Prognostic significance of mast cell and microvascular densities in malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1.

Malignant peripheral nerve sheath tumors (MPNSTs) are rare and aggressive soft tissue sarcomas with a significant susceptibility to metastasize early in their course. Pathogenesis is yet to be fully elucidated. Recently, the essential role of mast cells in the tumor onset of neurofibromatosis type 1 (NF1)-associated neurofibromas and MPNSTs was confirmed in both experimental and human studies. In this study, we investigate mast cell density (MCD), microvascular density (MVD), and proliferation index (Ki-67) in MPNST. A secondary aim was to correlate histological staining to clinical data and survival in patients with and without NF1. In total, 34 formalin-fixed paraffin-embedded MPNST tissues from 29 patients were eligible. MCD, MVD, and Ki-67 labeling index (LI) were analyzed in all stained tissues by a computer-based quantitative algorithm (Aperio ImageScope). In addition, chart review was performed for clinical data and survival analysis. Overall, MCD, MVD, and Ki-67 LI were evenly distributed throughout tumor tissue. There was a negative correlation of NF1 status (affected, P = 0.037), tumor size (>10 cm, P = 0.023), and MVD in the tumor periphery (higher tercile, P = 0.002) to survival. Multivariate analysis confirmed the association of MVD in the tumor periphery (higher tercile, P = 0.019) with a decreased overall survival. Diverse mast cell and microvascular distributions suggest that angiogenesis in MPNST occurs independently. The role of mast cells in tumor progression is unclear and lacks prognostic value. Higher MVD has prognostic significance with possible therapeutic implications in MPNST.

Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1.

OBJECTIVE: In this study, we review the institution's experience in treating malignant peripheral nerve sheath tumors (MPNSTs). A secondary aim was to compare outcomes between MPNSTs with and without neurofibromatosis type 1 (NF1). METHODS: Ninety-two patients with MPNSTs, over a period of 20 years, were reviewed. A retrospective chart review was performed. The median age was 43.5 years (range, 3-84 years) and 55.4% were female; 41 patients (44.6%) had NF1-associated tumors. RESULTS: Mean tumor sizes were 15.8 ± 8.2 cm and 10.8 ± 6.3 cm for patients with and without NF1, respectively. Combined two- and five-year overall survival was 48.5% and 29%. Multivariate analysis confirmed the association of tumor size greater than 10 cm (hazard ratio (HR) 2.99; 95% confidence interval (CI) 1.14-7.85; p = 0.0258) and presence of NF1 (HR 3.41; 95%CI 1.88-6.19; p < 0.001) with a decreased overall survival. CONCLUSION: Tumor size and NF1 status were the most important predictors of overall survival in our population.

Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1 / Tumor maligno da bainha de nervo periférico com e sem Neurofibromatose tipo 1

ABSTRACT Objective In this study, we review the institution’s experience in treating malignant peripheral nerve sheath tumors (MPNSTs). A secondary aim was to compare outcomes between MPNSTs with and without neurofibromatosis type 1 (NF1). Methods Ninety-two patients with MPNSTs, over a period of 20 years, were reviewed. A retrospective chart review was performed. The median age was 43.5 years (range, 3–84 years) and 55.4% were female; 41 patients (44.6%) had NF1-associated tumors. Results Mean tumor sizes were 15.8 ± 8.2 cm and 10.8 ± 6.3 cm for patients with and without NF1, respectively. Combined two- and five-year overall survival was 48.5% and 29%. Multivariate analysis confirmed the association of tumor size greater than 10 cm (hazard ratio (HR) 2.99; 95% confidence interval (CI) 1.14–7.85; p = 0.0258) and presence of NF1 (HR 3.41; 95%CI 1.88–6.19; p < 0.001) with a decreased overall survival. Conclusion Tumor size and NF1 status were the most important predictors of overall survival in our population.

