ABSTRACT Background: Peripheral blood stem cell concentrations are traditionally adjusted to 20–40 × 106 leukocytes/mL prior to freezing. This low cell concentration at cryopreservation implies larger volumes with more dimethyl sulfoxide being used, and higher cost and toxicity at the time of transplant. Higher cell concentrations have been reported but this is not widely accepted. Moreover, the influence of cell concentration on engraftment has not been well documented. Therefore, this study retrospectively analyzed the influence of peripheral blood stem cell concentration at freezing on engraftment after autologous hematopoietic stem cell transplantation. Method: Leukapheresis products were plasma-depleted and cryopreserved with 5% dimethyl sulfoxide, 6% hydroxyethylamide solution and 4% albumin in a −80 °C freezer. Individual patient data from hospital records were reviewed. Results: Fifty consecutive patients with oncological diseases underwent 88 leukaphereses. Median age was six years (range: 1–32 years) and median weight was 19 kg (range: 8–94 kg). Median leukocyte concentration was 109 × 106/mL at collection and 359 × 106 (range: 58–676 × 106) at freezing with 78% viability (range: 53–95%); leukocyte recovery after thawing was 95% (range: 70–100%). In multivariate analysis, cell concentration (p-value = 0.001) had a negative impact on engraftment. Patients infused with bags frozen with <200 × 106 leukocytes/mL engrafted after a median of nine days (range: 8–12 days), 200–400 × 106 leukocytes/mL after 11 days (range: 9–20 days); 400–600 × 106 leukocytes/mL after 12 days (range: 8–19 days) and with cell concentrations >600 × 106 leukocytes/mL, engraftment was after 14 days (range: 13–22 days). Conclusion: In patients with adequate CD34 cell collections, total leukocyte concentrations of 282 × 106/mL, freezing with 5% dimethyl sulfoxide and 6% hydroxyethylamide solution without a controlled-rate freezer, and storing cells at −80 ºC yielded excellent engraftment. Further increases in cell concentration may delay engraftment, without affecting safety.

Objective: To investigate the correlation between the sagittal and frontal alignment and possible postural asymmetries found in patients submitted to total knee stent placement for osteosarcoma.Methods: Twenty two individuals were divided into two groups according to tumor location: femur group (13 patients) and tibia group (nine patients), who were evaluated through postural analysis software (SAPO).Results: No statistically significant difference was found between groups, supporting previous result showing that both groups present the same postural asymmetries.Conclusion: We conclude that both groups have the same postural imbalances, especially the knee of the affected limb that presents hyperextension and center of gravity shifted anteriorly and laterally to the non-affected limb, indicating changes in weight bearing and influencing the gait pattern and balance. Level of Evidence II, Prospective Comparative Study.

OBJETIVO: Traçar o perfil epidemiológico e o prognóstico do sarcoma de Ewing na população brasileira. Material e MÉTODO: Foram avaliados, retrospectivamente, os prontuários de 64 pacientes tratados, com sarcoma de Ewing intraósseo, no Instituto de Oncologia Pediátrica, IOP-GRAACC-Unifesp, no período de 1995 a 2010. RESULTADOS: A análise estatística dos dados obtidos não correlacionou fatores como sexo, trauma, fratura patológica e tempo ao diagnóstico com o desfecho do tratamento. Fatores como metástase inicial, metástase pulmonar, local do tumor, idade, recidiva e tipo de cirurgia indicam resultados que corroboram a literatura consagrada. CONCLUSÃO: O prognóstico no sarcoma de Ewing foi influenciado principalmente pela presença de metástase ao diagnóstico.

OBJECTIVE: To outline the epidemiological profile and prognosis for Ewing's sarcoma in the Brazilian population. Material and METHODS: The medical records of 64 patients with intraosseous Ewing's sarcoma who were treated at the Pediatric Oncology Institute, IOP-GRAACC-Unifesp, between 1995 and 2010, were retrospectively evaluated. RESULTS: The statistical analysis on the data obtained did not correlate factors such as sex, trauma, pathological fracture and time taken for case diagnosis with the treatment outcome. Factors such as initial metastasis, lung metastasis, tumor site, age, recurrence and type of surgery showed results corroborating what has been established in the literature. CONCLUSION: The prognosis in cases of Ewing's sarcoma was mainly influenced by the presence of metastases at the time of diagnosis.

