Giant cell tumor of bone: a multicenter epidemiological study in brazil / Tumor de células gigantes ósseas: estudo epidemiológico multicêntrico no brasil

ABSTRACT Introduction: Giant cell tumor of bone (GCTB) mainly affects young adults' long bone epiphyses, threatening bone strength and joint function. Surgery is the primary treatment, although post-surgery recurrence is significant. This study analyzes patient profiles, treatments, and outcomes for GCTB in Brazil. Methods: We retrospectively assessed local recurrence, metastasis, and treatment approaches in 643 GCTB patients across 16 Brazilian centers (1989-2021), considering regional differences. Results: 5.1% (n=33) developed pulmonary metastases, 14.3% (n=92) had pathological fractures, and the local recurrence rate was 18.2% (n=114). Higher rates of pulmonary metastases (12.1%) and advanced tumors (Campanacci III, 88.9%) were noted in lower-income North and Northeast regions. The North also had more pathological fractures (33.3%), extensive resections (61.1%), and amputations (27.8%). These regions faced longer surgical delays (36-39 days) than the South and Southeast (27-33 days). Conclusions: Our findings corroborate international data, underscoring regional disparities in Brazil that may lead to worse outcomes in disadvantaged areas. This highlights the need for improved orthopedic oncology care in Brazil's economically and structurally challenged regions. Level of Evidence III; Retrospective Cohort.

RESUMO Introdução: O tumor de células gigantes do osso (TCG) atinge principalmente epífises de ossos longos em adultos jovens, impactando a resistência óssea e a funcionalidade articular. O tratamento principal é cirúrgico, mas há significativa recorrência pós-operatória. Este estudo analisa o perfil de pacientes e tumores de TCG no Brasil, abordagens de tratamento e resultados. Métodos: Avaliamos retrospectivamente taxas de recorrência, metástase e tratamentos em 643 pacientes tratados em 16 centros brasileiros de 1989 a 2021, considerando a distribuição geopolítica. Resultados: 5,1% desenvolveram metástases pulmonares e 14,3% tiveram fraturas patológicas. A recorrência local foi de 18,2%. Regiões economicamente menos favorecidas, como Norte e Nordeste, mostraram maiores incidências de metástases pulmonares (12,1%) e tumores avançados (Campanacci III, 88,9%). O Norte teve alta ocorrência de fraturas patológicas (33,3%), cirurgias extensas (61,1%) e amputações (27,8%). Nessas regiões, o tempo pré-cirúrgico foi mais longo (médias de 36 e 39 dias) comparado ao Sul e Sudeste (27 e 33 dias, respectivamente). Conclusões: Os resultados refletem disparidades regionais no Brasil, sugerindo que condições socioeconômicas influenciam os desfechos clínicos. Estes achados são importantes para melhorar o cuidado oncológico ortopédico em regiões desfavorecidas do país. Nível de Evidência III; Coorte Retrospectiva.

Anaplastic (undifferentiated) carcinoma of pancreas, an uncommon variant: Diagnosed on endoscopic-guided fine needle aspiration

Anaplastic carcinoma of pancreas (ACP) are rare pancreatic neoplasms. They are well known to be associated with more aggressive tumor behavior and less favorable prognosis than usual pancreatic ductal adenocarcinoma. Endoscopic-guided fine needle aspiration (EUS-FNA) is now a widely accepted modality in diagnosis of pancreatic lesions. However, only a few reports are available describing cytological features of anaplastic carcinoma. Here, we report two cases of ACP diagnosed on EUS-FNA.

Argon Beam Coagulation as an Adjuvant for Extended Curettage for Giant Cell Tumors of the Bone: A Study of 50 Cases / Coagulação por plasma de argônio como adjuvante à curetagem estendida em tumores de células gigantes do osso: Um estudo de 50 casos

Abstract Objective Extended curettage with adjuvants of giant cell tumors of bone is associated with a lower rate of recurrence of the tumor while preserving the adjacent joint. The present study was conducted to estimate the recurrence rate and functional outcome after using argon beam as an adjuvant for extended curettage. Methods We selected 50 patients with giant cell tumors, meeting all the inclusion criteria, who underwent extended curettage using high speed burr and argon beam photocoagulation between July 2016 to January 2019. On their follow-up visit, they were assessed for any complaints of pain and signs like tenderness, locally raised temperature, and decreased range of motion of the adjacent joint. Radiologically, the patients were assessed for any increased lucency around the cement mantle and uptake of the subarticular graft. Musculoskeletal Tumor Society Score (MSTS) was administered to the patients, and range of motion of the adjacent joint was compared with the contralateral joint. Results Recurrence was found in 4 patients, that is, an 8% recurrence rate. Twenty-six out of 28 patients with a tumor in the lower limb had a grade-5 weight bearing status 6 months from the surgery, and their range of motion was comparable to contralateral healthy joint with an average MSTS score of 27 (18-30). Conclusion Extended curettage of giant cell tumors using argon beam coagulation is associated with low recurrence rates of the tumor and is an effective modality in the treatment of these tumors besides having a functional outcome comparable to the healthy limb.

