). In this report we describe an infrequent case of focal PVNS localized in the hip, which was treated at "Clínica Alemana", Santiago, Chile.

La sinovitis villonodular pigmentada (SVNP) es una proliferación sinovial benigna asociada a depósitos de hemosiderina, que se origina en articulaciones, bursas o vainas tendíneas. La articulación más comprometida es la rodilla (80 por ciento de los casos), seguida por la cadera. Normalmente, esta patología es de presentación difusa y en un menor porcentaje se da en forma focal. La SVNP posee características imaginológicas muy particulares, lo que permite una buena aproximación diagnóstica de la mano de los diferentes métodos radiológicos, en particular la resonancia magnética. En este reporte se describe un caso poco frecuente de SVNP focal en la cadera, que se presentó en la Clínica Alemana de Santiago.

Um caso de sinovite vilonodular do ombro em adolescente: diagnóstico por imagem e anatomopatológico / A case of villonodular synovitis of the shoulder in an adolescent: imaging and pathologic diagnosis

Em quadros de monoartrite crônica devem ser investigadas doenças inflamatórias como a artrite reumatoide (AR), doenças infecciosas como a tuberculose e outras doenças que causem espessamento sinovial e derrame articular como sinovite vilonodular pigmentada (SVNP), hemangioma sinovial, osteocondromatose sinovial e lipoma arborescente sinovial. Relatamos o caso de uma jovem paciente com quadro de monoartrite em ombro, cujo exame por imagem mostrou sinovite e cujo exame histopatológico obtido através de artroscopia com biópsia revelou tratar-se de SVNP. RELATO DO CASO: J C M, 15 anos, sexo feminino, branca, estudante, foi encaminhada ao reumatologista com hipótese diagnóstica de artrite idiopática juvenil (AIJ) pauciarticular. Apresentava, há um ano, dor no ombro D, que melhorava com o uso de anti-inflamatório não-esteroidal (AINE) em dois dias. Teve nesse período de cinco a seis destes episódios que duravam poucos dias. Negava outras queixas articulares ou sistêmicas. Trazia exames normais ou negativos: hemograma, VHS, proteína C reativa, fator reumatoide, sedimento de urina. O FAN era positivo 1/80, pontilhado fino. Trazia Ressonância Magnética do ombro indicativa de sinovite glenoumeral com derrame articular com conteúdo expansivo de tecidos moles na bursa subescapular, segundo o laudo, podendo corresponder a pannus. Como história e exame físico não eram compatíveis com AIJ, foi realizada outra RM que mostrou aumento da lesão já descrita. Foi então encaminhada à artroscopia para biópsia, que revelou SVNP. Durante o procedimento, foi realizada sinovectomia, e uma nova RM feita após nove meses mostrou ausência de sinovite. A SVNP do ombro é incomum e a sinovectomia foi curativa nesse caso.

Chronic monoarthritis demand an investigation of inflammatory diseases, such as rheumatoid arthritis (RA), infectious diseases like tuberculosis; and other diseases that cause synovitis and joint effusion, such as pigmented villonodular synovitis (PVNS), synovial hemangioma, synovial osteochondromatosis and arborescence lipoma. We report the case of a young patient with chronic right shoulder monoarthritis, who's magnetic resonance imaging (MRI) showed synovitis. Arthroscopy was performed and the biopsy revealed PVNS. CASE REPORT: J C M, 15 years-old, female, Caucasian, student. She was sent to a Rheumatologist along with a diagnosis of juvenile idiopathic arthritis (JIA). The patient presented, for one year, a mild pain of insidious onset in her right shoulder, with relief of the symptoms in two days under nonsteroidal anti-inflammatory therapy (NSAIDs). During this year, the patient presented five or six episodes of pain with the same characteristics. No other signs and symptoms were related. The following tests showed normal or negative results:complete blood count, ESR, C-reactive protein, rheumatoid factor and urinalysis. The antinuclear antibody (ANA) was 1/80 speckled pattern. The MRI of the shoulder showed glenohumeral synovitis with joint effusion and soft tissue swelling in the subscapular bursa, which could correspond to pannus. As the medical history and physical examination were not compatible to JIA, a second MRI was performed, which showed an increase of the synovitis. The patient was submitted to an arthroscopy with biopsy and the histopathological examination showed PVNS. A complete synovectomy was performed and a new MRI, nine months later, showed no synovitis. PVNS of the shoulder is uncommon, and synovectomy was curative in this case.

Sinovitis villonodular pigmentada de rodilla: resultado en el tratamiento artroscópico en 10 pacientes / Pigmented villonodular synovitis of the knee: arthroscopic treatment in 10 patients

Objective: To report our experience in the diagnosis, treatment and follow up of patients with clinical and histopathological diagnosis of knee pigmented villonodular synovitis (PVNS). We performed a descriptive retrospective study of a consecutive series of 10 patients treated by knee PVNS in a ten years period. The mean follow-up was 39 months. Clinical records and surgery protocols were reviewed; functional clinical results were documented using the Lysholm score. Results: Nine patients consulted by pain and one case corresponded a incidental finding. All patients were initially treated with an arthroscopic synovectomy. Two patients presented relapse and as a final treatment they underwent total knee arthroplasty. The mean post-operative Lysholm score at follow up was 95 (90-100) points. Conclusion: We documented low incidence of knee PVNS. The total recurrence in our series was of 20 percent.

Objetivo: Reportar nuestra experiencia en el diagnóstico, tratamiento y evolución de pacientes con diagnóstico clínico-histopatológico de sinovitis villonodular pigmentada (SVNP) de rodilla tratados en nuestro centro. Métodos: Estudio retrospectivo, descriptivo, de una serie consecutiva de diez pacientes tratados por SNVP de rodilla en un período 10 años con un seguimiento promedio de 39 meses. Se revisaron fichas clínicas, protocolos quirúrgicos y documentamos los resultados funcionales subjetivos mediante el puntaje de Lysholm. Resultados: Nueve pacientes consultaron por dolor y en un caso correspondió a un hallazgo. A todos los pacientes se les realizó inicialmente una sinovectomía artroscópica. Dos pacientes presentaron recidiva y como tratamiento final se les realizó una artrosplastía total de rodilla. El promedio del puntaje Lysholm postoperatorio consignado durante el seguimiento fue 95 (90-100). Conclusión: La SVNP de rodilla corresponde a una patología de baja frecuencia, que presenta una recidiva de un 20 por ciento tras un tratamiento artroscópico.