Maria Luisa Borsato

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.

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.

Aplastic crisis in sickle cell anemia induced by parvovírus B19

PURPOSE: Transient aplastic crisis is reported in an eight-month old child with sickle cell anemia and acute B19 parvovirus infection. This fact is uncommon in this age. PATIENT AND METHODS: The authors review the literature and describe a clinical case of an eight-month old child with sickle cell anemia presented with profound anemia and reticulocytopenia. His peripheral blood was analyzed for parvovirus B19 using the polymerase chain reaction (PCR), and for anti B19 immunoglobulin Ig M, and Ig G by enzyme-linked immunosorbent assay (ELISA). RESULTS: An eight-month old child with sickle cell anemia was admitted to the hospital with fever and profound anemia (HB = 3.8g/ dl) and reticulocytopenia (2%). A diagnosis of aplastic crisis was established. The results indicate that Ig M and PCR were positive and Ig G negative. The patient needed erytrocyte transfusion, and was discharged on hospital day 4. CONCLUSIONS: The clinical and laboratory features indicate that human parvovirus B19 was the etiologic agent of an aplastic crisis in an eight-month old child. According to the international literature this event is uncommon for this age; in addition, this is the first time it appears in the Brazilian literature.

Results of a randomized, prospective clinical trial evaluating metronomic chemotherapy in nonmetastatic patients with high-grade, operable osteosarcomas of the extremities: A report from the Latin American Group of Osteosarcoma Treatment

Metronomic chemotherapy (MC) consists of the administration of a low dose of chemotherapy on a daily or weekly basis without a long break to achieve an antitumoral effect through an antiangiogenic effect or stimulation of the immune system. The potential effect of MC with continuous oral cyclophosphamide and methotrexate in patients with high‐grade operable osteosarcomas (OSTs) of the extremities was investigated.

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.