Sarcoma epitelióide: aspectos clínicos, fatores prognósticos e sobrevida

OBJETIVO: Descrever os aspectos epidemiológicos, clínicos, o tratamento e o prognóstico dos pacientes com sarcoma epitelióide. MÉTODOS: Revisão do prontuário de 25 pacientes matriculados no INCA com o diagnóstico de sarcoma epitelióide, no período de 05 de junho de 1987 à 15 de julho de 2005. RESULTADOS: A idade mediana foi de 33 anos, variando de 10 à 70 anos. A localização primária mais freqüente foi os membros superiores em doze casos (48%). O tamanho do tumor foi descrito em 19 casos e a mediana foi de 5cm, variando de 1,5 à 15cm. A cirurgia foi realizada em dezessete pacientes com onze amputações. As margens cirúrgicas estavam livres em quinze pacientes, comprometidas em três e em sete não foram relatadas. Seis receberam tratamento com algum tipo de quimioterapia e quatorze receberam tratamento com radioterapia com dose mediana de 46,5Gy. Recidiva local ocorreu em treze casos (52%). Recidiva nodal foi diagnosticada em nove pacientes (36%). Metástase pulmonar foi diagnosticada em sete pacientes (28%). Seis pacientes realizaram o tratamento oncológico na sua totalidade no INCA. Atualmente doze estão vivos sem doença, dois estão vivos com doença e onze pacientes foram a óbito. CONCLUSÃO: O sarcoma epitelióide é um subtipo raro de sarcoma de partes moles que apresenta alta taxa de recidiva local, regional e metástase à distância. Incide principalmente nas extremidades de pacientes jovens. O tratamento cirúrgico consiste em ressecção alargada com margens livres

Sarcoma epitelióide: aspectos clínicos, fatores prognósticos e sobrevida

OBJETIVO: Descrever os aspectos epidemiológicos, clínicos, o tratamento e o prognóstico dos pacientes com sarcoma epitelióide. MÉTODOS: Revisão do prontuário de 25 pacientes matriculados no INCA com o diagnóstico de sarcoma epitelióide, no período de 05 de junho de 1987 à 15 de julho de 2005. RESULTADOS: A idade mediana foi de 33 anos, variando de 10 à 70 anos. A localização primária mais freqüente foi os membros superiores em doze casos (48%). O tamanho do tumor foi descrito em 19 casos e a mediana foi de 5cm, variando de 1,5 à 15cm. A cirurgia foi realizada em dezessete pacientes com onze amputações. As margens cirúrgicas estavam livres em quinze pacientes, comprometidas em três e em sete não foram relatadas. Seis receberam tratamento com algum tipo de quimioterapia e quatorze receberam tratamento com radioterapia com dose mediana de 46,5Gy. Recidiva local ocorreu em treze casos (52%). Recidiva nodal foi diagnosticada em nove pacientes (36%). Metástase pulmonar foi diagnosticada em sete pacientes (28%). Seis pacientes realizaram o tratamento oncológico na sua totalidade no INCA. Atualmente doze estão vivos sem doença, dois estão vivos com doença e onze pacientes foram a óbito. CONCLUSÃO: O sarcoma epitelióide é um subtipo raro de sarcoma de partes moles que apresenta alta taxa de recidiva local, regional e metástase à distância. Incide principalmente nas extremidades de pacientes jovens. O tratamento cirúrgico consiste em ressecção alargada com margens livres

Cisto ósseo aneurismático parosteal

resumo O cisto ósseo aneurismático tem uma incidência de 0,14 a cada 100 mil indivíduos. O subtipo parosteal é o menos prevalente, representa 7% de todos. Apresentamos um paciente mas culino, 38 anos, com dor e abaulamento em braço direito havia oito meses. Diagnosticado previamente como tumor de células gigantes, teve sua lâmina revisada e então foi feito o diagnóstico de cisto ósseo aneurismático parosteal. O paciente foi tratado com infiltração intralesional de corticosteroide e calcitonina e evoluiu com melhoria clínica e radiológica já nas primeiras cinco semanas pós-operatórias

The usefulness of chemical-shift magnetic resonance imaging for the evaluation of osteoid osteoma

<div><p>Abstract Objective: The purpose of this study was to determine whether chemical-shift magnetic resonance imaging (MRI) could be useful in the diagnosis of osteoid osteoma when clinical and radiological tumor features are inconclusive. Materials and Methods: This retrospective study included 17 patients who underwent chemical-shift MRI for the evaluation of osteoid osteoma. For all patients, two musculoskeletal radiologists independently recorded signal intensities on in-phase and out-of-phase images in the nidus of the tumor, in abnormal-intensity bone marrow surrounding the lesion, and in normal-appearing bone marrow. For each region, relative signal intensity ratios were calculated by dividing out-of-phase by in-phase values. Relative ratios > 1 were considered indicative of neoplastic lesions. Statistical analysis was carried out to analyze the sample. Inter-observer and intra-observer agreement for each imaging method were assessed using intraclass correlation coefficients according to the Fleiss method and a value > 0.65 was considered to indicate substantial agreement. Results: The mean relative signal intensity ratios were 1.2 (range, 0.9-1.4) for the nidus and 0.35 (range, 0.11-0.66) for the surrounding tissue; these values differed significantly from the relative signal-intensity ratios for normal-appearing bone marrow (p < 0.05). Conclusion: Chemical-shift MRI is useful for the diagnosis and evaluation of osteoid osteoma.</p></div

