Paula Bruniera

Results of the brazilian osteosarcoma treatment group studies III and IV : Prognostic factors and impact on survival

PURPOSE To evaluate the impact of chemotherapy and surgery on the outcome of osteosarcoma (OS) of the extremities and to identify prognostic factors in Brazilian patients. PATIENTS AND METHODS A total of 225 patients with metastatic and nonmetastatic OS of the extremities were enrolled and assessed in two consecutive studies designed and implemented by the Brazilian Osteosarcoma Treatment Group. RESULTS The 5-year survival and event-free survival rates for the 209 assessable patients were 50.1% and 39%, respectively; for the 178 patients with nonmetastatic disease at diagnosis, the rates were 60.5% and 45.5%, respectively. The multivariate analysis showed that the following variables were associated with a shorter survival: metastases at diagnosis (P < .001), necrosis grades 1 and 2 (P = .046), and tumor size (P = .0071). CONCLUSION The overall 5- and 10-year survival rates were lower than the rates reported in North American and European trials. A pattern of advanced disease at diagnosis was often present, with a high proportion of patients having metastases (20.8%) and large tumor size (42.9%). However, these features were not necessarily associated with longer duration of prediagnostic symptoms. These findings were considered in the strategic planning of the current Brazilian cooperative study, with the aim of improving survival and quality of life of a large number of patients with OS.

Exposure to magnetic fields and childhood acute lymphocytic leukemia in Sao Paulo, Brazil

BACKGROUND Epidemiological studies have identified increased risks of leukemia in children living near power lines and exposed to relatively high levels of magnetic fields. Results have been remarkably consistent, but there is still no explanation for this increase. In this study we evaluated the effect of 60 Hz magnetic fields on acute lymphocytic leukemia (ALL) in the State of São Paulo, Brazil. METHODS This case-control study included ALL cases (n=162) recruited from eight hospitals between January 2003 and February 2009. Controls (n=565) matched on gender, age, and city of birth were selected from the São Paulo Birth Registry. Exposure to extremely low frequency magnetic fields (ELF MF) was based on measurements inside home and distance to power lines. RESULTS For 24h measurements in children rooms, levels of ELF MF equal to or greater than 0.3microtesla (μT), compared to children exposed to levels below 0.1 μT showed no increased risk of ALL (odds ratio [OR] 1.09; 95% confidence interval [95% CI] 0.33-3.61). When only nighttime measurements were considered, a risk (OR 1.52; 95% CI 0.46-5.01) was observed. Children living within 200 m of power lines presented an increased risk of ALL (OR 1.67; 95% CI 0.49-5.75), compared to children living at 600 m or more of power lines. For those living within 50 m of power lines the OR was 3.57 (95% CI 0.41-31.44). CONCLUSIONS Even though our results are consistent with the small risks reported in other studies on ELF MF and leukemia in children, overall our results do not provide support for an association between magnetic fields and childhood leukemia, but small numbers and likely biases weaken the strength of this conclusion.

Cranial vault lymphoma: a systematic review of five patients

Bone involvement is a common finding in many types of lymphoma (Clin Oncol 9(3): 195–196, 1997). However, cranial vault affliction has been regarded as an exceedingly rare presentation, particularly in the case of primary lymphoma (J Neurosurg 108(5): 1018–1020, 2008). Our objective is to describe a series of five immunocompetent patients with histologically confirmed cranial vault lymphoma (CVL), and to conduct a systematic review of the current literature. Our review points out identical imaging patterns in most of the lesions for all reported CVL cases, despite their different histological subtypes. This typical pattern can be seen on computed tomography (CT) scans and magnetic resonance imaging (MRI) as an expansive tumor that affects all three compartments of the cranial vault, including the scalp, skull bone, and pachymeninges, even in the absence of osteolysis. We argue that the absence of osteolysis might enhance diagnostic capability. In the appropriate clinical setting, these features represent important disease characteristics that may help with an earlier diagnosis. Large B-cell lymphoma was the most common subtype of primary CVL.

Two-Year Analysis of Efficacy and Safety of Deferasirox Treatment for Transfusional Iron Overload in Sickle Cell Anemia Patients

The efficacy and safety of a 2-year treatment with deferasirox was evaluated in 31 patients with sickle cell anemia and transfusional iron overload. At 24 months, there were significant decreases from baseline in mean serum ferritin (from 2,344.6 to 1,986.3 µg/l; p = 0.040) and in mean liver iron concentration (from 13.0 ± 5.4 to 9.3 ± 5.7 mg Fe/g dry weight; p < 0.001). Myocardial T2* values were normal (>20 ms) in all patients at baseline and did not change significantly over the course of the study. However, there was a significant improvement from baseline in left ventricular ejection fraction at 24 months (62.2–64.6%; p = 0.02). Deferasirox was generally well tolerated with no progressive increases in serum creatinine or renal failure observed. These data confirm that deferasirox is effective in reducing body iron burden in patients with sickle cell anemia and transfusional iron overload.