Resumo Objetivo A curetagem estendida com adjuvantes de tumores de células gigantes do osso está associada a uma menor taxa de recidiva da neoplasia e à preservação da articulação adjacente. Este estudo foi feito para estimar a taxa de recidiva e o resultado funcional após o uso de plasma de argônio como adjuvante à curetagem estendida. Métodos Cinquenta pacientes com tumores de células gigantes que atendiam a todos os critérios de inclusão foram selecionados para o estudo e submetidos à curetagem estendida com broca de alta velocidade e fotocoagulação com plasma de argônio entre julho de 2016 e janeiro de 2019. À consulta de acompanhamento, os pacientes foram avaliados quanto a quaisquer queixas de dor e sinais como sensibilidade, aumento local da temperatura e diminuição da amplitude de movimento da articulação adjacente. Radiologicamente, os pacientes foram avaliados quanto à presença de qualquer aumento de radiotransparência ao redor do manto de cimento e incorporação do enxerto subarticular. O questionário Musculoskeletal Tumor Society Score (MSTS) foi administrado aos pacientes e a amplitude de movimentação da articulação adjacente foi comparada à articulação contralateral. Resultados Quatro pacientes apresentaram recidiva, o que corresponde a uma taxa de 8%. Seis meses após a cirurgia, 26 de 28 pacientes com tumor no membro inferior tinham capacidade de sustentação de peso de grau 5 e amplitude de movimento comparável à articulação saudável contralateral, com pontuação MSTS média de 27 (intervalo de 18 a 30). Conclusão A curetagem estendida de tumores de células gigantes com coagulação por plasma de argônio está associada a baixas taxas de recidiva da neoplasia; é uma modalidade eficaz no tratamento desses tumores e o resultado funcional é comparável ao do membro saudável.

Outcomes of Extended Curettage with and without Bone Allograft for Grade II Giant Cell Tumors around the Knee. A Retrospective Comparative Study / Resultados da curetagem estendida com e sem aloenxerto ósseo para tumores de células gigantes de grau II no joelho. Um estudo comparativo retrospectivo

Abstract Objective This is the first study to establish the utility of extended curettage with or without bone allograft for Grade II giant cell tumors GCTs around the knee joint with the aim of exploring postoperative functional outcomes. Methods We retrospectively reviewed 25 cases of Campanacci grade II GCTs undergoing extended curettage between January 2014 and December 2019. The participants were divided into two groups: one group of 12 patients underwent extended curettage with bone allograft and bone cement, while the other group of 13 patients underwent extended curettage with bone cement only. Quality of life was assessed by the Revised Musculoskeletal Tumor Society Score and by the Knee score of the Knee Society; recurrence and complications were assessed for each cohort at the last follow-up. The Fisher test and two-sample t-tests were used to compare the categorical and continuous outcomes, respectively. Results The mean age was 28.09 (7.44) years old, with 10 (40%) males and 15 females (60%). The distal femur and the proximal tibia were involved in 13 (52%) and in 12 (48%) patients, respectively. There was no significant difference in the musculoskeletal tumor society score (25.75 versus 27.41; p= 0.178), in the knee society score (78.67 versus 81.46; p= 0.33), recurrence (0 versus 0%; p= 1), and complications (25 versus 7.69%; p= 0.21). Conclusions Extended curettage with or without bone allograft have similar functional outcomes for the knee without any major difference in the incidence of recurrence and of complications for Grade II GCTs. However, surgical convenience and cost-effectiveness might favor the bone cement only, while long-term osteoarthritis prevention needs to be investigated to favor bone allograft.

