GIANT CELL TUMOR OF BONE: A MULTICENTER EPIDEMIOLOGICAL STUDY IN BRAZIL.

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.

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.

Validation of the Brazilian version of the musculoskeletal tumor society rating scale for lower extremity bone sarcoma.

BACKGROUND: The Musculoskeletal Tumor Society (MSTS) rating scale is an English-language instrument used worldwide to assess functional evaluation of patients with musculoskeletal cancer. Despite its use in several studies in English-speaking countries, its validity for assessing patients in other languages is unknown. The translation and validation of widely used scales can facilitate the comparison across international patient samples. OBJECTIVES/PURPOSES: The objectives of this study were (1) to translate and culturally adapt the MSTS rating scale for functional evaluation in patients with lower extremity bone sarcomas to Brazilian Portuguese; (2) analyze its factor structure; and (3) test the reliability and (4) validity of this instrument. METHOD: The MSTS rating scale for lower limbs was translated from English into Brazilian Portuguese. Translations were synthesized, translated back into English, and reviewed by a multidisciplinary committee for further implementation. The questionnaire was administered to 67 patients treated for malignant lower extremity bone tumors who were submitted to limb salvage surgery or amputation. They also completed a Brazilian version of the Toronto Extremity Salvage Score (TESS). Psychometric properties were analyzed including factor structure analysis, internal consistency, interobserver reliability, test-retest reliability, and construct validity (by comparing the adapted MSTS with TESS and discriminant validity). RESULTS: The MSTS rating scale for lower limbs was translated and culturally adapted to Brazilian Portuguese. The MSTS-BR proved to be adequate with only one latent dimension. The scale was also found to be reliable in a population that speaks Brazilian Portuguese showing good internal consistency (Cronbach's alpha = 0.84) and reliability (test-retest reliability and interobserver agreement of 0.92 and 0.98, respectively). Validity of the Brazilian MSTS rating scale was proved by moderate with TESS and good discriminant validity. CONCLUSIONS: The Brazilian version of the MSTS rating scale was translated and validated. It is a reliable tool to assess functional outcome in patients with lower extremity bone sarcomas. It can be used for functional evaluation of Brazilian patients and crosscultural comparisons.

IMMUNOHISTOCHEMICAL ANALYSIS BY KI67 AND IDH1 IN PATIENTS WITH CHONDROSARCOMA.

Objective: To perform an immunohistochemical evaluation using the IDH1 and Ki67 markers in patients who underwent treatment for chondrosarcoma in a reference service center in Brazil. Methods: Retrospective analytical observational study using medical records of patients diagnosed with chondrosarcoma. Besides the epidemiological and clinical profile, important variables for prognosis and correlation with immunohistochemical analysis results with Ki67 and IDH1 markers were evaluated. Results: Histopathological examinations by immunohistochemistry of 56 patients were analyzed, 52% of which were women, with the age group 20-60 years being more prevalent. Grade 1 and 2 histological subtypes corresponded to most chondrosarcomas. The femur, humerus, and tibia were the most frequent anatomical sites. Most tumors (59%) were larger than 8 cm. Ki67 expression was very low (< 10%) in 98% of patients. The analysis of IDH1 was positive in 43% of the cases. The correlation between IDH1 positivity and tumor size was statistically significant, but regarding survival, we observed no significance. Conclusion: Immunohistochemical analysis using IDH1 and Ki67 markers in patients with conventional chondrosarcoma is not useful for prognostic guidance.Level of Evidence II, Prognostic Assessment, Results of Immunohistochemical Tests and Correlation with Survival.

Objetivo: Fazer uma avaliação imuno-histoquímica usando os marcadores IDH1 e Ki67 em pacientes que fizeram tratamento para condrossarcoma em um serviço de referência no Brasil. Métodos: Estudo retrospectivo, analítico e observacional de prontuários de pacientes com diagnóstico de condrossarcoma. Além do perfil epidemiológico e clínico, foram avaliadas variáveis importantes para o prognóstico e a correlação com os resultados da análise imuno-histoquímica utilizando os marcadores Ki67 e IDH1. Resultados: Foram analisados exames histopatológicos por imuno-histoquímica de 56 pacientes, dos quais 52% eram do sexo feminino. A faixa etária mais prevalente foi entre 20 e 60 anos. Os subtipos histológicos graus 1 e 2 corresponderam à maioria dos casos. Fêmur, úmero e tíbia foram os sítios anatômicos mais frequentes. A maioria dos tumores (59%) tinha tamanho superior a 8 cm. O Ki67 teve expressão muito baixa (< 10%) em 98% dos pacientes. Já a análise do IDH1 foi positiva em 43% dos casos. A correlação entre a positividade do IDH1 e o tamanho do tumor foi estatisticamente significativa; já em relação à sobrevida, não houve significância. Conclusão: A análise imuno-histoquímica por meio dos marcadores IDH1 e Ki67 em pacientes com condrossarcoma convencional não é útil para orientação prognóstica. Nível de Evidência II, Avaliação Prognóstica, Resultados de Exames Imuno-Histoquímicos e Correlação com Sobrevida.

