Registro oncopediátrico hospitalario argentino / The argentina ocopediatric hospital registry

El registro Oncopediátrico Hospitalario Argentino (ROHA). Fundación Laleidos registra pacientes menores de 15 años con nuevo diagnóstico de cáncer desde el año 2000. El primer objetivo fue desarrollar un registro de cáncer infantil siguiendo los lineamientos internacionales (OMS/IARC) conun modelo único. El segundo objetivo fue la coordinación y centralización de la información para análisis estadístico de los datos a nivel local, provincial, regional y nacional. Población y Metodo: Las fuentes de ROHA son las instituciones públicas y privadas que atienden niños con patología oncológíca, registros regionales de cáncer, grupos coopertivos de trabajo, datos de defunciones aportados por la Dirección de Estadísticas e Información de Salud del MInisterio de Salud de la Nación y profesionales que atienden niños con cancer en forma particular. Actualmente ROHA cuenta con 67 fuentes registrantes, 8 registros de Tumores Poblacionales y 2 grupos médicos cooperativos quienes son responsables de la notificación de los casos y del seguimiento activo. Resltados: La estimaición de la cobertura actual de ROHA de los casos registrados en relación con los casos esperados para todo el país es de 92. En el período 2000-2005 se registraron 7583 niños con patología oncológica, promedio anual 1264, el 56 es de sexo masculino, las leucemias representan el 36,7, le siguen en orden de frecuencia los tumores cerebrales con el 18,9. El 86 de los pacientes registrados, se atienden eninstituciones públicas y el 37 deben migrar para realizar parte de su tratamiento. Conclusiones: ROHA alcanzó una cobertura nacional estimada de aproximadamente 92. Existe una concordancia de los resultados generales de RPHA con las publicaciones internacionales. Se observó una desigualdad de oportunidades diagnósticas de sostén y terapéuticas, dependiendo de las instituciones en las diferentes áreas geográficas del país.

Retinoblastoma with low risk for extraocular relapse.

OBJECTIVE: To define a subgroup of patients with retino-blastoma and low risk of extraocular relapse through histopathological and clinical variables. PATIENTS AND METHODS: Inclusion criteria consisted of stage I (intraocular disease), stage IIb1 (without concomitant choroid and/or scleral invasion), and nonenucleated patients (according to the Grabowski-Abramson classification). A total of 112 consecutive patients admitted to Hospital JP Garrahan from 1987 to 1997 were evaluable. Treatment included enucleation or local therapy and no chemotherapy. RESULTS: Forty-one patients had stage Ia (intraretinal), 8 stage Ib (prelaminar optic nerve invasion), 40 stage Ic (uveal invasion), and 12 stage IIb1 (postlaminar optic nerve invasion and cut end free of tumor). Eleven patients had neither eye enucleated. Median follow-up was 60 months. Only two events occurred: one patient had progressive disease in the contralateral globe and died of CNS metastasis and another had an orbital relapse that was successfully treated. Both had choroidal invasion. Five-year pEFS and pOS were 0.97 and 0.98, respectively. Neither length of the optic nerve stump, tumor size, anterior chamber invasion, degree of differentiation, nor degree of ocular coat invasion correlated with increased risk of metastasis. CONCLUSIONS: A subset of patients with retinoblastoma with low risk of relapse can be determined using histopathological evaluation of the invasion of ocular coats. Adjuvant chemotherapy is not warranted for patients with intraretinal extension and prelaminar optic nerve invasion. It is also probable that those patients with isolated choroidal invasion and those with postlaminar optic nerve extension with surgical margins clear of tumor do not need chemotherapy.

Late diagnosis of retinoblastoma in a developing country.

OBJECTIVES: To assess the diagnostic process of retinoblastoma in a developing country. STUDY DESIGN: Prospective survey of 95 consecutive parents of patients with retinoblastoma. RESULTS: Fifty six parents consulted initially with a paediatrician. Their children tended to be younger, with a significantly higher frequency of advanced disease. Only half of the patients who consulted with a paediatrician were appropriately referred to an ophthalmologist; the paediatrician underestimated the complaints in the remainder. Children taken to an ophthalmologist were older and had less advanced disease. In about three quarters of these children, a diagnosis of retinoblastoma was suspected by the ophthalmologist on the first visit. Parents of patients with more advanced disease consulted significantly later. Poor parental education correlated significantly with late consultation. Lack of health insurance and living outside Buenos Aires City correlated significantly with an increased risk of extraocular disease. CONCLUSIONS: Paediatricians are the first health professional seen by most children with retinoblastoma. However, the diagnosis is not readily established. There is also a delay in consultation by parents, which is significantly longer in cases with advanced extraocular disease. Socioeconomic factors and access to health care might play a role in delayed diagnosis.