Objetivo Descrever o algoritmo e os resultados globais após sua implantação. Métodos Ensaio clínico randomizado realizado com pacientes com câncer e desnutrição. O acompanhamento foi guiado por algoritmo, e desnutridos leves eram randomizados para suplementação oral industrializada ou artesanal, reavaliados após três, oito e doze semanas. Dependendo da resposta no grupo suplementação oral indus-trializada, indicava-se suplemento industrializado por sonda, mantinha-se suplementação oral industrializada ou ela era suspensa. O grupo suplementação oral artesanal com resposta positiva permaneceu com a mesma orientação; se negativa iniciou com suplementação oral industrializada. Nos desnutridos graves, resposta negativa indicou suplemento industrializado por sonda, positiva manteve suplementação oral industrializada ou ela era suspensa no caso de eutrofia. A avaliação das respostas foi realizada por meio do escore-Z de peso para estatura, índice de massa corpórea, prega cutânea triciptal e circunferências. Resultados Cento e dezessete de 141 pacientes completaram três semanas iniciais; 58 desnutridos grave e 59 leve. Evolução nutricional positiva ocorreu em 41% dos graves, em 97% dos leves com suplementação oral industrializada e em 77% dos desnutridos leves com suplementação oral artesanal. Dos 117, 42 tiveram indicação de suplemento industrializado por sonda; 23 usaram e 19 recusaram, mantendo suplementação oral industrializada. Pacientes que usaram suplemento industrializado por sonda apresentaram consumo de suplemento superior aos demais; o consumo de suplemento industrializado por sonda também foi superior quando comparado ao período anterior com suplementação oral industrializada nos mesmos pacientes. O uso de suplemento industrializado por sonda demonstrou melhorar o estado nutricional, com associação positiva entre sua duração e evolução nutricional. Conclusão Suplementação oral industrializada reduziu o deficit nutricional, principalmente em desnutridos leves. Os resultados sugerem que a suplemento industrializado por sonda favoreceu a recuperação nutricional, principalmente com o uso mais prolongado.

Objective This study aimed to describe the algorithm and the global results after its implementation. Methods This was a randomized clinical trial done with malnourished cancer patients. Follow-up followed an algorithm and patients with mild malnutrition were randomized to receive store-bought or homemade oral supplementation. The patients were reassessed after three, eight and twelve weeks. Depending on how the group supplemented with store-bought supplements responded, the supplementation was either continued orally, by tube-feeding or discontinued. The group receiving homemade supplementation either continued on it if the response was positive or received store-bought oral supplementation if the response was negative. The severely malnourished patients either received store-bought supplementation by feeding tube or orally, or it was discontinued if an adequate nutritional status was reached. The patients' responses to supplementation were determined by weight-for-height Z-scores, body mass indices, triceps skinfold thicknesses and circumferences. Results One hundred and seventeen out of 141 patients completed the first three weeks; 58 were severely malnourished and 59 were mildly malnourished. The nutritional status of 41% of the severely malnourished patients and 97% of the mildly malnourished patients receiving store-bought supplement orally improved. The nutritional status of 77% of the mildly malnourished patients receiving homemade supplement orally also improved. Of the 117 patients, 42 had to be tube-fed; of these, 23 accepted and 19 refused tube feeding and continued taking store-bought supplement orally. Consumption of store-bought supplement was higher in tube-fed patients than in orally-fed patients. Consumption also increased as orally-fed patients became tube-fed. Store-bought supplements given by tube improved nutritional status, showing a positive association between duration of tube feeding and nutritional status. Conclusion Store-bought oral supplements improved the nutrient intake of malnourished patients, especially those who were mildly malnourished. The results also suggest that store-bought supplements given by tube improve nutritional status, especially as duration of tube feeding increases.