Resumo Objetivo Este é o primeiro estudo a estabelecer a utilidade da curetagem estendida com ou sem enxerto ósseo em tumores de células gigantes (TCGs) de grau II na articulação do joelho com o objetivo de explorar os resultados funcionais pós-operatórios. Métodos Revisamos retrospectivamente 25 casos de TCGs de grau II de Campanacci submetidos a curetagem estendida entre janeiro de 2014 e dezembro de 2019. Os participantes foram divididos em 2 grupos: um grupo de 12 pacientes foi submetido a curetagem estendida com aloenxerto ósseo e cimento ósseo, enquanto o outro grupo, com 13 pacientes, foi submetido a curetagem estendida apenas com cimento ósseo. A qualidade de vida foi avaliada pela Pontuação Revista da Musculoskeletal Tumor Society (MTS, na sigla em inglês) e pela Pontuação da Knee Society (KS, na sigla em inglês), enquanto as taxas de recidiva e complicações foram avaliadas em cada coorte na última consulta de acompanhamento. O teste de Fisher e os testes t de duas amostras foram usados para comparação de resultados categóricos e contínuos, respectivamente Resultados A média de idade dos pacientes foi de 28,09 (7,44) anos; 10 (40%) pacientes eram do sexo masculino e 15 (60%) pacientes eram do sexo feminino. O fêmur distal e a tíbia proximal foram acometidos em 13 (52%) e 12 (48%) dos pacientes, respectivamente. Não houve diferença significativa na pontuação revista da MTS (25,75 versus 27,41; p= 0,178), na pontuação da KS (78,67 versus 81,46; p= 0,33) e nas taxas de recidiva (0 versus 0%; p= 1) e complicações (25 versus 7,69%; p= 0,21). Conclusões A curetagem estendida com ou sem aloenxerto ósseo tem resultados funcionais semelhantes em pacientes com TCGs de grau II no joelho, sem qualquer diferença importante na incidência de recidivas e complicações. No entanto, a conveniência cirúrgica e o custo-benefício podem favorecer a utilização apenas de cimento ósseo, enquanto a prevenção da osteoartrite em longo prazo precisa ser investigada para favorecer o enxerto ósseo.

Accuracy of Doppler ultrasound in the diagnosis of giant cell arteritis: a systematic review and meta-analysis

Abstract Background Giant cell arteritis (GCA) is the most common primary systemic vasculitis in people 50 years of age and over, and it is considered a medical emergency due to the potential risk of permanent visual loss. Color Doppler ultrasound (CDU) of the temporal arteries is a rapid, noninvasive method to diagnose GCA. This study aims to determine the diagnostic accuracy of the halo sign in temporal arteries by CDU in people with suspected GCA. Methods The systematic literature review included the search for publications in the following electronic databases: PubMed, Embase, CENTRAL, LILACS, WHO ICTRP, ClinicalTrials.gov, gray literature up to December 2022, and no date or language restrictions were applied. We analyzed studies including patients over 50 years of age with suspected GCA evaluating CDU of temporal arteries as a diagnostic tool against clinical diagnosis as a standard reference. Paper titles and abstracts were selected by two investigators independently for all available records. The quality of the studies was assessed using the Quality of Diagnostic Accuracy Studies tool (QUADAS-2) and the R software (version 4.2.1) was used for data analysis. The protocol of this review is registered with PROSPERO (CRD42016033079). Results Twenty-two studies including 2893 participants with suspected GCA who underwent temporal artery CDU were evaluated. The primary analysis results showed a sensitivity of 0.76 [95% confidence interval (95 CI) 0.69-0.81] and specificity of 0.93 (95 CI 0.89-0.95) when the halo sign was compared to clinical diagnosis. The sensitivity value of 0.84 (95 CI 0.72-0.92) and specificity of 0.95 (95 CI 0.88-0.98) were found in five studies involving 1037 participants that analyzed the halo sign and temporal artery compression sign. A sensitivity of 0.86 (95 CI 0.78-0.91) and specificity of 0.95 (95 CI 0.89-0.98) were found in four studies with 603 participants where the halo sign was evaluated CDU on temporal and axillary arteries. Conclusion The detection of the halo sign by CDU of temporal arteries has good accuracy for the diagnosis of cranial GCA. The compression sign in temporal arteries and the addition of axillary arteries assessment improves the diagnostic performance of CDU for GCA. Trial registration PROSPERO CRD42016046860.

