ABSTRACT 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.

RESUMO 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.

RESUMO A osteonecrose multifocal é uma doença rara. O uso crônico de corticoides é considerado seu principal fator de risco. Em pacientes com insuficiência renal crônica é descrita a intoxicação por alumínio, causando osteomalácia e encefalopatia. A relação entre osteonecrose e toxicidade pelo alumínio já foi relatada em estudos que envolveram pacientes com insuficiência renal dialítica. A exposição ocupacional ao alumínio pode, raramente, ocasionar doenças pulmonares. Não existem casos relatados de lesões ósseas decorrentes da exposição a esse metal. Descrevemos neste artigo, o relato inédito de um paciente com osteonecrose multifocal associada à exposição ocupacional crônica ao alumínio. Nível de Evidência IV, Relato de Caso.

ABSTRACT Multifocal osteonecrosis is a rare disease; chronic use of corticosteroids is considered the main risk factor. Patients with chronic renal failure can develop aluminum toxicity, which can lead to osteomalacia and encephalopathy. An association between osteonecrosis and aluminum toxicity has been reported among patients with dialytic renal insufficiency. Occupational exposure to aluminum rarely causes lung disease and no cases of bone lesions resulting from exposure to this metal have been reported. In this manuscript, we describe a novel case of a patient with multifocal osteonecrosis associated with chronic occupational exposure to aluminum. Level of Evidence IV, Case Report.

RESUMO O lipossarcoma é um tipo comum de sarcomas em adultos, com incidência entre 15 e 20% entre os sarcomas. No entanto, o acometimento do estômago é raro. Relatamos um caso de um lipossarcoma primário gástrico gigante com apresentação clínica de hemorragia digestiva. Foi submetido a tratamento cirúrgico sem diagnóstico definitivo, apesar de três biópsias realizadas. Revisamos diagnósticos diferenciais, influência genética e estratégias diagnósticas e terapêuticas.

ABSTRACT Liposarcoma is one of the most common soft tissue sarcomas in adults, occurring in 15 to 20% of all patients with sarcoma. Primary liposarcoma of the stomach is rare. We report a case of patient with giant gastric liposarcoma who underwent surgery after a gastrointestinal bleeding. Preoperative hystopathological diagnosis was not established, even after three biopsy attempts. We discuss differential diagnosis, genetic causes, diagnosis strategies and treatment.

Objective: To present a series of 50 consecutive patients with non-metastatic extremity osteosarcoma, and attempt to correlate expression of the vascular endothelial growth factor (VEGF) protein in biopsy tissue to their prognosis regarding overall survival, disease-free survival and local recurrence.Methods: Fifty cases of non-metastatic osteosarcoma of the extremities treated between 1986 and 2006 at Instituto de Ortopedia e Traumatologia da Universidade de São Paulo, São Paulo, Brasil, were evaluated regarding expression of the VEGF protein. There were 19 females and 31 males. The mean age was 16 years old (range 5-28 years old) and the mean follow-up was 60.6 months (range 25-167 months). The variables studied were age, gender, anatomic location, type of surgery, surgical margins, tumor size, post chemotherapy necrosis, local recurrence, pulmonary metastasis and death.Results: Thirty-six patients showed VEGF expression on 30% or less cells (low), and the remaining 14 cases had VEGF expression above 30% (high). Among the 36 patients with low VEGF expression, nine developed pulmonary metastasis and four died (11.1%). Among the 14 patients with high VEGF expression, six developed pulmonary metastasis and three died (21.4%).Conclusion: There was no statistically significant correlation between the expression of VEGF and any of the variables studied. Level of Evidence IV, Therapeutic Study.

Lipoma arborescens é uma condição rara de moléstia intra-articular, usualmente monoarticular, caracterizada por extensa proliferação dos vilos sinoviais e hiperplasia da gordura subsinovial. O tecido sinovial é progressivamente substituído por células maduras de gordura na membrana sinovial. O presente trabalho é o relato de caso de uma condição rara de lipoma arborescens tanto intra-articular (glenoumeral) como da bursa subacromial-subdeltoide além de ruptura do tendão do supraespinhoso. As apresentações clínicas, histológicas e radiográficas assim como o tratamento são discutidos no presente estudo. A apresentação do caso contempla também a avaliação radiográfica, ressonância magnética e exame patológico. Apesar do lipoma arborescens ser uma condição rara, tal hipótese deve ser considerada frente a um caso com hiperproliferação sinovial e lipossubstituição da sinovial.