RESUMO Objetivo Relatamos a experiência institucional no tratamento de tumores malignos da bainha de nervo periférico (TMBNP) e comparamos o prognóstico entre pacientes com e sem neurofibromatose tipo 1 (NF1). Métodos Foram incluídos neste estudo 92 pacientes num período de 20 anos. Foi realizada uma análise retrospectiva dos prontuários, das características do tumor e do tratamento. A idade mediana era 43,5 anos (variação 3–84 anos) e 55,4% dos pacientes eram mulheres; 41 pacientes (44,6%) tinham tumores associados à NF1. Resultados O diâmetro médio dos tumores era 15,8 ± 8,2cm e 10,8 ± 6,3cm para pacientes com e sem NF1, respectivamente. A sobrevida combinada em 2 e 5 anos foi de 48,5% e 29%. A análise multivariada confirmou que o tamanho do tumor acima de 10cm (hazard ratio (HR) 2.99; 95% intervalo de confiança (IC) 1.14–7.85; p = 0.0258) e a presença de NF1 (HR 3.41; 95%IC 1.88–6.19; p < 0.001) estão associados a uma pior sobrevida. Conclusões O tamanho do tumor e a associação com NF1 foram os preditores mais importantes de sobrevida na nossa população.

Survival rate and perioperative data of patients who have undergone hemipelvectomy: a retrospective case series.

BACKGROUND: Hemipelvectomy is a major orthopedic surgical procedure indicated in specific situations. Although many studies discuss surgical techniques for hemipelvectomy, few studies have presented survival data, especially in underdeveloped countries. Additionally, there is limited information on anesthesia for orthopedic oncologic surgeries. The primary aim of this study was to determine the survival rate after hemipelvectomy, and the secondary aims were to evaluate anesthesia and perioperative care associated with hemipelvectomy and determine the influence of the surgical technique (external hemipelvectomy [amputation] or internal hemipelvectomy [limb sparing surgery]) on anesthesia and perioperative care in Brazil. METHODS: This retrospective case series collected data from 35 adult patients who underwent hemipelvectomy between 2000 and 2013. Survival rates after surgery were determined, and group comparisons were performed using the Kaplan-Meier method and the log-rank test. Mantel-Cox test and multiple linear regression analysis with stepwise forward selection were performed for univariate and multivariate analyses, respectively. RESULTS: Mean survival time was 32.8 ± 4.6 months and 5-year survival rate was 27 %. Of the 35 patients, 23 patients (65.7 %) underwent external hemipelvectomy and 12 patients (34.3 %) underwent internal hemipelvectomy. The survival rate was significantly higher in patients with bone tumors than in those with soft tissue sarcomas (P = 0.024). The 5-year cumulative probability of survival was significantly lower in patients who underwent external hemipelvectomy than in those who underwent internal hemipelvectomy (P = 0.043). In the univariate and multivariate analyses, only advanced disease stage (3 and 4) was identified as a significant independent predictor of reduced survival (P = 0.0003). Balanced general anesthesia combined with epidural block was the most frequent anesthesia technique. Median intraoperative crystalloid volume and red blood cell transfusions were 3500 mL and 2 units, respectively. CONCLUSIONS: Overall mean survival time after hemipelvectomy was 32.8 months. Advanced disease stage might be independently associated with reduced survival. Smaller amounts of fluids and transfusions were administered and time to discharge was shorter. Acute and chronic pain as well as wound complications are still important challenges in hemipelvectomy.

Analysis of HFE genes C282Y, H63D, and S65D in patients with hyperferritinemia from northeastern Brazil.

BACKGROUND: Hereditary hemochromatosis (HH) is a genetic disease caused by the high absorption and deposition of iron in several organs. This accumulation results in several clinical complications such as cirrhosis, arthritis, cardiopathies, diabetes, sexual disorders, and skin darkening. The H63D and C282Y mutations are well defined in the HH etiology. The objective of this article is identification of the H63D and C282Y mutations in the HFE protein gene and the frequency assessment of these mutations in patients with persistent increase of serum ferritin in patients from Natal City from state of Rio Grande do Norte, located in northeastern Brazil. RESULTS: Of the 299 patients studied for C282Y and H63D, 48.49% showed absence of mutation and 51.51% showed some sort of mutation: heterozygous C282Y mutation in 4.35% patients, homozygous C282Y mutation in 2.67% patients, heterozygous H63D mutation in 31.44% patients, homozygous H63D mutation in 8.03% patients, and heterozygous for the mutation in both genes (C282Y/H63D) in 5.02% patients. The S65C mutation was studied in 112 patients and heterozygous mutation (S65D/WT) in 2.67% of patients and double mutation (H63D/S65C) in 1.78% of patients were observed. CONCLUSION: Due to the high prevalence of hemochromatosis, its genetic diagnosis has become a challenge, especially in the high-risk group.