Tumor de células gigantes malignizado: a propósito de un caso

Introducción: La rodilla es la región anatómica donde asientan la mayor cantidad de tumores benignos y malignos que afectan el esqueleto humano, entre ellos se encuentra el tumor de células gigantes. Objetivo: Conocer un paciente con un tumor de células gigantes que sufrió transformación maligna. Caso clínico: Paciente, masculino de 28 años de edad, blanco sin antecedentes mórbidos de salud, que acudió a la consulta externa de la especialidad de Ortopedia y Traumatología por presentar desde hace dos años una tumoración dolorosa a nivel de la rodilla que en los últimos dos meses ha aumentado de tamaño. Se realizaron exámenes imagenológicos mediante radiografía simple, tomografía axial computarizada e imagen de resonancia magnética, además de toma de biopsia incisional para confirmar el diagnóstico. Al analizar los resultados de los exámenes anteriores el equipo multidisciplinario decidió la amputación de la extremidad. Conclusiones: El tumor de células gigantes es una enfermedad que se presenta con mayor frecuencia desde la tercera a quinta décadas de la vida, sus complicaciones principales son la recidiva, las metástasis pulmonares y la transformación maligna. Por lo general, los enfermos con esta última complicación necesitan de procedimientos como la amputación de la extremidad.

Introduction: The knee is the anatomical region where the largest number of benign and malignant tumors that affect the human skeleton settle, among them is the giant cell tumor. Objective: To present a patient with a giant cell tumor that underwent malignant transformation. Clinical case: A 28-year-old white male with no morbid health history, who attended the Orthopedics and Traumatology outpatient clinic for presenting a tumor accompanied by pain at the level of the right knee. It appeared two years ago, but has increased in size rapidly in the last two months. Imaging tests were performed using plain radiography, computed tomography, and magnetic resonance imaging, as well as incisional biopsy to confirm the diagnosis. After analyzing the results of the previous examinations, the multidisciplinary team decided to amputate the limb. Conclusions: The giant cell tumor is an entity that occurs most frequently from the third to fifth decades of life; its main complications are recurrence, lung metastases and malignant transformation. In general, patients with this last complication need procedures such as amputation of the limb.

Ankle arthrodesis with intramedullary retrograde nail for bone tumors. preliminary results and surgical technique / Artrodese do tornozelo com cavilha intramedular retrógrada para tumores ósseos. resultados preliminares e técnica cirúrgica

ABSTRACT Objective Present the preliminary results of a case series using the surgical ankle arthrodesis technique with an intramedullary retrograde nail for bone tumors. Methods We present the preliminary data of 4 patients, 3 males and 1 female, with a mean age of 46,2 (range 32 to 58) years, with histology proven Giant Cell Tumour of bone in 3 and osteosarcoma in 1. The mean resection length of distal tibia was 11,75 (range 9 to 16) cm, and all the patients underwent reconstruction with a tibiotalocalcaneal arthrodesis with an intercalary allograft fixed by a retrograde intramedullary nail. Results Oncological follow-up evolved without evidence of local recurrence or disease progression in all patients. After a mean time of 69.5 (range 32 to 98 months), patients had a mean MSTS12 functional score of 82.5% (range 75 to 90). All tibial arthrodesis and diaphyseal osteotomy sites were fused within 6 months with a return to activities without complications related to coverage skin or infection. Conclusion No complications were recorded; all arthrodesis and diaphysial tibial osteotomy sites fused by 6 months, and the mean follow-up of those patients was 69,5 (range 32 to 988) months, with a mean functional MSTS score of 82,5% (range 75-90). Level of Evidence: IV; Retrospective Case Series.

RESUMO Objetivo Apresentar os resultados preliminares de uma série de casos utilizando a técnica cirúrgica de artrodese do tornozelo com haste intramedular retrógada para tumores ósseos. Métodos Apresentamos os dados preliminares de quatro pacientes, três homens e uma mulher, com idade média de 46,2 (variação de 32 a 58) anos, com histologia comprovada de tumor de células gigantes em três e osteossarcoma em um. O comprimento médio de ressecção da tíbia distal foi de 11,75 (variação de 9 a 16) cm, e todos os pacientes foram submetidos à reconstrução com uma artrodese tibiotalocalcaneana com um aloenxerto intercalar fixado por uma haste intramedular retrógrada. Resultados O acompanhamento oncológico evoluiu sem evidências de recidiva local ou progressão da doença, em todos os pacientes. Após um tempo médio de 69,5 (variação de 32 a 98 meses), os pacientes tiveram uma pontuação média funcional MSTS12 de 82,5% (variação de 75 a 90). Todos os locais de artrodese e osteotomia diafisária tibiais foram fundidos em 6 meses com retorno às atividades de vida diária sem complicações relacionadas à cobertura ou infecção. Conclusão Não foram registradas complicações; todos os locais de artrodese e osteotomia diafisária da tíbia fundiram-se em 6 meses, e o acompanhamento médio desses pacientes foi de 69,5 (variação de 32 a 988) meses, com uma pontuação média funcional MSTS de 82,5% (variação de 75-90). Nível de Evidência IV; Série de Casos Retrospectivos.