Deficiência de ferro no feto e no recém-nascido

A principal causa de anemia no feto e a doenca hemolitica do recem-nascido (RN). As gestantes anemicas na sua forma moderada nao acarretam baixos estoques de ferro no concepto, porem podem evoluir para o trabalho de parto prematuro e RN com baixo peso ao nascer. O ferro e transportado para o feto por via transplacentaria, principalmente durante o terceiro trimestre de gestacao. A deficiencia de ferro nao ocorre no periodo neonatal, porem os prematuros e ou RN com baixo peso constituem o principal grupo de risco para desenvolver a deficiencia de ferro. Nos RN nascidos a termo podemos observar uma deficiencia de ferro naqueles que sofreram resseccao cirurgica do duodeno devido a malformacao congenita. A fim de evitarmos a deficiencia de ferro neste grupo de risco, indica-se a suplementacao de ferro a partir dos 30 dias de vida. A via de administracao preferencial e a enteral, apesar de sabermos que no prematuro ocorre uma deficiencia do controle da absorcao do ferro. O complexo de ferro polimaltosado e o ferro aminoquelado sao os de escolha para a profilaxia da deficiencia de ferro em prematuros. A via endovenosa e segura e nao acarreta piora das lesoes causadas pela acao oxidativa do ferro em prematuros.

Osteossarcoma: experiência do Serviço de Oncologia Pediátrica da Santa Casa de Misericórdia de São Paulo

OBJETIVOS: Descrever as caracteristicas clinico-laboratoriais das criancas e adolescentes com osteossarcoma acompanhadas no Servico de Oncologia Pediatrica da Santa Casa de Misericordia de Sao Paulo e determinar as taxas de sobrevida livre de evento e sobrevida global, correlacionando-as com os principais fatores prognosticos. METODOS: Estudo retrospectivo realizado no Servico de Oncologia Pediatrica da Santa Casa de Misericordia de Sao Paulo compreendendo o periodo entre abril de 1991 e setembro de 2002. Nesse periodo foram diagnosticados 60 casos de osteossarcoma. Observaram-se predominio do sexo masculino (61,7%), raca nao-branca (65%) e idade superior a 10 anos (70%); historia de trauma previo em 53,3%, relato de dor em 95% e aumento de volume em 80% dos casos. O joelho foi a regiao mais comprometida (74,9%). Em 70,7% dos casos o tumor apresentava tamanho inferior a 1/3 do comprimento do osso afetado, em 25% o nivel serico de desidrogenase lactica era igual ou maior que 500U/L e em 48,3% o nivel de fosfatase alcalina era igual ou maior que 1.000U/L, 25% dos pacientes apresentavam metastase pulmonar visivel ao diagnostico e 53,3% pertenciam ao subtipo osteoblastico. O tratamento quimioterapico pre e pos-operatorio foi realizado em 78,3% dos pacientes, 64% foram submetidos a procedimento cirurgico conservador, 68,1% apresentaram necrose tumoral graus I e II e houve recidiva em 60% dos casos. RESULTADOS: Com seguimento de 48 meses observaram-se sobrevida livre de doenca em 28,6% dos pacientes e sobrevida global de 38,4%. CONCLUSOES: Os autores concluem que o diagnostico precoce e fundamental, assim como novos estudos sao necessarios para aprimorar o manuseio e o tratamento desses pacientes e melhorar a sobrevida.

Avaliação de dois anos de tratamento da doença de Gaucher tipo 1 com terapia de reposição enzimática em pacientes do estado de São Paulo, Brasil