Carcinomatous degeneration of pilonidal cyst with sacrum destruction and invasion of the rectum.

Malignant degeneration of pilonidal cysts is rare. The most common histologic type is the squamous cell carcinoma, triggered by the chronic inflammatory process. The growth of the lesion is typically slow. The diagnosis must be early and the appropriate treatment is ample surgical resection, including the presacral fascia. In some cases, as the present one, the diagnosis is made at a stage when the disease has progressed and invaded adjacent structures. In these cases the surgery involves multiple organ resection. We report the case of a patient with carcinomatous degeneration of pilonidal cyst, with bulky disease that extended up to the wall of the rectum. The treatment was extended resection, sacrectomy and abominoperineal resection of the rectosigmoid with permanent colostomy.

Carcinomatous degeneration of pilonidal cyst with sacrum destruction and invasion of the rectum / Degeneracao carcinomatosa do cisto pilonidal com destruicao do sacro e invasao da parede do reto

Malignant degeneration of pilonidal cysts is rare. The most common histologic type is the squamous cell carcinoma, triggered by the chronic inflammatory process. The growth of the lesion is typically slow. The diagnosis must be early and the appropriate treatment is ample surgical resection, including the presacral fascia. In some cases, as the present one, the diagnosis is made at a stage when the disease has progressed and invaded adjacent structures. In these cases the surgery involves multiple organ resection. We report the case of a patient with carcinomatous degeneration of pilonidal cyst, with bulky disease that extended up to the wall of the rectum. The treatment was extended resection, sacrectomy and abominoperineal resection of the rectosigmoid with permanent colostomy.

A degeneração maligna do cisto pilonidal é rara. O tipo histológico mais freqüente é o carcinoma epidermóide e tem como fator desencadeante o processo inflamatório crônico. O tumor é de crescimento lento. O diagnóstico deve ser precoce e o tratamento cirúrgico adequado é a ressecção ampla incluindo a fáscia pré-sacral. Em alguns casos, como o que apresentamos, o diagnóstico é feito numa fase em que a doença progrediu e invadiu as estruturas adjacentes. Nestes a cirurgia necessária envolve a ressecção multiorgânica. Apresentamos paciente com degeneração carcinomatosa do cisto pilonidal, com doença volumosa que se estendia até a parede do reto. O tratamento realizado foi ressecção alargada, sacralectomia e ressecção abominoperineal do retossigmóide com colostomia definitiva.

Flow cytometry immunophenotyping evaluation in acute lymphoblastic leukemia: correlation to factors affecting clinic outcome.

The authors conducted a flow cytometry immunophenotyping study in patients with acute lymphoblastic leukemia (ALL) from Natal, Rio Grande do Norte, Brazil. The patients (n = 126) were newly diagnosed using a panel of monoclonal antibodies: CD1a, CD2, CD3, CD4, CD7, CD8, CD10, CD13, CD33, CD14, CD19, CD22, CD79a, CD117, CD34, anti-IgM, anti-TdT, anti-HLA-Dr, and anti-human kappa and lambda light chains. Additional data, such as patients' age and gender, clinical and laboratory findings such as presence of tumor masses, lymphadenopathy, hepatomegaly, splenomegaly, leukemic infiltration in the central nervous system (CNS) were also investigated. Results showed that 56.7% of the cases were B-lineage ALL and 55% were T-cell ALL. Also, we found that males were more affected by the disease, regardless of immunological classification. The correlation between age and immunological subtypes showed that the B-lineage ALL occurred more frequently in patients aged under 15 while the T-cell ALL subtype was more frequent in adults. Immunophenotypic profiles and morphological subtypes showed a direct correlation between L3 subtype and B-lineage ALL, while L1 and L2 subtypes correlated more often with B-cell lineage and T-cell ALL, respectively. Correlation analysis between immunophenotypic and clinical profiles showed that T-cell ALL was more associated with a higher incidence of lymphadenopathy, hepatomegaly, splenomegaly and CNS leukemic infiltration, also showing a greater blast cell count in peripheral blood than the other subgroups. The presented data suggest that immunophenotyping is an important method in the diagnosis, monitoring and prognostic assessment in determining the pathological mechanisms of evolution of ALL.