The Efficacy of Bisphosphonate in the Treatment of Giant Cell Tumour of the Bone: A Systematic Review and MetaAnalysis

@#Introduction: Anti-osteoclastic mechanism of Bisphosphonate (BP) is crucial to treat Giant Cell Tumour of the Bone (GCTB), however no established guidelines of its use have been published. This systematic review and metaanalysis is the first to summarise recent clinical studies on the subject. Materials and methods: A systematic search was performed based on PRISMA guidelines for clinical trials of BP administration in GCTB. Baseline data including BP regimen, dose and timing was summarised. The primary outcomes assessed were recurrence rate, metastases, survival rate, functional outcome, clinical outcome, radiological outcome, and adverse effect. Results: We identified 8 articles from 2008-2020. Most studies administer 4mg of Zoledronic acid post-operatively, with five studies mentioning pre-operative administration and six studies describing post-operative administration. There was a total of 181 GCTB cases analysed in this study. The BP group presented lower recurrence rate than control group (three studies; Odds Ratio [OR] 0.15; 95% Confidence Interval [CI], 0.05 – 0.43; p<0.05; heterogeneity, I2=0%). As for survival rate, BP group is comparable to control group (two studies; OR 1.67; 95% CI, 0.06 – 48.46; p=0.77; heterogeneity, I2=65%). Conclusion: Bisphosphonate therapy offers satisfactory recurrence rate, functional outcome, clinical outcome, radiological outcome, survival rate and metastases rate in patients with GCTB, with minimal adverse effects. Pre- and post-operative administration of bisphosphonates in combination might be the most beneficial in minimalising the recurrence rate.

A Case Report of Annular Elastolytic Giant Cell Granuloma / 罕见病研究

Annular elastolytic giant cell granuloma(AEGCG) is a rare granulomatous skin condition. We present a case of a 67-year-old man with annular plaques on the face, neck, shoulder, back and upper limbs, and mildly pruritis exceeding one year. Histopathological examination demonstrated granulomatous inflammatory infiltration of lymphocytes, histiocytes and multinuclear giant cells in the middle and the upper dermis, with more local eosinophils. Elastic fiber staining showed that elastic fibers were absent in granuloma area and were engulfed by multinucleated giant cells. Based on the clinical and histopathological findings, a diagnosis of AEGCG was made. The etiology and pathogenesis of this condition are unclear, and atypical manifestations of non-exposed areas can also occur.It is usually related to systemic diseases lacks specific treatment at present.

Recommendations of diagnosis and treatment of giant cell arteritis and polymyalgia rheumatic in China / 中华内科杂志

Polymyalgia rheumatica (PMR) is a syndrome characterized by pain and morning stiffness in the neck and shoulder and pelvic girdles, as well as raised acute-phase reactants, with or without systemic symptoms, such as fever. Giant cell arteritis (GCA) is a systemic vasculitis of unclear etiology that involves systemic arteries, principally affecting medium- and large-sized arteries with skipped, segmental alterations and granulomatous vasculitis seen on histopathology. In China, epidemiological data describing GCA are still limited; thus, the prevalence might be underestimated. The involvement of vessels in GCA can cause irreversible visual impairment or loss and stroke, which are serious complications. PMR is three times more prevalent than GCA, and other specific diseases should be excluded before the diagnosis is established. PMR symptoms can be present in 40%-60% of patients with GCA. Conversely, GCA can develop in 15% of patients with PMR. Chinese Rheumatology Association, based on the clinical diagnosis and treatment guidelines in 2005, utilizing the experience and guidelines of diagnosis and treatment at home and abroad, formulated this specification to standardize the diagnosis and treatment of GCA and PMR and improve the patient′s prognosis.

Mid-term efficacy of surface knee prosthesis combined with bionic block in joint reconstruction after resection of giant cell tumor in proximal tibia / 中华骨科杂志

A total of 6 patients were treated with surface knee joint prosthesis combined with 3D-printed customized bionic tibial block for reconstruction of bone defect after giant cell tumor (GCT) in proximal tibia (1 male and 5 females, aged 50, 40, 68, 53, 35, 42, respectively). 3 patients with primary and 3 patients with recurrence of GCT. After resection of the tumor, the bone defect was filled with 3D-printed block combined with surface knee prosthesis, the surrounding ligaments were reconstructed with microporous structure and artificial mesh. All cases were followed up for 60, 90, 60, 60, 75, and 50 months, respectively. During the follow-up, there was no local recurrence, no radiolucent lines around prosthesis, and no signs of loosening. The clinical scores of the American Knee Society Score (KSS) were 87, 92, 85, 90, 95 and 78. The functional scores were 70, 100, 70, 100, 100 and 80 respectively. Musculoskeletal Tumor Society Score (MSTS) were 27, 28, 26, 26, 26, 27, respectively. Surface knee prosthesis combined with bionic block can effectively fill the bone defect after resection of GCT in proximal tibia, achieve anatomical and functional reconstruction of knee joint.