Epidemiological study on giant cell tumor recurrence at the Brazilian National Institute of Traumatology and Orthopedics

ABSTRACT OBJECTIVE: Giant cell tumors are benign bone neoplasms that are relatively rare in adults and their biological behavior is still unpredictable. The incidence of local recurrence has presented variation between 0% and 65% in studies conducted worldwide, but few data are available on this complication in the Brazilian population. METHODS: Information on 155 patients with confirmed histological diagnoses of giant cell tumor who were treated in our institution's orthopedic oncology service between January 2000 and July 2014 was gathered. Demographic characteristics were evaluated and compared between patients who presented local recurrence during the clinical follow-up. RESULTS: Local recurrence was observed in 26 patients (16.7%), of whom 22 were female (84.6%). The most common site of local recurrence was the distal femur (38.4%). Eleven patients presented early recurrence, while 15 cases were diagnosed after 15 months, representing 42.3% and 57.7%, respectively. Metastases were identified in five patients (3.2%). CONCLUSION: Tumor-related factors did not show any increased incidence of local recurrence of giant cell tumors. Surgical treatment with an intralesional margin is a valid option for treating local recurrences and does not show any difference in disease-free survival in relation to other types of procedures. Clinical treatment is reserved for cases of unresectable tumors or when surgical treatment is impossible

Patient-derived osteosarcoma cells are resistant to methotrexate.

Osteosarcoma is the most common primary bone tumor in children and young adults. The median survival of osteosarcoma patients has not significantly improved since 1990, despite administration of different classes of chemotherapy agents, such as methotrexate, cisplatin and doxorubicin. Cancer stem cells (CSCs) are responsible for the resistance of osteosarcoma to chemotherapy and OCT4, SOX2 and SSEA4 have been used to identify CSCs in osteosarcoma. Here, we used low-passage patient-derived osteosarcoma cells and osteosarcoma cells directly isolated from patients before and after chemotherapy treatments to evaluate the effects of chemotherapy on stem cell markers expression. We demonstrate that primary osteosarcoma cells are resistant to methotrexate treatment and sensitive to cisplatin and doxorubicin in vitro. We also verified that cisplatin and doxorubicin reduce the expression of SOX2 and OCT4 in primary osteosarcoma cells whereas methotrexate does not alter SOX2 and OCT4 expression, however it increases SSEA4 expression in primary osteosarcoma cells. Finally, we found that, although the combination treatment cisplatin plus doxorubicin inhibited the in vivo growth of osteosarcoma cells in NOD-SCID gamma mice subcutaneously injected with SaOs2, the combination treatment cisplatin plus doxorubicin plus methotrexate did not inhibit the in vivo growth of these cells. These observations may provide an explanation for the poor response of osteosarcomas to chemotherapy and point to the need of reevaluating the therapeutic strategies for human osteosarcomas

An association between successful engraftment of osteosarcoma patient-derived xenografts and clinicopathological findings

Although osteosarcoma is a rare disease, with a global incidence rate estimated at 5.0/million/ year, it is the most frequent primary bone sarcoma in children and adolescents. In translational research, the patient-derived xenograft (PDX) model is considered an authentic in vivo model for several types of cancer, as tumorgrafts faithfully retain the biological characteristics of the primary tumors. Our goal was to investigate the association between PDX formation and clinical findings of osteosarcoma patients and the ability of the model to preserve in immunocompromised mice the characteristics of the parental tumor. A fresh sample of the patient tumor obtained from a representative biopsy or from surgical resection was implanted into nude mice. When tumor outgrowths reached ~1,500 mm 3 , fresh PDX fragments were re-transplanted into new hosts. Engraftment in mice was obtained after a latency period of 19-225 days (median 92 days) in 40.54% of the implanted samples. We confirmed the histopathological fidelity between the patient tumor and their respective established PDXs, including the expression of biomarkers. PDX take rate was higher in surgical resection samples, in post-chemotherapy surgical samples and in samples from patients with metastatic disease at presentation. In conclusion, we have shown that the osteosarcoma PDX model reliably recapitulates the morphological aspects of the human disease after serial passage in mice. The observation that more aggressive forms of osteosarcoma, including those with metastatic disease at presentation, have a higher efficiency to generate PDXs provides a promising scenario to address several unanswered issues in clinical oncology