UNDIFFERENTIATED PLEOMORPHIC SARCOMA: PROGNOSTIC FACTORS IN 42 EXTREMITY CASES.

Introduction: Soft tissue undifferentiated pleomorphic sarcoma (UPS) in extremities is considered a rare neoplasm, corresponding to 5% of soft tissue sarcomas (STS) today. The objective was to evaluate prognostic factors related to death, local recurrence (LR), and impact on survival rates. Methods: A retrospective study including 42 patients with UPS in extremities treated surgically in a single center. Comparisons were made between demographic data, characteristics of the neoplasia, and treatment. Between the variables with statistical significance, logistic regression analysis was used. Survival rates were evaluated using Kaplan-Meier plots. To compare the effect of variables on survival rates, the Log-Rank test was used. Results: Age group of patients was from 25 to 85 years (mean 58 years), with a mean follow-up of 29.6 months. The variables with the highest effect on survival rates were sizes larger than 15 cm (T4) with p = 0.01, presence of metastatic lesions, and prognostic stage IV according to the American Joint Committee of Cancer (AJCC) with p < 0.001. The mean survival was 25.9 months. Metastasis and stage IV of AJCC were associated with a reduction in patient survival (17.8 months) with Log-Rank test p < 0.001. Conclusion: The main factors of poor prognosis related to mortality and reduction of survival of UPS in extremities were metastatic lesions and stage IV of AJCC. Level of Evidence III, Retrospective Study.

O sarcoma pleomórfico indiferenciado (SPI) de tecidos moles em extremidades, neoplasia rara, correspondente a 5% dos sarcomas de tecidos moles (STM). Objetivo: Avaliar fatores de prognóstico relacionados a óbito, recorrência local (RL) e impacto na sobrevida geral. Métodos: Estudo retrospectivo de 42 pacientes com SPI tratados cirurgicamente em centro único. Foram comparados dados demográficos, características da neoplasia e de tratamento; e para as variáveis com significância estatística, foi realizada análise de regressão logística. A sobrevida foi avaliada através de gráficos de Kaplan-Meier; e os efeito das variáveis sobre a sobrevida, por meio do teste de log-rank. Resultados: Os pacientes tinham idades entre 25 e 85 anos (média de 58 anos), com seguimento ambulatorial médio de 29,6 meses. As variáveis com maior relação com o desfecho óbito foram tamanho maior que 15 cm (T4), com p = 0,01, metástases e estágio IV de prognóstico da American Joint Committee on Cancer (AJCC), com p < 0,001. A sobrevida média dos pacientes foi de 25,9 meses. Presença de metástase e estágio IV da AJCC foram associados à redução na sobrevida dos pacientes (17,8 meses; p < 0,001). Conclusão: Os principais fatores de mau prognóstico relacionados ao óbito e à redução da sobrevida dos pacientes com SPI foram doença metastática e estágio IV da AJCC. Nível de Evidência III, Estudo Retrospectivo.

Imaging of solitary and multiple osteochondromas: From head to toe - A review.

Osteochondromas account for 20%-50% of all benign bone lesions. These tumors may present as solitary non-hereditary lesions, which are the most common presentation, or as multiple tumors associated with hereditary conditions. Plain radiography is the imaging method of choice and demonstrates the typical cortical and medullary continuity of the tumor with the underlying bone. Magnetic resonance imaging is often performed to evaluate cartilage cap thickness, which correlates with malignant transformation. Other local complications include compression of adjacent neurovascular bundles, muscles, and tendons, bursitis, tendon tears, stalk fracture, and angular or rotational long bone deformities. Although the imaging features of osteochondromas are largely known, only a few papers in the literature have focused on their main complications and image-based follow-up. This paper aimed to illustrate the main complications of osteochondromas, suggest an image-based algorithm for management and follow-up and discuss differential diagnosis.