A doenca de Gaucher tipo 1 e a doenca de deposito lisossomico mais frequente. De heranca autossomica recessiva, e caracterizada pela deficiencia da atividade da enzima glicocerebrosidase e o acumulo patologico de seu substrato, a glicosilceramida, nas celulas da linhagem dos monocitos/macrofagos principalmente no baco, figado e medula ossea. As manifestacoes sao heterogeneas e incluem hepatoesplenomegalia, anemia, trombocitopenia, infiltracao da medula ossea e lesoes esqueleticas. Avaliaram-se os efeitos da terapia de reposicao enzimatica (TRE) sobre a anemia, trombocitopenia, hepatoesplenomegalia, estatura (criancas e adolescentes) e dor ossea em noventa pacientes com doenca de Gaucher tipo 1 tratados por periodo de 24 meses no estado de Sao Paulo. Os principais sinais e sintomas antes do inicio do tratamento foram anemia (50%), trombocitopenia (59%), hepatomegalia (97%), esplenomegalia (96%), baixa estatura (46%) e dor ossea (62%). A dose media de TRE foi 35U/kg a cada duas semanas. A recuperacao da anemia, da trombocitopenia e da dor ossea foi mais intensa aos seis meses de tratamento, e da hepatoesplenomegalia e da baixa estatura aos 18 meses. Apos a melhora, a maioria dos pacientes manteve-se estavel. Ao final de 24 meses de TRE, pelo menos 88% dos pacientes atingiram os objetivos terapeuticos para anemia, 80% para trombocitopenia, 34% para hepatoesplenomegalia, 77% para baixa estatura e 76% para dor ossea. Novos estudos sao necessarios para avaliar a resposta terapeutica em longo prazo, principalmente em relacao as alteracoes osseas e de estatura, que podem apresentar resposta mais tardia e lenta.

Detection of Epstein–Barr virus in children and adolescents with Burkitt's lymphoma by in situ hybridization using tissue microarrays

Abstract In this retrospective study, we evaluated 53 patients diagnosed with Burkitts lymphoma (BL). Patients were admitted to the Pediatric Department of Santa Casa de São Paulo, Brazil from August 1988 to September 2006. The male gender was found more frequently in the proportion of three males to one female. At the time of the diagnosis, the mean age was 6 years. The most frequent clinical presentation was abdominal (extra nodal) in 42 (79·2%) cases. The patients were treated with chemotherapy using two different treatment methods, 30 cases until September 1994 with BGLTI -87 and the other 23 until September of 2006 with BGLTI -96. According to Murphys classification, 90·2% of the patients were diagnosed as stages III and VI showing an advanced disease at time of diagnosis. Using Tissue Microarrays (TMA); we searched for the expression of the Epstein–Barr virus (EBV) RNA in 50 patients. The presence of EBV using in situ hybridization was found in 33/50 (66%) and there was no association between the presence of the virus in the tumor cells or patient age, as well as the survival rate. There was 74·5% global survival rate until September, 2005 of 12 years and 8 months. The disease free survival rate was 86·5%. The frequency of the EBV association with this neoplasia in Brazil is intermediate between the endemic and sporadic forms of disease.

Crise de seqüestro esplênico na doença falciforme

Splenic Sequestration is an acute complication responsible for high morbidity and mortality in patients with sickle cell disease. It is characterized by a reduction in the concentration of hemoglobin of 2g/dl or more compared to the basal value of the patient, and increases in erythropoietin and the size of the spleen. Clinical manifestations include hypovolemic shock which may be associated with viral or bacterial infections. Its incidence is variable depending on the region studied and it is more common in children between 3 months and 5 years old. A high percentage of fetal hemoglobin at 6 months of life seems to be a protective factor, while treatment with hydroxiurea to maintain splenic function seems to contribute with the increase in the rate of sequestration crises. Recurrence is common, occurring in about 50% of the survivors of first episodes. Treatment of sequestration crises should be immediate with volumetric expansion, transfusion which will immobilize the sequestered red blood cells.Duo to its common recurrence, these patients should receive preventive therapy. The best conduct is still the subject of many studies. It is recommended that under 2- years-old patients who present with severe crises should be maintained in chronic transfusion program up to 2 years old when they will be splenectomized. Over 2 years old infants should be referred for surgery. Educational measures of parents to quickly recognize the symptoms have contributed to a good evolution of .

Osteossarcoma e leucemia mielóide aguda: dois casos em crianças

A secondary neoplasia after chemotherapy often occurs in patients with Hodgkins disease or brain tumors.1-4 There are a few cases of acute myeloid leukemia (AML) after osteosarcoma treatment reported in the literature. The authors report on two children who developed AML after being treated by chemotherapy for osteosarcoma. The first patient had a telangiectatic osteosarcoma; he was treated with carboplatin, ifosfamide, epirubicin and methotrexate and developed AML thirty eight months after therapy. The second patient had chondroblastic osteosarcoma; she was treated with cisplatin, doxorubicin and ifosfamide and she developed AML six months after treatment. The authors discuss the role of alkylating agents, topoisomerase II inhibitors and platinum compounds in the development of AML.