OBJETIVO: Avaliar a eficiência da nutrição parenteral administrada a pacientes oncológicos pediátricos quanto à oferta de energia, indicações e principais dificuldades no procedimento. MÉTODOS: Estudo observacional descritivo, realizado entre julho de 2003 e julho de 2004 no Instituto de Oncologia Pediátrica da Universidade Federal de São Paulo. Critérios de inclusão: pacientes com câncer recebendo nutrição parenteral nas unidades de internação. A análise da adequação energética foi efetuada usando-se equações preditivas para o cálculo do gasto energético basal ajustado para a progressão inicial. RESULTADOS: Foram 41 episódios de nutrição parenteral de, 1.016, internações (4%): 54% do sexo masculino, com mediana de uso de 10 dias (1-51). Demonstrou-se que em 56% dos episódios não se atingiu o gasto energético basal ajustado; 26% ficaram entre 100% e 120% e 18% acima de 120%. A média do gasto energético basal ajustado foi 106%, com desvio-padrão de 38% e a mediana 96%. Os motivos associados às dificuldades para atingir o gasto energético basal foram intolerância metabólica, via de nutrição parenteral não exclusiva, suspensão ou interrupção da sua administração, prescrição sem discussão com nutricionista e uso de via periférica. A principal indicação foi por toxicidade gastrintestinal. O volume desprezado de nutrição parenteral demonstrou-se superior a 10% em 17 de 35 episódios (49%). Proteção contra luz ambiente (capa), avaliada em 19 pacientes durante os dias da administração, detectou ausência em 37 de 315 dias (12%). CONCLUSÃO: A oferta de energia não contemplou as necessidades de alta porcentagem de pacientes, principalmente devido à gravidade do quadro clínico, às interrupções e ao uso de via não exclusiva, reduzindo o período disponível para administração da nutrição. A eficiência dos procedimentos por atuação de equipe multidisciplinar poderia reduzir o prejuízo, garantindo uma oferta mais adequada, aumentando os benefícios da nutrição parenteral.

OBJECTIVE: The objective of this study was to evaluate parenteral nutrition efficiency in supplying energy for pediatric oncology patients, indications and main difficulties in the procedure. METHODS: This was an observational descriptive study done from July 2003 to July 2004 at the Pediatric Oncology Institute of the Universidade Federal de São Paulo. Inclusion criteria: hospitalized cancer patients receiving parenteral. Energy adequateness was analyzed by equations that calculated basal energy expenditure adjusted for initial progression. RESULTS: Forty-one (4%) of the 1016 hospitalized patients received parenteral nutrition: 54% were males and received parenteral nutrition for a median of 10 days (1-51). Fifty-six percent of the episodes did not reach the adjusted basal energy expenditure; 26% remained between 100 and 120% and 18% were above 120%. The mean adjusted basal energy expenditure was 106% with a standard deviation of 38% and a median of 96%. The reasons associated with difficulties to achieve the basal energy expenditure were metabolic intolerance, non-exclusive parenteral nutrition, suspension or interruption of its use, prescription without discussing with nutritionist and use of peripheral route. Parenteral nutrition was mainly indicated in gastrointestinal toxicity. The discarded volume of parenteral nutrition was above 10% in 17 of 35 episodes (49%). Protection from room light (cover) assessed in 19 patients during the days of administration detected an absence of 37 of 315 days (12%). CONCLUSION: Energy supply did not meet the demands of a high percentage of the patients, mainly because of the severity of the clinical scenario, interruptions and non-exclusive use, reducing the amount of time available to administer the nutrition. A multidisciplinary team which would improve the efficiency of the procedures could reduce the losses, guaranteeing a more adequate supply and increasing the benefits of parenteral nutrition.

O objetivo deste estudo foi avaliar a evolução do perfil metabólico-nutricional de crianças e adolescentes com câncer que realizaram transplante de medula óssea. Dezoito pacientes submetidos a transplante de medula óssea foram avaliados prospectivamente de outubro de 2003 a agosto de 2004. A avaliação foi realizada por meio da análise bioquímica de sangue para albumina, lipídeos e glicose em três momentos: antes da infusão da medula óssea, após sete dias e após catorze dias do transplante de medula óssea. O teste de Friedman foi aplicado para comparar as distribuições nos períodos e o teste de Mann'Whitney para comparar as diferenças na evolução entre os grupos de transplante de medula óssea autólogo versus alogênico. Quinze dos dezoito pacientes foram elegíveis: sete portadores de leucemia, quatro de linfomas e quatro de tumores de células germinativas. Os quinze pacientes apresentaram em média 10,7±7,1 anos. Nove realizaram transplante de medula óssea autólogo e seis alogênico aparentado; dois utilizaram ciclosporina A como imunossupressor e três receberam irradiação corporal total como parte da terapia de condicionamento. Treze de quinze usaram nutrição parenteral. A média de internação foi 33±14 dias. As prevalências nos déficits de albumina e HDL-C aumentaram progressivamente durante o acompanhamento: 15%, 31% e 46% e 54%, 69% e 85%, respectivamente. O mesmo ocorreu com os níveis de glicose e triglicérides, com aumento progressivo nas prevalências de anormalidades no decorrer do transplante de medula óssea, que foram de 7%, 43% e 50% e de 31%, 69% e 77%, respectivamente. Níveis de colesterol total e de LDL-C acima do normal foram observados somente em um paciente no primeiro momento (antes da infusão da medula óssea). A análise da evolução das variáveis no decorrer dos três períodos demonstrou que os níveis de HDL-C, glicemia e triglicérides se modificaram significantemente. Quando se compararam pacientes que realizaram transplante de medula óssea alogênico versus autólogo, não foi possível detectar diferença estatisticamente significante. Porém os níveis de HDL-C e de triglicérides apresentaram alterações mais acentuadas nos pacientes que realizaram transplante de medula óssea alogênico. Os resultados sugerem que alterações metabólicas e bioquímicas ocorrem durante o período do transplante. Tais alterações, possivelmente, são multifatoriais, podendo estar associadas com o déficit nutricional, catabolismo protéico e distúrbios no metabolismo energético. Provavelmente, a desnutrição, o uso de nutrição parenteral, bem como a resposta inflamatória e as toxicidades dos medicamentos estejam implicadas como fatores causais desses distúrbios.