ROLE OF INCISIONAL VACUUM THERAPY IN ENDOPROSTHETIC BONE RECONSTRUCTION SURGERY.

Reconstructive surgery with endoprostheses is the chosen method for treating bone malignancies. Postoperative infections are frequent complications, and their treatment involves prolonged hospital stays and antibiotic therapy. Among the advancements aimed at reducing the rate of postoperative infection, the use of incisional negative pressure therapy (iNPT) has shown promising results, with no reports in the literature regarding its use in patients with such conditions. Objective: To evaluate the effectiveness of iNPT in reducing postoperative complications in surgeries for resection of bone tumors associated with modular endoprosthesis reconstruction. Methods: Retrospective case series of 16 patients diagnosed with osteosarcoma, who underwent resection and reconstruction with endoprosthesis associated with iNPT during the postoperative period. Follow-up was performed for a period of six months, and the evaluated outcomes were the incidence of postoperative infection and complications of the surgical wound. Results: The use of iNPT for a postoperative period of seven days resulted in only three (18.7%) cases of postoperative infection. No cases of wound dehiscence, seroma formation, or hematoma at the surgical site were observed. Conclusion: The rate of surgical wound complications in our case series is lower than that reported in most of the literature, and iNPT appears to be an efficient way to reduce the rate of local complications in reconstructive surgeries with endoprosthesis after resection of bone malignancies. Level of Evidence III, Retrospective Study.

A cirurgia reconstrutiva com endopróteses é o método escolhido no tratamento de malignidades ósseas. As infecções pós-operatórias são complicações frequentes, e seu tratamento envolve internações e antibioticoterapia prolongadas. Entre os avanços que visam reduzir a taxa de infecção pós-operatória, o uso da terapia com pressão negativa incisional (TPNi) vem mostrando resultados promissores, não havendo relatos na literatura de seu emprego em pacientes com tal quadro. Objetivo: Avaliar a eficácia da TPNi em reduzir complicações pós-operatórias em cirurgias de ressecção de tumores ósseos associadas à reconstrução com endopróteses modulares. Métodos: Série de casos retrospectiva de 16 pacientes diagnosticados com osteossarcoma, submetidos à ressecção e reconstrução com endoprótese associada à TPNi durante o pós-operatório. Foi realizado seguimento por um período de seis meses e os desfechos avaliados foram incidência de infecção pós-operatória e complicações da ferida operatória. Resultados: O uso da TPNi por um período pós-operatório de sete dias resultou em apenas três (18,7%) casos de infecção pós-operatória. Não foram observados casos em que ocorreu deiscência da ferida operatória, formação de seromas ou hematomas no sítio cirúrgico. Conclusão: A taxa de complicações de ferida operatória em nossa série de casos é menor que a da maior parte da literatura, e a TPNi parece ser uma forma eficiente de reduzir a taxa de complicações locais em cirurgias reconstrutivas com endoprótese após ressecção de malignidades ósseas. Nível de Evidência III, Estudo Retrospectivo.

Does adjunctive chemotherapy reduce remission rates compared to cortisone alone in unifocal or multifocal histiocytosis of bone?

BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare disorder that can affect almost any organ, including bone. Treatment options include local corticosteroid infiltration in isolated bone lesions and oral corticosteroids and chemotherapy in multifocal bone lesions. Several studies show local corticosteroid injection in unifocal bone lesions heal in more than 75% of patients with minimal side effects. Therefore, it is unclear whether chemotherapy adds materially to the healing rate. QUESTIONS/PURPOSES: We therefore compared overall survival, remission rate, and recurrence rate in patients with bone LCH treated with chemotherapy and corticosteroids or corticosteroids alone. METHODS: We retrospectively reviewed the records of 198 patients with LCH since 1950. Median age at diagnosis was 5 years, male-to-female ratio was 1.33, and the most frequent symptom was local pain (95%). We recorded the disease presentation, demographics, treatment, and clinical evolution of each patient. Minimum followup was 4 months (median, 24 months; range, 4-360 months). RESULTS: The survival rate of the systemic disease group was 76.5% (65 of 85) while the survival rate in the unifocal and multifocal bone involvement groups was 100% at a median 5-year followup. All patients with unifocal bone involvement and 40 of 43 (93%) with multifocal bone involvement had complete remission. One of 30 patients with multifocal bone involvement treated with chemotherapy and oral corticosteroids did not achieve remission whereas two of six receiving only corticosteroids did not achieve remission. CONCLUSIONS: Our observations suggest intralesional corticosteroid injection without adjunctive chemotherapy achieves remission in unifocal bone LCH but may not do so in multifocal single-system bone involvement. Larger series would be required to confirm this observation. LEVEL OF EVIDENCE: Level IV, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.