Reconstrução de extremidades com retalho livre de fíbula após ressecções oncológicas / Extremity reconstruction using a free fibula flap after oncological resection

INTRODUÇÃO: O tumor primário de ossos longos é raro, correspondendo de 0,2% a 1% dos tumores malignos. No passado, a amputação era o tratamento padrão, ocasionando grande impacto na morbidade e na mortalidade desses pacientes. Com o avanço das técnicas cirúrgicas e o envolvimento multidisciplinar, a cirurgia conservadora dos membros tornou-se o tratamento de escolha, sendo a reconstrução com retalho microcirúrgico de fíbula a mais utilizada. Este trabalho tem como objetivo apresentar a experiência do Instituto Nacional de Câncer (INCA) nas reconstruções de membros com retalho microcirúrgico de fíbula após ressecções de tumores de ossos longos. MÉTODO: Foi realizada análise retrospectiva de 7 casos de retalho livre de fíbula operados no INCA, no período de 1997 a 2009, para reconstrução de defeitos de extremidades após ressecções de tumores ósseos. Foram avaliados os seguintes parâmetros: sexo, idade, diagnóstico, localização do tumor, tipo e tamanho da ressecção, tipo e tamanho da reconstrução, vasos utilizados para anastomose, complicações pós-operatórias, estado da doença na última consulta, seguimento e tempo até deambulação. RESULTADOS: No total, 7 pacientes com média de idade de 11,8 anos (variando de 5 anos a 14 anos) foram submetidos a reconstrução de extremidades com retalho livre de fíbula, com 100% de viabilidade e consolidação óssea. As lesões eram localizadas em fêmur, tíbia ou úmero. O tumor mais comum foi o osteossarcoma. O tempo médio de retorno à deambulação foi de 14,7 meses. CONCLUSÕES: O uso do retalho livre de fíbula é uma excelente alternativa para reconstrução de membros, apresentando alta taxa de consolidação óssea, deambulação precoce, boa funcionalidade e baixa taxa de complicações.

BACKGROUND: Primary tumors of the long bones are rare, accounting for 0.2-1% of malignant tumors. In the past, amputation was the standard treatment and had a large impact on patient morbidity and mortality. With advances in surgical techniques and multidisciplinary involvement, conservative surgery of the limbs has become the treatment of choice, and reconstruction using a microsurgical fibula flap is the most commonly used technique. In this study, we aimed to present the experience of the National Cancer Institute (INCA) with limb reconstruction using a microsurgical fibula flap following tumor resection from the long bones. METHODS: We retrospectively analyzed 7 cases of free fibular flap surgery at the INCA from 1997 to 2009 for the reconstruction of defects of the extremities after bone tumor resection. We evaluated the following parameters: gender, age, diagnosis, tumor location, resection size and type, reconstruction size and type, vessels used for the anastomosis, postoperative complications, disease status at the last visit, follow-up, and time to ambulation. RESULTS: Seven patients with a mean age of 11.8 years (range, 5-14 years) underwent extremity reconstruction with a free fibula flap with 100% bone viability. The lesions were located within the femur, tibia, or humerus. Osteosarcoma was the most common tumor type. The average return to ambulation was 14.7 months. CONCLUSIONS: The use of a free fibula flap is an excellent alternative for limb reconstruction and features a high bone healing rate, early ambulation, good functionality, and a low complication rate.

[Epithelioid sarcoma: clinical behavior, prognostic factors and survival]. / Sarcoma epitelióide: aspectos clínicos, fatores prognósticos e sobrevida.

OBJECTIVE: To relate the clinical characteristics and evaluations of patients with epithelioid sarcomas. METHODS: Careful analysis of 25 epithelioid sarcoma cases registered in Instituto Nacional do Cancer between june 1987 and july 2005. RESULTS: Mean age at diagnosis was 33 years old, ranged from 10 to 70. The primary site of presentation was the upper extremity in twelve patients (48%). The size of the tumor was given in 19 cases, with the mean size of 5 cm, while they ranged from 1.5 to 15 cm. Surgery was made in 17 patients, with eleven amputation. Tumors margins were free in fifteen patients, positive in three and in seven were not studied. Six received any type of chemotherapy and 14 received treatment with radiotherapy with mean dose of 46,5 Gy. Local recurrence occurred in thirteen cases (52%). Nodal spread was diagnosed in nine (36%). Pulmonary metastases were diagnosed in seven patients (28%). Six patients underwent cancer treatment in its entirety at the National Institute of Cancer. At present twelve patients are alive without disease, two have disease and eleven patients have died. CONCLUSION: Epithelioid sarcoma is a rare subset of soft tissue sarcoma with high rate of local recurrence, regional node and distant metastases. Occurs predominantly in young patients, mainly on the superior member extremities. Surgical treatment of epithelioid sarcoma consists of early wide local resection to negative microscopic margins. These patients require carefully follow-up to evaluate local recurrence, nodal metastases, and pulmonary metastases.