The objective was to evaluate the nutritional metabolic profiles in children and adolescents with cancer undergoing bone marrow transplants. Eighteen bone marrow transplantation patients were prospectively evaluated from October 2003 to August 2004. The assessment was based on the biochemical blood analyses of albumin, lipids and glucose at 3 different moments: before the bone marrow transplant, 7 days after the bone marrow transplant and 14 days after the bone marrow transplant. The Friedman test was performed to compare the distributions between the periods and the Mann' Whitney test to compare differences in the evolution between the bone marrow transplant groups: autologous versus allogeneic. Fifteen of the 18 patients were eligible: seven had leukemia, four lymphoma and four germ cell tumors. The mean age of the 15 patients was 10.7±7.1 years. Nine received autologous and six allogeneic-related bone marrow transplants; two used cyclosporin A as an immunosuppressor and three total body irradiation as part of the conditioning regimen. Thirteen of the 15 received parenteral nutrition. The length of stay was 33±14 days. The prevalence of albumin and HDL-C deficits increased progressively during follow up: 15%, 31% and 46% and 54%, 69% and 85%, respectively. The same result was observed with the glucose and triglyceride levels, showing a progressive increase in the prevalence of abnormalities during bone marrow transplantation, with values of 7%, 43% and 50% and 31%, 69% and 77%, respectively. Total cholesterol and LDL-C levels above the normal were only observed in one patient at the first moment (before the bone marrow transplant). The analysis of the evolution of the variables during the three periods demonstrated that the HDL-C, glucose and triglyceride levels changed significantly. No statistically significant differences were observed when comparing patients submitted to allogeneic and autologous bone marrow transplants. However, changes in the HDL-C and triglyceride levels were more accentuated in patients submitted to allogeneic bone marrow transplants. The results suggested that metabolic and biochemical changes occur during the period of bone marrow transplantation. These changes are probably multi-factorial, being associated with nutritional deficits, protein catabolism and disturbances in the energy metabolism. It is probable that malnutrition, the use of parenteral nutrition, as well as the inflammatory response to and toxicity of the drugs applied are implicated as causal factors of these abnormalities.

CONTEXTO E OBJETIVO: Em pacientes com câncer, a desnutrição tem múltiplas causas. Medidas antropométricas de peso e estatura são o método mais utilizado para avaliação do estado nutricional. Infelizmente, esse método é limitado em pacientes com câncer, pois o peso inclui o tumor e a relação peso/estatura não leva em conta alterações específicas de tecido magro. O objetivo deste estudo é avaliar diferenças entre medidas antropométricas e de composição corporal em crianças e adolescentes com câncer. TIPO DE ESTUDO E LOCAL: Estudo de corte transversal realizado no Instituto de Oncologia Pediátrica, Universidade Federal de São Paulo - Escola Paulista de Medicina, São Paulo, Brasil. MÉTODOS: Crianças e adolescentes com câncer com idade acima de um ano foram avaliadas de março de 1998 a janeiro de 2000. Medidas antropométricas tradicionais foram coletadas no primeiro mês de tratamento oncológico (terapia de indução) por meio do escore-z de peso para estatura (P/E) nas crianças e índice de massa corpórea (IMC) nos adolescentes. A avaliação da composição corporal foi composta por medidas de prega cutânea triciptal (PCT), circunferência do braço (CB) e circunferência muscular do braço (CMB). Os dados foram analisados comparando-se as prevalências de desnutrição entre os métodos de avaliação nutricional. O teste do qui-quadrado e o grau de associação foram usados para comparar as taxas entre portadores de tumores sólidos e hematológicos. RESULTADOS: 139 pacientes foram avaliados, 127 tinham dados completos para análise. O estudo demonstrou maior percentual de déficit nos portadores de doenças sólidas não-hematológicas pelo P/E ou IMC, CB, e CMB. A análise global também sugere que a PCT (40%) e a CB (35%) demonstraram maior percentual de déficit quando comparadas ao escore-z de P/E ou IMC (19%). CONCLUSÃO: Pacientes com tumores sólidos apresentaram maior prevalência de desnutrição. As medidas de composição corporal por meio da PCT e CB detectaram mais pacientes desnutridos do que o P/E e o IMC.

