Helaine Cristina de Castro

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.

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.

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.

Osteopetrose maligna: transplante de medula óssea

A osteopetrose e uma osteopatia hereditaria caracterizada pela deficiencia na reabsorcao ossea que ocorre por disfuncao dos osteoclastos. Com o acumulo de material osteoide que oblitera o canal medular, ocorre hematopoiese extramedular (hepato-esplenomegalia), obliteracao dos forames dos nervos cranianos (cegueira, surdez, paralisias faciais), macrocefalia, protusao da fronte, hipertelorismo, exoftalmo, aumento da pressao intracraniana, retardo na erupcao dentaria, atraso no crescimento, atraso no desenvolvimento neuropsicomotor, e a morte ocorre precocemente nos primeiros anos de vida. A unica alternativa terapeutica curativa e o transplante de medula ossea (TMO) de doador HLA identico, pois restabelece a hematopoiese e a funcao monocito-macrofago, com melhora das lesoes osseas e anormalidades hematopoieticas, embora nao reverta as alteracoes sensoriais ja instaladas. Os autores relatam casos de duas criancas portadoras de osteopetrose maligna submetidas ao transplante de medula ossea com sucesso. A primeira encontra-se no dia +1260 do TMO, com melhora evidente da radiologia esqueletica, sem progressao das deficiencias neurologicas que apresentava, e com biopsia ossea sem sinais de osteopetrose. O segundo paciente encontra-se no dia + 700, com sinais de reabsorcao ossea e sem progressao dos danos neurologicos. Os autores chamam a atencao para a necessidade de diagnostico precoce da osteopetrose e o rapido encaminhamento para o transplante de medula ossea antes da instalacao de sequelas neurologicas definitivas.

Linfoma de Hodgkin em baixa faixa etária: relato de dois casos

The cases of two male under five-year-olds diagnosed with Hodgkins lymphoma presenting as lymphadenopathy in evolution for some months and treated in our hospital are reported. Immunohistopathological findings of the tumors proved the existence of Hodgkins lymphoma nodular sclerosis and positive Epstein-Barr virus in tumoral material in both cases. After 13 months of complete remission one of the patients relapsed and presented aggressive disease. Laboratorial analyses of the tumor showed the same characteristics as the initial disease including positive Epstein-Barr virus. Early and intense exposure to Epstein-Barr virus may increase the risk related to the very early development of the disease and the unexpected evolution may be connected to the association of the histological subtype and the positive Epstein-Barr virus.

Púrpura trombocitopênica idiopática e linfoma não-Hodgkin de células T na infância

Abstract Lymphomas represent 10% of all malignant tumors in childhoodand from these non-Hodgkins lymphomas are the most frequent.Children who have autoimmune diseases have a higher probabilityof developing lymphoproliferative diseases, which can happenbefore, during or after the appearance of the neoplasia. Theassociation between idiopathic thrombocytopenic purpura andlymphomas is not common (3%) especially in children. Twotheories try to explain the origin of this association. In the firstone, the thrombocytopenia would be a result of an autoantibodyanti-blood platelet production by the tumoral clone. In the secondone, the idiopathic thrombocytopenic purpura would be a result ofa persistent antigenic stimulus subordinate to a disorder in thelymphoid proliferation. The aim of this work is to report the unusualassociation between idiopathic thrombocytopenic purpura and T-cell non-Hodgkins lymphoma in childhood. Rev. bras. hematol.hemoter. 2006; 28 (1):73-75. Key words : Idiopathic throm bocytopenic purpura; non-Hodgkinslymphoma; autoimmune diseases; thrombocytopenia.

Development of a Web-based bone marrow transplant registry system

A secure Web-based bone marrow transplant (BMT) registry tool has been developed inside an existing portal for paediatric oncology. The registry ensures identity protection and facilitates data organization and analysis. The registration interface is based on a Portuguese translation of the International Bone Marrow Transplantation Registry (IBMTR) forms. The objectives are to improve the information flow in collaborative BMT research protocols and to facilitate registration of BMT patients, thus improving data analysis and statistics. The system imports pre-existing data from the patient registry on the portal and has an interface that guides the user about completing the data fields according to the diagnosis. It also performs real-time data validation. After completion, the information is kept on the database for future use and the system generates an IBMTR printout form, translated into English, to be sent to the IBMTR centre.