Comparison of Prophylactic Intravenous Antibiotic Regimens After Endoprosthetic Reconstruction for Lower Extremity Bone Tumors: A Randomized Clinical Trial.

IMPORTANCE: The use of perioperative, prophylactic, intravenous antibiotics is standard practice to reduce the risk of surgical site infection after oncologic resection and complex endoprosthetic reconstruction for lower extremity bone tumors. However, evidence guiding the duration of prophylactic treatment remains limited. OBJECTIVE: To assess the effect of a 5-day regimen of postoperative, prophylactic, intravenous antibiotics compared with a 1-day regimen on the rate of surgical site infections within 1 year after surgery. DESIGN, SETTING, AND PARTICIPANTS: This randomized clinical superiority trial was performed at 48 clinical sites in 12 countries from January 1, 2013, to October 29, 2019. The trial included patients with a primary bone tumor or a soft tissue sarcoma that had invaded the femur or tibia or oligometastatic bone disease of the femur or tibia with expected survival of at least 1 year who required surgical management by excision and endoprosthetic reconstruction. A total of 611 patients were enrolled, and 7 were excluded for ineligibility. INTERVENTIONS: A 1- or 5-day regimen of postoperative prophylactic intravenous cephalosporin (cefazolin or cefuroxime) that began within 8 hours after skin closure and was administered every 8 hours thereafter. Those randomized to the 1-day regimen received identical saline doses every 8 hours for the remaining 4 days; patients, care providers, and outcomes assessors were blinded to treatment regimen. MAIN OUTCOMES AND MEASURES: The primary outcome in this superiority trial was a surgical site infection (superficial incisional, deep incisional, or organ space) classified according to the criteria established by the Centers for Disease Control and Prevention within 1 year after surgery. Secondary outcomes included antibiotic-related complications, unplanned additional operations, oncologic and functional outcomes, and mortality. RESULTS: Of the 604 patients included in the final analysis (mean [SD] age, 41.2 [21.9] years; 361 [59.8%] male; 114 [18.9%] Asian, 43 [7.1%] Black, 34 [5.6%] Hispanic, 15 [2.5%] Indigenous, 384 [63.8%] White, and 12 [2.0%] other), 293 were randomized to a 5-day regimen and 311 to a 1-day regimen. A surgical site infection occurred in 44 patients (15.0%) allocated to the 5-day regimen and in 52 patients (16.7%) allocated to the 1-day regimen (hazard ratio, 0.93; 95% CI, 0.62-1.40; P = .73). Antibiotic-related complications occurred in 15 patients (5.1%) in the 5-day regimen and in 5 patients (1.6%) allocated to the 1-day regimen (hazard ratio, 3.24; 95% CI, 1.17-8.98; P = .02). Other secondary outcomes did not differ significantly between treatment groups. CONCLUSIONS AND RELEVANCE: This randomized clinical trial did not confirm the superiority of a 5-day regimen of postoperative intravenous antibiotics over a 1-day regimen in preventing surgical site infections after surgery for lower extremity bone tumors that required an endoprosthesis. The 5-day regimen group had significantly more antibiotic-related complications. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT01479283.

Extremities Soft Tissue Sarcomas, more Common and as Dangerous as Bone Sarcomas.