Treatment of Patients Diagnosed with Giant Cell Tumor of Bone: Experience of a Philanthropic Hospital in the State of Piauí, Brazil / Tratamento de pacientes com diagnóstico de tumor ósseo de células gigantes: Experiência de um hospital filantrópico no Piauí, Brasil

Abstract Objective To evaluate the treatment of patients with giant cell tumors of bone treated from 2009 to 2019 in a philanthropic hospital, as well as to try and determine the regional clinical and epidemiological profile, aiming to enrich the Brazilian data set and compare our findings with those of the literature. Methods An analytical, observational, and cross-sectional study with retrospective data collection and a quantitative approach, analyzing medical records of patients with giant cell tumors treated at a philanthropic hospital from 2009 to 2019. Results We evaluated 49 medical records; 55.1% of the patients were women, 53.1% were aged between 20 and 40 years, 69.4% of the cases were Campanacci grade III, and 30.6% affected the proximal end of the tibia. The rate of pathological fractures secondary to the tumor and pulmonary metastasis was low. More than 69% of the patients underwent intralesional surgery. Recurrence occurred in 16.3% of the cases. Conclusion The criteria used for the diagnosis, classification, and treatment at our service followed the standards established by the literature, and they can guide further research and improve local prognosis in the future.

Resumo Objetivo Avaliar o tratamento fornecido a pacientes com diagnóstico de tumor de células gigantes ósseo atendidos no período de 2009 a 2019 em um hospital filantrópico, bem como determinar o perfil clínico e epidemiológico regional, visando enriquecer os dados nacionais e comparar os achados com a literatura existente. Métodos Estudo analítico, observacional e transversal, com coleta retrospectiva e abordagem quantitativa, com análise de prontuários de pacientes diagnosticados com tumor de células gigantes atendidos em um hospital filantrópico no período de 2009 a 2019. Resultados Foram avaliados 49 prontuários, sendo que 55,1% eram de mulheres, com 53,1% dos casos na faixa etária de 20 a 40 anos, 69,4% de casos de grau III de Campanacci, e 30,6% acometendo a extremidade proximal da tíbia. Observou-se baixo índice de fratura patológica secundária ao tumor e de metástase pulmonar. A cirurgia intralesional foi realizada em 69,5% dos pacientes. Houve recidiva em 16,3% dos casos. Conclusão Os critérios usados para diagnóstico, classificação e tratamento em nosso serviço seguiram os padrões estabelecidos pela literatura, e podem orientar novas pesquisas e melhorar o prognóstico local futuramente.

Anti-Histone H3.3 G34W antibody is a sensitive and highly specific immunohistochemistry marker for the diagnosis of Giant cell tumor of bone. A validation based on analysis of 198 cases from a single centre in India

Context: The diagnosis of giant cell tumor of bone (GCTB) is difficult in small biopsies with unusual age of presentation, location, and extensive secondary changes. Most of the GCTBs harbor H3F3A G34W mutations with a subset of cases showing alternate G34V, G34R, and G34L mutations. Objectives: To analyze the expression of anti-histone H3.3G34W antibody in different cellular components of GCTB across different locations and presentations (including the unusual ones) and validate the utility of this antibody in the diagnosis of GCTB and differentiate it from the other osteoclast-like giant-cell-rich lesions. Design: Immunohistochemistry was performed using anti-histone H3.3G34W antibody in the diagnosed cases of GCTB (136 cases of GCTB from 133 patients, including two malignant GCTBs) and other giant cell-containing lesions (62 cases). The presence of unequivocal crisp nuclear staining was considered positive. Results: Immunohistochemistry revealed unequivocal nuclear positivity in the mononuclear cells in 87.3% of the cases of GCTB. Of these, most showed diffuse expression with moderate to strong intensity staining. The positive staining was restricted to the nuclei of mononuclear cells with the nuclei of osteoclastic giant cells being distinctly negative. In addition to conventional GCTBs, two cases each of multicentric and malignant GCTB showed positive staining. The other giant-cell containing lesions were distinctly negative. The present study showed a sensitivity of 87.3% with specificity and positive predictive value of 100%. Conclusion: The anti-histone G34W antibody is a highly sensitive and specific marker for the diagnosis of GCTB and differentiating it from its mimics. The positive staining is restricted to the mononuclear cell component of GCTB with sparing the osteoclastic giant cells further reiterating the fact that the mononuclear stromal cells are the true neoplastic component of GCTB.