Lipoma arborescens is a rare intra-articular disease that is usually monoarticular and is characterized by extensive proliferation of the synovial villi and hyperplasia of the subsynovial fat. The synovial tissue is progressively replaced by mature fat cells in the synovial membrane. The present study reports a case of a rare condition of lipoma arborescens that was simultaneously intra-articular (glenohumeral joint) and in the subacromial-subdeltoid bursa, in association with a torn supraspinatus tendon. The clinical, histological and radiographic presentations and treatment are discussed here. The description of this case includes radiographic and magnetic resonance evaluations and pathological examination. Although lipoma arborescens is a rare condition, it should be taken into consideration in cases presenting synovial hyperproliferation and synovial fat replacement.

OBJETIVO: Verificar se havia risco de contaminação por células neoplásicas no trajeto da biópsia e estudar as alterações histológicas locais. MÉTODOS: Realizou-se um estudo prospectivo com trajetos de biópsias de pacientes operados por tumores musculoesqueléticos malignos no Instituto de Ortopedia e Traumatologia da Faculdade de Medicina da Universidade de São Paulo no período de abril de 2006 a abril de 2007. Foram estudados por histopatologia 25 casos. RESULTADOS: Houve implante de células neoplásicas em 32% dos trajetos. As alterações histológicas mais comuns nos casos positivos foram classificadas como: fibrose acentuada, componente inflamatório leve e neovascularização acentuada. CONCLUSÃO: Sugerimos a ressecção tradicional oncológica do trajeto junto com a peça.

OBJECTIVE: To investigate whether there is any risk of seeding by neoplasic cells along biopsy tracks and to study local histological abnormalities. METHODS: This was a prospective study on biopsy tracks in patients who underwent operations due to malignant musculoskeletal tumors between April 2006 and April 2007. RESULTS: Neoplasic cell implantation occurred in 32% of the tracks. The most common histological abnormalities in positive cases were classified as severe fibrosis, mild inflammatory component and severe neovascularization. CONCLUSION: We suggest that traditional oncological resection of the track should be carried out together with excision of the specimen.

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.

OBJETIVO: Avaliar os aspectos clínicos, diagnósticos, fatores de prognóstico e porcentagem de evolução dos casos de plasmocitoma para mieloma múltiplo. MATERIAS E MÉTODOS: Foram levantados 103 prontuários do Hospital das Clínicas da FMUSP, entre os anos de 1950 e 1998. Destes, 73 não foram utilizados por perda de seguimento ou por apresentarem diagnóstico diferente de plasmocitoma. RESULTADOS: Concluímos que a idade dos pacientes que evoluíram para mieloma múltiplo é inferior a dos pacientes que não evoluíram. A média do primeiro grupo foi de 52,3 ± 2,6 anos e a do segundo 62,6 ± 3,4 anos (média ± SEM; p=0,02). Não houve diferença estatística quanto ao sexo. Analisando pacientes com plasmocitoma que evoluiu para mieloma múltiplo, foi observada uma incidência maior de recidivas múltiplas (75%, p=0,049). Em ambos os grupos houve predominância de lesões da coluna vertebral. Não houve nenhuma diferença significativa entre os grupos com relação ao tempo de doença (desde o aparecimento dos sintomas até o diagnóstico) (p=0,20) e à sobrevida (p=0,34). Quanto ao tempo de evolução de plasmocitoma para mieloma, a média foi de 41 meses (DP=38,8), com uma taxa de evolução aproximadamente igual a 57%. CONCLUSÃO: Os pacientes que evoluíram para mieloma múltiplo são mais jovens. Não houve diferença significativa entre os dois grupos quanto ao sexo, tempo de doença e tempo de sobrevida. Em ambos os grupos a localização anatômica mais acometida foi a coluna vertebral. O tempo médio de evolução para mieloma múltiplo foi de 41 meses. Não foi possível calcular os fatores que influem na sobrevida dos pacientes com plasmocitoma e dos pacientes com plasmocitoma que evoluiu para mieloma múltiplo.