Musculoskeletal sarcomas are rare diseases that require attention. They often present high degree of malignancy at diagnosis and, if underestimated, they can evolve aggressively locally and systemically. They present as soft tissue sarcoma and bone sarcomas, with soft tissue being four to five times more common. Most soft tissue sarcomas occur in the extremities. The most common subtypes in children and adolescents are rhabdomyosarcoma and synovial sarcoma; in adults, undifferentiated pleomorphic sarcoma, liposarcoma, leiomyosarcoma, myxofibrosarcoma and synovial sarcoma; all with a high degree of histological malignancy. Many soft tissue sarcomas are confused with benign soft tissue tumors, 100 times more common, so they are resected without the necessary planning, resulting in amputation of a limb that could have been preserved. As in all cancers, the most important prognostic factor is metastatic disease. When it is present, the overall survival rate falls around 20 to 30%. Survival rates are generally similar between bone and soft tissue sarcomas. So soft tissue sarcomas, in addition to being more prevalent, are as aggressive as bone sarcomas, deserving a lot of attention from orthopedic surgeons, who are often the first line of care of carriers of these tumors.

THREE-DIMENSIONAL PRINTING IN ORTHOPEDICS: WHERE WE STAND AND WHERE WE ARE HEADING.

Three-dimensional printing is a technology in expansion in the medical field. It also presents many applications in orthopedics. Our review article aims to describe 3D printing, types of 3D printers, and its use in the orthopedic field. 3D models can be created using tomography scans. Those models can then be manipulated, even simulating surgeries. It is possible to print biomodels, which will help us understand deformities and plan surgeries. Orthopedic surgeons must be updated in these disruptive technologies that may help their daily practice. Level of Evidence V, Expert opinion.

A impressão 3D é uma tecnologia em expansão na medicina, possuindo diversas utilidades na ortopedia. O objetivo deste artigo de revisão é descrever o que é a impressão 3D, seus tipos e suas aplicações na ortopedia. Modelos em 3 dimensões podem ser criados a partir da tomografia computadorizada. Estes modelos podem ser manipulados em softwares específicos, onde inclusive cirurgias podem ser simuladas. Utilizando impressoras 3D podemos criar biomodelos que nos ajudam a compreender deformidades e planejar cirurgias. É importante que o ortopedista se mantenha atualizado nestas novas tecnologias disruptivas que podem auxiliar muito no seu dia a dia. Nível de Evidência V, Opinião do especialista.

Chondrosarcoma of bone: lessons from 46 operated cases in a single institution.

BACKGROUND: Bone chondrosarcomas are rare malignant tumors that have variable biologic behavior, and their treatment is controversial. For low-grade tumors, there is no consensus on whether intralesional en bloc resections are the best treatment. QUESTIONS/PURPOSES: We therefore compared patients with Grade 1 and Grade 2 primary central chondrosarcomas to (1) determine difference in survival and (2) local recurrence rates; and (3) determine any association of histological grade with some clinical and demographic characteristics. METHODS: We retrospectively reviewed 46 patients with grade 1 and 2 chondrosarcomas. There were 25 men and 21 women with a mean age of 43 years (range, 17-79 years). Minimum followup was 32 months (mean, 99 months; range, 32-312 months) for the patients who remained alive in the end of the study. Twenty-three of the tumors were intracompartmental (Enneking A); of these, 19 were Grade 1 and 4 were Grade 2. Twenty-three tumors were extracompartmental (Enneking B); of these, 4 were Grade 1 and 19 were Grade 2. Twenty-five patients underwent intralesional resection, 18 had wide resection, and three had amputations. RESULTS: The overall survival rate was 94% and the disease-free survival rate was 90%. Among the 23 Grade 1 tumors, we observed six local recurrences and none of these patients died; among the 23 Grade 2 tumors, 10 recurred and two patients died. Local recurrence negatively influenced survival. CONCLUSIONS: For lesions with radiographic characteristics of intracompartmental Grade 1 chondrosarcoma, we believe intralesional resection followed by electrocauterization and cement is the best treatment. When the imaging suggests aggressive (Grade 2 or 3) chondrosarcoma, then wide resection is promptly indicated.

PRIMARY LEIOMYOSARCOMA OF BONE: CLINICOPATHOLOGIC AND PROGNOSTIC FACTORS ANALYSIS IN A SINGLE INSTITUTION.