CONTEXT AND OBJECTIVE: Malnutrition in cancer patients has many causes. Nutritional status is usually assessed from weight/height indices. These present limitations for the nutritional assessment of cancer patients: their weights include tumor mass, and lean mass changes are not reflected in weight/height indices. The objective was to evaluate differences between two anthropometric methods and compare deficits, in non-hematological tumor patients and hematological disease patients. DESIGN AND SETTING: Cross-sectional study at Instituto de Oncologia Pediátrica, Universidade Federal de São Paulo. METHODS: Children and adolescents were evaluated between March 1998 and January 2000. Traditional anthropometric measurements were obtained in the first month of treatment (induction therapy), by weight-for-height (W/H) using z-scores index for children and body mass index (BMI) for adolescents. Body composition evaluations consisted of specific anthropometric measurements: triceps skinfold thickness (TSFT), mid-upper arm circumference (MUAC) and arm muscle circumference (AMC). Data were analyzed to compare nutritional assessment methods for diagnosing malnutrition prevalence. The chi-squared test was used for comparative analyses between tumor patients and hematological disease patients. RESULTS: Analysis was done on 127 patients with complete data. Higher percentages of deficits were found among tumor patients, by W/H z-scores or BMI and by MUAC and AMC. Higher percentages of deficits were shown by TSFT (40.2%) and MUAC (35.4%) than by W/H z-scores or BMI (18.9%). CONCLUSION: Non-hematological tumor patients presented higher malnutrition prevalence than did hematological disease patients. Body composition measurements by TSFT and MUAC detected more patients with malnutrition than did W/H or BMI.

O fibroistiocitoma maligno variante angiomatóide constitui entidade rara e geradora de controvérsia, especialmente quanto a sua histogênese. Apresenta como peculiaridades o acometimento preferencial da faixa etária pediátrica e o comportamento biológico indolente, tendo baixas taxas de metastatização. No presente caso, paciente feminina de 6 anos apresentou, em decorrência de lesão em extremidade inferior, metástase linfonodal inguinal.

Angiomatoid malignant fibrous histiocytoma is a rare and controversial entity, particularly as to its histogenesis. The tumor affects mostly the pediatric group, with an indolent clinical course and low rate of metastatization. In this report, the patient presented a lesion in the right foot and metastasis in an inguinal lymph node.

BACKGROUND: The empirical use of antibiotic treatments is widely accepted as a means to treat cancer patients in chemotherapy who have fever and neutropenia. Intravenous monotherapy, with broad spectrum antibiotics, of patients with a high risk of complications is a possible alternative. METHODS: We conducted a prospective open-label, randomized study of patients with lymphoma or leukemia who had fever and neutropenia during chemotherapy. Patients received either monotherapy with ticarcillin/clavulanic acid (T) or ceftriaxone plus amikacin (C+A). RESULTS: Seventy patients who presented 136 episodes were evaluated, 68 in each arm of the study. The mean neutrophil counts at admission were 217cells/mm³ (T) and 201cells/mm³ (C+A). The mean duration of neutropenia was 8.7 days (T) and 7.6 days (C+A). Treatment was successful without the need for modifications in 71% of the episodes in the T group and 81% in the C+A group (p=0.23). Treatment was considered to have failed because of death in two episodes (3%) in the T group and three episodes (4%) in the C+A group, and because of a change in the drug applied in one episode in the T group and two episodes in the C+A group. Overall success was 96% (T) and 93% (C+A). Adverse events that occurred in group T were not related to the drugs used in this study. CONCLUSION: In pediatric and adolescent patients with leukemia or lymphoma, who presented with fever and neutropenia, during chemotherapy, ticarcillin/clavulanic acid was as successful as the combination of ceftriaxone plus amikacin. It should be considered an appropriate option for this group of patients at high risk for infections.