PURPOSE: To evaluate the clinical aspects, diagnoses, prognostic factors, and percent progression of plasmacytoma to multiple myeloma. MATERIALS AND METHODS: 103 medical records of patients suspected of plasmacytoma were surveyed covering the period between 1950 and 1998, and 30 were selected for analysis. Patients were classified into 2 groups: patients who did (n = 17) and did not (n = 13) progress to multiple myeloma. Comparative statistics regarding a variety of clincial aspects were developed. RESULTS: Patients who progressed to multiple myeloma were younger than those who did not (52.3 ± 2.6 vs 62.6 ± 3.4 years; mean ± SEM; P = 0.02). There were no significant differences in gender between groups. A higher incidence of multiple recurrence was observed in patients who progressed to multiple myeloma (75%, P = 0.049). Both groups showed a prevalence of vertebral column injuries. No significant differences were found between groups regarding the disease period (from the onset of symptoms until diagnosis) (P = 0.20) and survival (P = 0.34). The average time to progression from plasmacytoma to myeloma was 41 ± 39 months (mean ± SD), and the progression rate was 57%. CONCLUSION: Patients who progressed to multiple myeloma were younger than those who did not. No significant differences were found between groups regarding sex, time from symptom onset to diagnosis, and survival time. In both groups, the most affected anatomic location was the vertebral column, and most affected sex was male. The average time to progression to multiple myeloma was 41 months. It was not possible to determine the factors that influenced the survival of patients with plasmacytoma or for those who progressed to multiple myeloma.

FINALIDADE: Eeste estudo propõ-se a esclarecer as características do osteossarcoma e do sarcoma de Ewing, bem como definir o intervalo de tempo decorrido, no Brasil, entre o início dos sinais e dos sintomas e o diagnóstico definitivo, fornecendo subsídios para um diagnóstico precoce desses tumores. MÉTODO: Alguns aspectos dos diagnósticos clínicos do prontuário de 365 pacientes com menos de 30 anos de idade, portadores de osteossarcoma ou sarcoma de Ewing foram analisados de forma retrospectiva e comparativa, seguindo-se uma análise estatística. RESULTADOS: O tempo entre o início de sinais e sintomas e o diagnóstico foi de 5,25 meses no osteossarcoma e 8,1 meses no sarcoma de Ewing; o sintoma mais freqüente (89,5) nas duas neoplasias foi a dor localizada. CONCLUSÃO: O tempo para o diagnóstico de ambas as neoplasias é maior do que o observado em estatísticas norte-americanas e européias.

OBJECTIVE: The purposes of this study were to describe the early signs and symptoms of osteosarcoma and Ewing's sarcoma, identify symptoms that could be used to help differentiate the two types of tumors, and determine the time elapsed between the onset of signs and symptoms and the definitive diagnosis in our service, providing information and imputus for earlier diagnosis of these tumors. METHODS: A retrospective analysis of the medical dossiers of 365 patients under 30 years of age diagnosed with osteosarcoma or Ewing's sarcoma was performed, and the aspects of the clinical diagnosis were statistically analyzed and compared. RESULTS: The time between the onset of signs and the symptoms was 5.25 months for osteosarcoma and 8.1 months for Ewing's sarcoma, and the most frequent (89.5%) early symptom of osteosarcoma and Ewing's sarcoma was local pain. Symptoms that might aid diagnosis included early local volume increase and the presence of fever. CONCLUSION: The time until diagnosis of both neoplasias was higher than that reported for North America and Europe. Education of the lay public and medical professionals regarding suspicious early signs and symptoms might shorten the delay of diagnosis.

O Tumor de Células Gigantes (TGC), é um tumor benigno, com ocorrência de recidiva em cerca de 20-34% dos casos. A localização habitual é na epífise dos ossos longos. O objetivo deste trabalho é relatar um caso de TGC em vértebra, com diagnóstico inicial de Cisto Ósseo Aneurismático (COA), e discutir os diagnósticos diferenciais possíveis, correlacionando-os com as características dos exames de imagens. Paciente com 37 anos, do sexo feminino, com quadro clínico de dor na coluna e paraparesia há 2 meses. O diagnóstico inicial foi de COA. Na radiografia a lesão era lítica, com, erosão e destruição da cortical..A tomografia e ressonância evidenciavam lesão cística e hemorrágica, com extensão para partes moles. A revisão das lâminas e análise do espécime cirúrgico ressecado, submetido à coloração HE e imunoistoquímica com marcador para p53, permitiram o diagnóstico de TGC. Muitas lesões, benignas apresentam células gigantes multinucleadas. Os exames de imagem nem sempre permitem um diagnóstico conclusivo. O diagnóstico definitivo de TGC depende do exame anatomopatológico, com avaliação cuidadosa do componente estromal e a imunoexpressão positiva para a proteína p53. O tratamento é a ressecção cirúrgica, com margens amplas seguida por instrumentação nos casos de tumor localizados na coluna vertebral.