OBJECTIVE: Primary leiomyosarcoma of bone (PLB) is a rare type of malignant bone tumor considered as a variant of the spindle cell sarcomas (SCS). The objective of this study was to analyze the clinicopathologic and the prognostic factors of patients with PLB treated at a single institution. METHODS: We retrospectively reviewed the records of 22 patients with pathologically confirmed PLB. The data collected were: age, sex, tumor size and location, grade and stage of the disease and histopathologic features. Mean age was 45.5 years (range, 17 to 73 y). Location was: upper limb (27.3%), lower limb (68.2%) and pelvis (4.5%). Patients had high grade in 90.9% of the reports. Margins were negative in 77.3% of the cases. Histological reports describe spindly sarcomatous cells arranged in fascicles with increased vascular formation without osteoid or chondroid matrix production. On immunohistochemistry, smooth muscle actin and desmin where positive in all cases. RESULTS: Mean follow-up time was 73.5 months (range, 5.3 to 331.1 m). We found 22.7% of local recurrence (LR). Distant metastasis (DM) was reported in 9 (40.9%) patients. Lung metastasis was the only DM affected site. Overall survival (OS) rate in 5 years was 59.1%. Predictors of OS were LR and DM. CONCLUSIONS: PLB is an extremely rare malignant bone tumor that has a higher rate of DM and similar OS prognosis compared with other bone sarcomas. Level of Evidence IV, Case Series.

OBJETIVOS: O leiomiossarcoma primário do osso (LPO) é um tumor ósseo maligno raro, considerado uma variante do sarcoma de células fusiformes (SCF). O objetivo deste estudo foi fazer uma análise clínico-patológica e dos fatores de prognóstico dos pacientes diagnosticados com LPO tratados em uma instituição única. MÉTODOS: Foram analisados retrospectivamente os prontuários de 22 pacientes com diagnóstico confirmado de LPO. Os dados coletados foram: idade, sexo, tamanho e localização do tumor, grau histológico, estádio da doença e as características histopatológicas. A média de idade foi 45,5 anos (de 17 a 73 a). A localização foi: membro superior (27,3%), membro inferior (68,2%) e pelve (4,5%). Os pacientes apresentaram alto grau em 90,9% dos relatos. As margens foram livres em 77,3% dos casos. Os relatos histológicos descrevem células sarcomatosas finas e compridas, arranjadas em fascículos, com aumento da vascularização e sem produção de matriz osteoide ou condral. No estudo imuno-histoquímico, a actina do músculo liso e a desmina foram positivas em todos os casos. RESULTADOS: O tempo médio de seguimento foi 73,5 meses (de 5,3 a 331,1 m). Dos pacientes, 22,7% apresentaram recorrência local (RL). Metástase à distância (MD) foi reportada em 9 (40,9%) pacientes. O único local de MD foi o pulmão. O tempo médio de sobrevida em 5 anos foi de 59,1%. Os fatores preditivos de sobrevida global foram: RL e MD. CONCLUSÃO: O LPO é um tumor ósseo maligno extremamente raro que tem uma taxa maior de MD, com uma sobrevida global similar aos outros sarcomas ósseos. Nível de Evidencia IV, Série de Casos.

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.

Prognostic factors and expression of MDM2 in patients with primary extremity liposarcoma.

OBJECTIVE: The objective of this study was to investigate MDM2 (murine double minute 2) protein expression and evaluate its relationship with some anatomical and pathological aspects, aiming also to identify prognostic factors concerning local recurrence-free survival, metastasis-free survival and overall survival in patients with primary liposarcomas of the extremities. MATERIALS AND METHODS: Of 50 patients with primary liposarcomas of the extremities admitted to a Reference Service, between 1968 and 2004, 25 were enrolled in the study, following eligibility and exclusion criteria. RESULTS: The adverse factors that influenced the risk for local recurrence in the univariant analysis included male sex (P = 0.023), pleomorphic histological subtype (P = 0.027), and high histological grade (P = 0.007). Concerning metastasis-free survival, age less than 50 years (P = 0.040), male sex (P = 0.040), pleomorphic subtype (P < 0.001), and high histological grade (P = 0.003) had a worse prognosis. Adverse factors for overall survival were age under 50 years (P = 0.040), male sex (P = 0.040), pleomorphic subtype (P < 0.001), and high histological grade (P = 0.003). CONCLUSIONS: There was no correlation between immunohistochemically observed MDM2 protein expressions and the anatomical and pathological variables studied. The immunohistochemical expression of MDM2 protein was not considered to have a prognostic value for any of the surviving patients in this study (local recurrence-free survival, metastasis-free survival, or overall survival). The immunoexpression of MDM2 protein was a frequent event in the different subtypes of liposarcomas.