Sarcoma epitelióide: aspectos clínicos, fatores prognósticos e sobrevida / Epithelioid sarcoma: clinical behavior, prognostic factors and survival

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 por cento). 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 por cento). Recidiva nodal foi diagnosticada em nove pacientes (36 por cento). Metástase pulmonar foi diagnosticada em sete pacientes (28 por cento). 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.

OBJECTIVE: To relate the clinical characteristics and evaluations of patients with epithelioid sarcomas. METHODS: Careful analysis of 25 epithelioid sarcoma cases registered in Instituto Nacional do Cancer between june 1987 and july 2005. RESULTS: Mean age at diagnosis was 33 years old, ranged from 10 to 70. The primary site of presentation was the upper extremity in twelve patients (48 percent). The size of the tumor was given in 19 cases, with the mean size of 5cm, while they ranged from 1,5 to 15cm. Surgery was made in 17 patients, with eleven amputation. Tumors margins were free in fifteen patients, positive in three and in seven were not studied. Six received any type of quimiotherapy and 14 received treatment with radiotherapy with mean dose of 46,5 Gy. Local recurrence occurred in thirteen cases (52 percent). Nodal spread was diagnosed in nine (36 percent). Pulmonary metastases were diagnosed in seven patients (28 percent). Six patients underwent cancer treatment in its entirety at the National Institute of Cancer. At present twelve patients are alive without disease, two have disease and eleven patients have died. CONCLUSION: Epithelioid sarcoma is a rare subset of soft tissue sarcoma with high rate of local recurrence, regional node and distant metastases. Occurs predominantly in young patients, mainly on the superior member extremities. Surgical treatment of epithelioid sarcoma consists of early wide local resection to negative microscopic margins. These patients require carefully follow-up to evaluate local recurrence, nodal metastases, and pulmonary metastases.

Culex mosquitoes (Diptera: Culicidae) egg laying in traps loaded with Bacillus thuringiensis variety israelensis and baited with skatole.

The Southern house mosquito, Culex quinquefasciatus, is an important human health pest as a vector of several pathogens, including agents of lymphatic filariasis and arboviruses like West Nile virus. We conducted preliminary experiments in Recife, Brazil, to explore applications of Culex oviposition attractants in combination with Bacillus thuringiensis variety israelensis (Bti) in an attract-and-kill approach. Simple, cost-effective oviposition traps, BR-OVT, loaded with Bti and baited with or without attractant, were deployed in 10 homes for 30 d in 2 consecutive yr. Significantly higher numbers of egg rafts were deposited in traps baited with skatole or infusion than the control water traps. In the first year, 2006, significantly higher numbers of eggs were deposited in infusion-baited traps, particularly in the first 15 d of the experiment, than in skatole traps, but in the following year no significant difference was observed between synthetic and natural attractants. The tests strongly demonstrate that skatole or infusion can be used to enhance the number of egg rafts deposited on Bti-treated oviposition traps.

Avaliação da eficácia das reações de imunofluorescência indireta e hemoaglutinação indireta na investigação sorológica para toxoplasmose em parturientes e recén-natos / Efficacy evaluation of the indirect immunofluorescence and indirect hemaglutination reaction in pregnancy and new born serologic toxoplasmosis investigation

