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Background: Overt extraocular retinoblastoma is common in developing countries and little information about its treatment is available. The aim of this study is to report our experience in the treatment of these cases using a uniform approach., Procedure: Patients with overt extraocular retinoblastoma including orbital extension, preauricular lymph node invasion and/or metastatic disease on diagnosis or after extraocular relapse admitted to the Hospital JP Garrahan from August 1987 to December 2000 were retrospectively reviewed. Patients were treated according to two different protocols (1987-1993 and 1994-2000). Treatment included: neoadjuvant combination chemotherapy followed by limited surgery in case of orbital extension (enucleation or resection of residual orbital mass) and adjuvant chemotherapy and radiotherapy. Chemotherapy included cyclophosphamide, vincristine, etoposide, doxorubicin (in protocol 87), idarubicin (in protocol 94), cisplatin (in protocol 87), and carboplatin (in protocol 94)., Results: Forty-one patients were included. Fifteen of them had orbital or preauricular disease and had a 5-year event-free survival (pEFS) of 84%. Twenty-six had distant metastatic disease and non survived 5-years. One patient died of toxicity and one died in complete remission. One patient had a secondary leukemia. The remaining adverse events included CNS and/or systemic relapse., Conclusions: This treatment strategy was highly efficacious for patients with orbital and/or lymph node extension. Orbital exenteration is not necessary for these patients. Those patients with distant metastatic or CNS disease were not curable with this approach., (Copyright 2003 Wiley-Liss, Inc.)

Background: Acute myeloid leukemia (AML) is well-recognized as one of the most important second malignancies. We report the occurrence of secondary AML (sAML) in our institution., Procedure: From September 1987 to August 1996 we have observed sAML in 9 patients (median age 4 years), 5 of them previously treated for hematologic malignancies (group I): acute lymphoblastic leukemia (n = 2), AML (n = 1), non-Hodgkin lymphoma (n = 1). Hodgkin disease (n = 1), and 4 of these 9 patients treated for solid tumors (group II): neuroblastoma (n = 1), retinoblastoma (n = 1), Wilms tumor (n = 1), and central nervous system germinoma (n = 1)., Results: All the patients had topoisomerase II inhibitors as part of treatment of their first malignancy, but only 5 patients received epipodophyllotoxins. Alkylating agents were part of primary therapy in 8 of 9 patients. The latency period for the development sAML was 26.5 (range = 2-55) months. The morphologic FAB features of sAML were M5 (n = 5), M4 (n = 3), and M2 (n = 1). Cytogenetic studies showed r11q23 in 3 patients, all of them with prior hematological malignancies. Initial therapy for sAML in all cases was chemotherapy (including cytarabine in combination with idarubicin and etoposide or doxorubicin or mitoxantrone). Three patients died during induction and 6 achieved complete hematologic response. Three of these patients remain disease free at +15, +51, and +99 months post-remission (including one post allogeneic BMT). The remaining 3 patients died, 1 in complete remission one month after diagnosis and 2 relapsed and died with progressive disease (one post allogeneic BMT)., Conclusions: Secondary AML is a sequela of oncologic treatments with specific cytogenetic abnormalities and poor outcome. A few patients can achieve long-term survival even with standard chemotherapy.

Purpose: To compare prospective treatment strategies in childhood Hodgkin disease to the following subsets of patients: a) Favorable prognostic group: these patients were randomized to receive 6 vs. 3 CVPP chemotherapy cycles without radiotherapy (CVPP: cyclophosphamide, vinblastine, procarbazine, and prednisone. The scheme was repeated every 28 days). b) Intermediate prognostic group: these patients were randomized to receive 6 cycles of CVPP or AOPE (AOPE: Adriamycin, vincristine, prednisone, and etoposide). Between the third and fourth cycles, all patients in this group received radiotherapy (RT)(30-40 Gy to initially involved areas). c) Unfavorable prognostic group: those patients received a single arm regimen of 6 cycles of CCOPP/CAPTe (3 of each combination) every 28 days. All these patients received radiotherapy (30-40 Gy to initially involved areas)., Results: From October 1987 to December 1994, a total of 114 children and adolescents were evaluated. Mean age was 9 (range 2-17) years. There were 72 boys and 42 girls. With a median follow-up of 5 (range 1.5-8.7) years, at 80 months event-free survival (EFS) and overall survival (OS) for the whole cohort are 0.809 (SE: 0.04) and 0.873 (SE: 0.04), respectively (SE: Standard Error). Favorable prognostic group (n = 26) EFS is 0.831 (0.09) (Arm CVPP x 3:0.857 (0.13) and Arm CVPP x 6: 0.875 (0.08); p = non significant). Intermediate prognostic group (n = 64) EFS is 0.806 (0.05) (Arm CVPP x 6 + RT: 0.872 (0.05) and Arm AOPE x 6 + RT: 0.667 (0.10); p = 0.04). Unfavorable group (n = 24) EFS is 0.829 (0.07)., Conclusions: Results of treatment for the whole group are satisfactory. However, 3 cycles of CVPP without radiotherapy obtain equal EFS than 6 cycles without radiotherapy in the favorable prognostic group. In the intermediate prognostic group, 6 cycles of CVPP plus radiotherapy obtain a superior EFS than 6 cycles of AOPE plus radiotherapy. With the success of treatment for Hodgkin disease in children, future research needs to be focused in reducing toxicity without altering the excellent actual outcome.

Purpose: To report the feasibility and results of a study based on the BFM-ALL. NHL/86 protocol for B-non-Hodgin's Lymphoma (NHL) and B-Acute Lymphoblastic Leukemia (B-ALL) in Argentina. Design. Prospective, single arm, non-randomized trial., Patients and Methods: From August 1988 to December 1993, 87 consecutive patients with B-NHL/B-ALL were admitted and 82 were eligible. The therapy was stratified according to stage. All patients received a cytoreductive prephase with cyclophosphamide and prednisone. Those with stage I-II were treated with three 5-day blocks of combined intense chemotherapy including dexamethasone, cyclophosphamidie, ifosfamide, cytarabine, teniposide, doxorabicin, and 500 mg/m2 of methotrexate as a 24 hour continuous infusion. Stage III received 6 blocks and those with stage IV/B-ALL received 6 intensified blocks in which 2 g/m2 of 24 hour continuous infusion methotrexate and vincristine were added. Triple intrathecal therapy was given for CNS prevention. After the first two blocks the response was assessed and those with a partial response were offered optionallya second look surgery or local radiotherapy., Results: With a median follow-up of 38 (range 16-71) months, the event-free survival (pEFS) for the whole group was 0.69 (Stage I-II n = 16 pEFS = 0.94, stage III n = 50 pEFS = 0.66, Stage IV n = 7 pEFS = 0.43, B-ALL n = 9 pEFS = 0.66). Patients with stage III abdominal tumors who achieved a partial response by imaging studies after induction had a significantly higher risk of relapse than those with a complete response (p = 0.02). Relapse was the most frequent event Toxicity was mainly hematological., Conclusions: The application of this protocol was feasible in our setting and its results comparable to the German study. Patients with stage I-II had an excellent outcome. Those with stage III and B-ALL achieved an encouraging event-free survival, however those with abdominal tumors and partial response to induction chemotherapy fared less favourably. This strategy was less effective for patients with initial CNS disease.