Immunohistochemical analysis by ki67 and idh1 in patients with chondrosarcoma / Análise imuno-histoquímica por meio de ki67 e idh1 em pacientes com condrossarcoma

ABSTRACT Objective: To perform an immunohistochemical evaluation using the IDH1 and Ki67 markers in patients who underwent treatment for chondrosarcoma in a reference service center in Brazil. Methods: Retrospective analytical observational study using medical records of patients diagnosed with chondrosarcoma. Besides the epidemiological and clinical profile, important variables for prognosis and correlation with immunohistochemical analysis results with Ki67 and IDH1 markers were evaluated. Results: Histopathological examinations by immunohistochemistry of 56 patients were analyzed, 52% of which were women, with the age group 20-60 years being more prevalent. Grade 1 and 2 histological subtypes corresponded to most chondrosarcomas. The femur, humerus, and tibia were the most frequent anatomical sites. Most tumors (59%) were larger than 8 cm. Ki67 expression was very low (< 10%) in 98% of patients. The analysis of IDH1 was positive in 43% of the cases. The correlation between IDH1 positivity and tumor size was statistically significant, but regarding survival, we observed no significance. Conclusion: Immunohistochemical analysis using IDH1 and Ki67 markers in patients with conventional chondrosarcoma is not useful for prognostic guidance. Level of Evidence II, Prognostic Assessment, Results of Immunohistochemical Tests and Correlation with Survival.

RESUMO Objetivo: Fazer uma avaliação imuno-histoquímica usando os marcadores IDH1 e Ki67 em pacientes que fizeram tratamento para condrossarcoma em um serviço de referência no Brasil. Métodos: Estudo retrospectivo, analítico e observacional de prontuários de pacientes com diagnóstico de condrossarcoma. Além do perfil epidemiológico e clínico, foram avaliadas variáveis importantes para o prognóstico e a correlação com os resultados da análise imuno-histoquímica utilizando os marcadores Ki67 e IDH1. Resultados: Foram analisados exames histopatológicos por imuno-histoquímica de 56 pacientes, dos quais 52% eram do sexo feminino. A faixa etária mais prevalente foi entre 20 e 60 anos. Os subtipos histológicos graus 1 e 2 corresponderam à maioria dos casos. Fêmur, úmero e tíbia foram os sítios anatômicos mais frequentes. A maioria dos tumores (59%) tinha tamanho superior a 8 cm. O Ki67 teve expressão muito baixa (< 10%) em 98% dos pacientes. Já a análise do IDH1 foi positiva em 43% dos casos. A correlação entre a positividade do IDH1 e o tamanho do tumor foi estatisticamente significativa; já em relação à sobrevida, não houve significância. Conclusão: A análise imuno-histoquímica por meio dos marcadores IDH1 e Ki67 em pacientes com condrossarcoma convencional não é útil para orientação prognóstica. Nível de Evidência II, Avaliação Prognóstica, Resultados de Exames Imuno-Histoquímicos e Correlação com Sobrevida.

PERIPHERALLY INSERTED CENTRAL CATHETERS IN ORTHOPEDIC PATIENTS: EXPERIENCE FROM 1023 PROCEDURES.

OBJECTIVES: The advantages of using a peripherally inserted central catheter (PICC) in hospitalized patients make this device very important for intravenous therapy. This study describes the use of PICCs at the Institute of Orthopedics and Traumatology at the Hospital das Clinicas da Faculdade de Medicina da Universidade de São Paulo over the last 10 years. METHODS: This retrospective study analyzed 1,057 medical records and included 1,023 medical files with complete information on the punctured vein, diagnosis, duration of catheterization, complications, and catheter tip positioning. RESULTS: Seven hundred and twenty PICCs (70.4%) were considered successfully positioned, and mean duration of catheterization was 34.3 days. The basilic vein was used in 528 (51.6%) patients, while 157 (15.4%) catheters were removed due to complications. No cases of catheter-related thrombosis or infection were found. Eight hundred and sixty-six (84.6%) patients completed their treatment with PICC in place. CONCLUSION: PICC is a safe intravenous device that can be successfully utilized for medium- and long-course intravenous therapy in hospitalized and discharged orthopedic patients. Level of Evidence IV; Case series.