A toxoplasmose é uma parasitose prevalente em todo o mundo, quando adquirida durante a gestação pode ser transmitida ao feto e causar, entre outros problemas, atraso no desenvolvimento neuropsicomotor, déficit visual e de audição. A prevenção da transmissão vertical deve ser iniciada antes da concepção ou o mais precocemente possível durante o exame pré-natal. Neste trabalho, foi realizado um estudo objetivando a avaliação da soroprevalência da toxoplasmose em parturientes e seus recém-natos (RN),atendidos na Maternidade Escola Januário Cicco da UFRN, através de um estudo comparativo entre as técnicas da hemaglutinação indireta (HAI) e a imunofluorescência indireta (IFI). Paralelamente, também, se procurou padronizar a técnica de IFI, utilizando-se a coleta de sangue capilar com impregnação em papel de filtro. Das amostras sanguíneas de 63 parturientes e respectivos RN avaliadas por IFI, obteve-se uma reatividade para toxoplasmose de 30,2% e de 3,2%, respectivamente. A utilização da IFI empregando-se a coleta de sangue capilar com impregnação em papel de filtro apresentou baixa sensibilidade (71,4%), mostrando a necessidade de se intensificar os estudos para completar a padronização desse método. No entanto, foi observada uma boa correlação entre a IFI e a HAI. Os casos de IFI para cadeia pesada das imunoglobulina humana (IgH) apresentaram elevados títulos para IgG específica na maioria dos casos, sendo que três destes apresentaram reatividade para a IgM. Estes dados mostram a importância da investigação sorológica para toxoplasmose nesse grupo e pacientes

Sarcoma epitelióide: análise de 15 casos tratados no INCA / Epithelioid sarcoma: analyses of 15 cases treated in INCA

Objetivo: Descrever os aspectos epidemiológicos, clínicos, anatomopatológicos e o tratamento dos pacientes comsarcoma epitelióide matriculados no Instituto Nacional de Câncer, comparando-os com os resultados da literatura.Pacientes e Método: Revisão do prontuário de 15 pacientes matriculados no INCA com diagnóstico de sarcomaepitelióide, no período de 1984 a 2003 e revisão da literatura. Resultados: A idade mediana foi de 28 anos,variando de 3 - 69 anos. Foram 8 pacientes do sexo feminino e 7 do sexo masculino. A localização mais freqüentefoi a mão, em 6 casos. O tamanho do tumor foi descrito em 6 casos e a mediana foi de 5 cm, variando de 4 - 10cm. A primeira manifestação clínica referida em 100 por cento dos casos foi a presença de nódulo. Em um paciente aapresentação foi multicêntrica com doença primária no antebraço e com focos de doença na região lombar eparede torácica. Dos 15 pacientes matriculados, 13 foram tratados no INCA e 2 abandonaram o tratamento. Acirurgia foi empregada em 100 por cento dos pacientes, sendo 8 pacientes submetidos a amputação/desarticulação e 5 pacientes a ressecção local com preservação do membro. Quatro pacientes foram tratados com Radioterapia,sendo 3 adjuvantes e 1 paliativa. O tratamento quimioterápico foi empregado em 3 pacientes, sendo 1 neoadjuvante,1 adjuvante e 1 paliativo. A recidiva local ocorreu em 2 dos 13 pacientes, sendo realizada a ressecção local darecidiva em 1 e a amputação do coto em outro. Metástase pulmonar ocorreu em 1 paciente sendo tratado comtoracotomia e ressecção dos nódulos. Em 2 pacientes a recidiva foi linfonodal, sendo realizada a linfadenectomiaem ambos. Atualmente 10 pacientes estão vivos, sendo 1 com doença em tratamento com quimioterapia e 3pacientes foram a óbito. Conclusão: Sarcoma epitelióide é um subtipo raro de sarcoma de partes moles, incidindopredominantemente sobre as extremidades de pacientes jovens, principalmente nos membros superiores, tem umcomportamento clínico atípico comparado com os outros sarcomas de alto grau. O diagnóstico é realizado combase em características histológicas e imunohistoquímicas para marcadores epiteliais...

Artrodese do punho com placa de compressäo dinâmica / Wrist arthrodesis with dynamic compression plate

Foram avaliados sete casos de artrodese de punho (cinco seqüelas de trauma e dois tumores de células gigantes da metáfise distal do rádio), com fixaçao interna, com placa de compressao dinâmica, segundo a técnica AO. Em cinco casos, a fusao incluiu o rádio, ossos do carpo e III metacarpiano; e, em dois, houve a centralizaçao da ulna em substituiçao ao rádio. O seguimento pós-operatório variou de 6 a 20 meses; a consolidaçao foi obtida, em todos os casos, aos 3 meses de seguimento pós-operatório. A única complicaçao observada foi a formaçao de hematoma na ferida operatória (três casos), que foi facilmente sanada. Todos os pacientes ficaram satisfeitos com os resultados obtidos.