Recomendaciones de la Sociedad Argentina de Reumatología en el manejo de la arteritis de células gigantes / Recommendations of the Argentine Society of Rheumatology in the management of giant cell arteritis

La arteritis de células gigantes (ACG) es una vasculitis sistémica que afecta a personas adultas; compromete vasos arteriales de mediano y gran calibre, con potenciales complicaciones de gravedad, como la ceguera, y es considerada una emergencia médica. El objetivo de estas guías fue desarrollar las primeras recomendaciones argentinas para su tratamiento, basadas en la revisión de la literatura mediante metodología GRADE. Un panel de expertos en vasculitis elaboró las preguntas en formato PICO (población, intervención, comparador y outcomes), y luego un panel de expertos en metodología efectuó la revisión de la bibliografía con la extracción de la evidencia para cada una de las preguntas. Se realizó un focus group de pacientes para conocer sus preferencias y experiencias. Finalmente, con la información recabada, el panel de expertos en vasculitis procedió a la votación de las recomendaciones que a continuación se presentan.

Giant cell arteritis (GCA) is a systemic vasculitis affecting adult patients and involving large and medium vessels. Potential serious complications as blindness may occur and it is considered a medical emergency. The objective of elaborating this guideline was to develop first Argentinian GCA treatment recommendations using GRADE methodology. An expert panel generated clinically meaningful questions addressing aspects of the treatment of GCA in the Population, Intervention, Comparator and Outcome (PICO) format and then a group of methodology experts reviewed and extracted data from literature summarizing available evidence. A patient's focus group discussion took place gathering information on their preferences and experiences. Finally, the vasculitis expert panel, with all the information obtained, voted recommendations here presented.

Tumor de células gigantes / Giant Cell Tumor

Introducción: El tumor de células gigantes continúa siendo uno de los tumores óseos con muchas controversias en su diagnóstico y manejo, por ortopédicos, radiólogos y patólogos. Objetivo: Enriquecer el diagnóstico de esta enfermedad desde el aporte de las técnicas de imagen. Presentación de caso: Paciente masculino de 33 años de edad, remitido a la consulta de Ortopedia por presentar un aumento de volumen en la rodilla de meses de evolución, que empeoró progresivamente hasta llegar a la impotencia funcional. Al examen físico se constata un marcado aumento del volumen por lo que se indican estudios de imagen. Conclusiones: El diagnóstico temprano ayuda a mejorar el estilo de vida de estos pacientes. El tratamiento quirúrgico es el más indicado en tumores de células gigantes, ya que logra buenos resultados tanto en el tratamiento del tumor primario como de las recidivas (AU)

Introduction: The giant cell tumor continues to be one of the bone tumors with many controversies in diagnosis and management, by orthopedists, radiologists and pathologists. Objective: To enrich the diagnosis of this disease from the contribution of imaging techniques. Casereport: We report the case of a 33-year-old male patient, referred to the Orthopedics consultation for presenting an increase in volume in his knee, with months of evolution, which progressively worsened until functional impotence. Physical examination showed a marked increase in volume, so imaging studies are indicated. Conclusions: Early diagnosis helps to improve the lifestyle of these patients. Surgical treatment is the most indicated in giant cell tumors, since it achieves good results both in the treatment of the primary tumor and recurrences(AU)

Tongue Necrosis due to Giant Cell Arteritis / Necrose da língua por arterite de células gigantes

Giant Cell Arteritis is a vasculitis that mainly affects women over 50. The most common manifestations are headache, jaw claudication, and amaurosis. If not diagnosed early, it can lead to rare irreversible ischemic consequences, with tongue necrosis being one of these. We report a case of a previously undiagnosed patient with lateral tongue necrosis who responded well to oral corticosteroid treatment. The diagnosis is clinical, laboratory and histological and may be aided by imaging exams. Initial treatment is with oral corticosteroids, with methotrexate and tocilizumab as alternatives. Diagnostic suspicion and quick start of treatment favorably influence the prognosis of the disease (AU)

Apresentamos um caso de arterite de células gigantes com amaurose, seguida de necrose da língua, diagnosticado clinicamente com boa resposta terapêutica ao corticoide oral. Salientamos que os casos de necrose da língua são extremamente raros. A suspeita diagnóstica de arterite de células gigantes é de fundamental importância e mesmo na impossibilidade de realizar a biópsia de artéria temporal, não se deve retardar o início do tratamento sob pena de acarretar sequelas definitivas ou manifestações mais graves da doença (AU)

Diagnóstico e tratamento de granuloma periférico de células gigantes em mandíbula - relato de caso / Diagnosis and treatment of peripheral granuloma of giant cell in the jaw - case report

O granuloma periférico de células gigantes (GPCG) é uma lesão hiperplásica benigna causada por trauma local ou trauma crônico. Origina-se do ligamento periodontal ou mucoperiósteo. O objetivo deste trabalho é apresentar um caso de GPCG em mandíbula tratada com sucesso através de excisão cirúrgica, curetagem e ostectomia periférica.