OBJETIVOS: As vantagens da utilização do Cateter Central de Inserção Periférica (CCIP) no ambiente hospitalar faz com que esse cateter ocupe uma posição de destaque na terapia intravenosa. Este trabalho relata o uso do CCIP nos pacientes do Instituto de Ortopedia e Traumatologia do Hospital das Clinicas da Faculdade de Medicina da Universidade de São Paulo (IOT-HC-FMUSP) nos últimos 10 anos. MÉTODOS: Trata-se de um estudo retrospectivo, no qual foram analisados 1057 prontuários. Foram incluídos 1023 prontuários de pacientes submetidos à inserção do CCIP, em que foram analisados a veia puncionada, diagnóstico do paciente, tempo em que o paciente permaneceu com o cateter, complicações e posicionamento da ponta do CCIP. RESULTADOS: Setecentos e vinte CCIPs (70,4%) foram considerados adequadamente posicionados. O tempo médio de utilização do cateter foi de 34,3 dias. A veia basílica foi a mais utilizada em 528 (51,6%) pacientes. Cento e cinquenta e sete (15,4%) cateteres foram removidos devido a complicações. Nenhum caso de trombose ou infecção relacionada ao cateter foi observada. Oitocentos e sessenta e seis (84,6%) permaneceram com o CCIP até o final do tratamento. CONCLUSÃO: O CCIP é um dispositivo intravenoso seguro e pode ser utilizado para terapia intravenosa de média e longa duração em pacientes ortopédicos hospitalizados ou desospitalizados. Nível de Evidencia IV; Série de casos.

TIBIAL ADAMANTINOMA: ANALYSIS OF SEVEN CONSECUTIVE CASES IN A SINGLE INSTITUTION.

OBJECTIVE: Adamantinoma accounts for less than 1% of the primary bone neoplasms. The tibia is the most affected bone and it is predominant in male patients between the second and third decades of life. The objective of this study is to obtain epidemiological and clinical information on patients with adamantinoma of the tibia treated surgically between 1989 and 2016. METHODS: Retrospective series of seven patients diagnosed with adamantinoma of the tibia that underwent surgery at the orthopedic oncology service of our hospital. The information was obtained from the medical records and histopathological reports of our institution. RESULTS: A total of 2870 medical records with histological reports were evaluated. Seven cases of adamantinoma of the tibia were included. The mean age was 28.5 (17-49) years. We found a predominance of females (71.4%) and the most affected side was the left one, with four cases (57.1%). The biopsy revealed bone adamantinoma in four (57.1%) patients, while the diagnosis of the other patients was confirmed after the histological examination of the surgical specimen. All the patients underwent surgery as definitive treatment. No positive margins were reported. No local recurrence (LR) was reported and two patients had distant metastasis (DM). CONCLUSION: The prognosis of survival in cases of adamantinoma of the tibia is high. The rates of LR and DM were low. Surgical treatment with extensive tumor resection is the treatment of choice. Level of Evidence IV, Case Series.

OBJETIVO: O adamantinoma representa menos de 1% das neoplasias ósseas primárias. Afeta predominantemente a tíbia, em pacientes do sexo masculino entre a segunda e terceira décadas da vida. O objetivo deste trabalho é obter informação epidemiológica e clínica dos pacientes com adamantinoma da tíbia, tratados mediante cirurgia entre 1989 e 2016. MÉTODOS: Série retrospectiva de sete pacientes com diagnóstico de adamantinoma da tíbia, tratados cirurgicamente no serviço de oncologia ortopédica do nosso hospital. A informação foi obtida dos relatos clínicos e patológicos do instituto. RESULTADOS: Um total de 2870 prontuários com relatos anatomopatológicos foram revisados. Sete casos de pacientes com adamantinoma na tíbia foram encontrados. A média de idade foi de 28,5 anos (17-49). Encontramos predominância do sexo feminino (71,4%). O lado mais afetado foi o esquerdo, com quatro (57,1%) casos. A biópsia diagnosticou adamantinoma em 57,1% dos casos e o diagnóstico dos outros casos foi definido após exame da peça cirúrgica. Todos os pacientes receberam tratamento cirúrgico como terapia definitiva. Não foram reportadas margens comprometidas. Nenhum paciente apresentou recorrência local (RL). Dois pacientes apresentaram metástase à distância (MD). CONCLUSÃO: O prognóstico de sobrevida do adamantinoma da tíbia é alto. Apresenta taxas baixas de RL e MD. A cirurgia com ampla ressecção do tumor é o tratamento de escolha. Nível de Evidência IV, Série de Casos.