Giant Cell Tumor (GCT) is a benign tumor, with a recurrence rate of about 20% - 34% of the cases. It is usually located at long bone epiphysis. The objective of this study is to report a GCT case in a vertebra, which was early diagnosed as Aneurysmal Bone Cyst (ABC), and to discuss potential differential diagnosis, correlating them to patterns shown on imaging tests. This patient is a 37 year-old female, with clinical picture of pain in spine and paraparesis that started two months earlier. An early diagnosis of ACB was delivered. At X-ray, the injury was lithic, with erosion and cortical destruction. Tomography and resonance showed a cystic and hemorrhagic injury, extending to soft parts. Slides review and the analysis of dried surgical matter submitted to HE and immunohistochemical staining with p53 marker allowed for GCT diagnosis. Many benign lesions present with multi-nucleated giant cells. Imaging tests not always enable a conclusive diagnosis. A definite GCT diagnosis depends on anatomicopathological test, with careful evaluation of the stromal component and positive immunoexpression for p53 protein. Treatment is delivered as surgical resection, with wide margins, followed by instrumentation in cases of tumors located at spine.

OBJETIVO: Comparar o método da criopreservação de enxertos ósseos (- 80º C) com o da conservação em glicerol a 98% em temperatura ambiente (10º C a 35º C), testando os efeitos antibacterianos e antifúngicos do glicerol a 98% e analisando comparativamente as alterações histológicas verificadas e decorrentes do emprego dos dois métodos. MÉTODO: Este estudo foi constituído de 30 amostras de tecido ósseo trabecular provenientes de 10 pacientes, submetidos a Artroplastia Total do Quadril. Cada cabeça femoral forneceu 3 amostras e estas foram divididas aleatoriamente em 3 grupos, a saber: controle, criopreservado e conservado em glicerol a 98% à temperatura ambiente durante um ano. As amostras foram encaminhadas à Anatomia Patológica para estudo histomorfologico, de viabilidade celular, e microbiológico. Os resultados foram analisados estatisticamente pelo método de McNemar, com índice de significância de 0,05. RESULTADOS: A análise dos valores obtidos no teste de McNemar na comparação das distribuições de probabilidades das variáveis da histomorfologia (osso maduro ou lamelar, osso imaturo e necrose) e da viabilidade celular (osteoblastos e osteoclastos) indica não haver diferença entre as distribuições das variáveis nas três condições experimentais. A análise microbiológica da solução de glicerol a 98% e dos fragmentos ósseos das amostras armazenadas durante um ano em temperatura ambiente não apresentou crescimento bacteriano ou de fungos. As espécimens do grupo controle foram analisadas histológica e microbiologicamente logo após a coleta das mesmas. CONCLUSÃO: O método de conservação de enxertos ósseos mantidos no glicerol a 98% em temperatura ambiente (10ºC a 35ºC) foi similar ao da criopreservação quanto à preservação da matriz óssea e à ausência de crescimento de bactérias ou fungos.

OBJECTIVE: To compare the bone graft cryopreservation method (at -80ºC) with a preservation method using a 98% glycerol solution at room temperature (10ºC-35ºC), by testing the antibacterial and fungal effects of 98% glycerol and comparatively analyzing the observed histological changes resulting from the use of both methods. METHOD: This study was of 30 samples of trabecular bone tissue from 10 patients undergoing total hip arthroplasty. Each femoral head provided 3 samples that were randomized into 3 groups, namely, the control group, the cryopreserved group, and the group preserved in a 98% glycerol at room temperature for 1 year. The samples were submitted to histomorphologic, cell feasibility, and microbiologic analyses. The results were statistically analyzed using the McNemar test, with a statistical significance index of 0.05. RESULTS: Values obtained using the McNemar test to compare probability distributions of histomorphologic variables (mature or lamellar bone, immature bone, and necrosis) and cell feasibility (osteoblasts and osteoclasts) indicated that there is no difference between the distributions of variables under the 3 experimental conditions. Microbiological analysis of the 98% glycerol solution and bone fragments from samples stored for 1 year at room temperature did not show bacterial or fungal growth. The histological and microbiological investigation were performed at 2 different time points: immediately after the sample processing and after 1 year. CONCLUSION: The method used to preserve bone grafts kept in 98% glycerol at room temperature (10ºC-35ºC) was similar to cryopreservation in terms of bone matrix preservation; no bacteria or fungi were found in the samples.