Peripheral giant cell granuloma (PGCG) is a benign hyperplastic lesion caused by local trauma or chronic trauma. It originates from the periodontal ligament or mucoperiosteum. The objective of this work is to present a case of PGCG in the mandible successfully treated through surgical excision, curettage and peripheral ostectomy.

Aggressive Central Giant Cell Granuloma in a Child: A Case Report and Review

Introduction: An aggressive variant of central giant cell granuloma in a paediatric patient is a localized benign osteolytic prolif- eration. The lesion consist of fibrous tissue with haemorrhage, hemosiderin deposits and presence of osteoclast-like giant cells with reactive bone formation. Clinically shown with rapid growth, resorption of roots, displacement of teeth and thinning or perforation of cortical bone, and marked recurrence. Case presentation: A 9-year-old boy reported with a 3 days history of asymptomatic soft tissue swelling in left mandibular pos- terior region which was diagnosed as central giant cell granuloma based on clinical, radiological and histopathological features. Management and Prognosis: Surgical resection was performed without any signs of recurrence on 9 months follow up. Conclusion: Prompt diagnosis and treatment of Central giant cell granuloma can significantly improve morbidity and long- term outcomes.

Arteritis de células gigantes: ¿Cómo debutan pacientes chilenos? Análisis de casos y revisión de la literatura / Giant cell arteritis. Experience in 32 patients

BACKGROUND: Giant cell Arteritis (GCA) is the most common systemic vasculitis in patients over 50 years. Diagnosis is based on clinical, laboratory, imaging and biopsy. Temporal artery biopsy (TAB) may be inconclusive in up to 40% of patients. AIM: To describe disease features of patients diagnosed with GCA. MATERIAL AND METHODS: Review of pathology reports of giant cell arteritis and clinical records of patients seen with the diagnosis between 2000 and 2019. Demographic, clinical, laboratory, histopathology, imaging, treatment and follow-up variables were analyzed. RESULTS: We fetched 32 patients with a median age at diagnosis of 70.5 years (range 57-90), 81% women. Twenty eight percent had polymyalgia. 72% had only cranial symptoms, 12% had extracranial involvement and 13% exclusive extracranial involvement. The median time from onset of symptoms to diagnosis was two months (range 0.5-8). All had elevated erythrocyte sedimentation rate and c reactive protein. A TAB was performed in 27 patients and in 17 (65.4%) it confirmed the diagnosis. Transmural inflam- mation was the most frequent finding. All patients received steroids. Follow-up information was available from 25 patients and 92% received a steroid-spa- ring agent, usually methotrexate (74%). Ninety two percent achieved clinical remission in the first year and 59% had minor relapses during steroid tapering. CONCLUSIONS: Our patients showed frequent extracranial involvement and TAB was a useful diagnostic tool.

Granuloma actínico: reporte de un caso / Actinic granuloma: a case report

Resumen El granuloma actínico es una dermatosis granulomatosa poco frecuente, que se observa en adultos de mediana edad con antecedentes de fotoexposición. Su patogénesis no es clara; siendo la teoría más aceptada la radiación solar como factor disparador. Se caracteriza por placas anulares con atrofia central y bordes eritematosos elevados que son similares a los observados en el granuloma anular. Se presenta el caso clínico de una paciente de 71 años, con placas anulares en antebrazos compatibles con granuloma actínico. Se comunica el caso por lo infrecuente de esta entidad y se realiza una revisión bibliográfica.

Abstract Actinic granuloma is a rare granulomatous dermatosis, seen in middle-aged adults with a history of photoexposure. Its pathogenesis is not clear; the most accepted theory being solar radiation as a triggering factor. It is characterized by annular plaques with central atrophy and raised erythematous borders that are similar to those seen in granuloma annulare. The clinical case of a 71-year-old patient with annular plaques on the forearms compatible with actinic granuloma is presented. The case is reported due to the infrequent nature of this entity and a bibliographical review is carried out.