Papel del pediatra en el proceso diagnóstico del retinoblastoma / Role of pediatrician in the diagnostic process of retinoblastoma

Objetivos: describir el proceso diagnóstico en pacientes con retinoblastoma y el papel del pediatra en el mismo. Valorar los conocimientos acerca del retinoblastoma en una población de médicos residentes de pediatría. Material y métodos: estudio prospectivo transversal. Encuesta administrada a los médicos residentes e interrogatorio a familias de pacientes con retinoblastoma. Resultados: se estudiaron 69 familias. Consultaron al peidatra 39, siendo derivadas al oftalmólogo 17. Treinta pacientes consultaron al oftalmólogo. En 12 de ellos, no se práctico fondo de ojos se restó importancia al cuadro. Los pacientes que consultaron al pediatra eran significativamente más jóvenes y tenían mayor frecuencia de enfermedad bilateral. La mediana del tiempo transcurrido entre la aparición de los síntomas y la consulta fue de 9 semanas. De los cuales 41 médicos residentes encuestados, 30 reconocieron a la leucocoria como manifestación más frecuente del retinoblastoma y sólo 11 al estrabismo. Solo 9 residetes respondieron adecuadamente la edad de presentacion del retinoblastoma bilateral. No hubo asociación significativa entre el año de residencia y la probabilidad de responder acertadamente las pregutas. La gran mayoría había recibido información sobre el retinoblastoma en la facultad o en la residencia. conclusiones: La mayoría de los pacientes con retinoblastoma son llevados inicialmente al pediatra, en especial los niños más jovenes. En un alto porcentaje el pediatra no derivó al niño al oftalmólogo para su evaluación. Existió además una demora en llevar a la consulta a los niños por parte de la familia. Los médicos residentes de pediatría tienen escasa información acerca del retinoblastoma, independientemente del año de residencia o del hecho de haber visto pacientes con esta entidad.

Randomized trial of CVPP for three versus six cycles in favorable-prognosis and CVPP versus AOPE plus radiotherapy in intermediate-prognosis untreated Hodgkin's disease.

PURPOSE: To evaluate in a randomized trial the impact of three versus six cycles of cyclophosphamide, vinblastine, procarbazine, and prednisone (CVPP) chemotherapy in favorable-prognosis and CVPP versus doxorubicin, vincristine, prednisone, and etoposide (AOPE) plus involved-field radiotherapy (RT) in intermediate-prognosis previously untreated Hodgkin's disease. PATIENTS AND METHODS: Of 256 patients evaluated, 80 with a favorable prognosis according to a prognostic index designed by the Grupo Argentina de Tratamiento de Leucemia Aguda (GATLA) were randomized to three versus six cycles of CVPP without RT and 176 with intermediate risk to CVPP versus AOPE, both for six cycles with RT between the third and fourth cycles of 30 Gy to the involved areas at diagnosis. CVPP consisted of intravenous (I.V.) cyclophosphamide and vinblastine on days 1 and 8, and oral procarbazine and prednisone on days 1 to 14, every 28 days. AOPE consisted of I.V. doxorubicin and vincristine on day 1, oral prednisone on days 1 to 5, and I.V. etoposide on days 1 and 3, every 28 days. RESULTS: Complete remission was obtained in 39 of 41 (95%) patients treated with three cycles of CVPP and 36 of 39 (92%) treated with six cycles in the favorable-risk group (difference not significant [NS]). In the intermediate-risk group, 89 of 92 (97%) treated with CVPP plus RT versus 75 of 84 (89%) treated with AOPE plus RT achieved a complete remission (P = .05). At 60 months, the event-free survival (EFS) and overall survival rates in the favorable-risk group were 80% and 91% for CVPP x 3 and 84% and 97% for CVPP x 6, respectively (P = NS). In the intermediate-risk group, 60-month EFS rate for CVPP plus RT was 85%, compared with 66% for AOPE plus RT (P = .009). The overall survival rate was 95% versus 87% respectively (P = .157). CONCLUSION: Three cycles of CVPP without RT are equally effective as six cycles in the favorable-risk group. However, in the intermediate-group, CVPP plus RT is superior to AOPE plus RT, with significantly fewer events before and after induction (P = .009), without a difference in overall survival.

Results of a stage-based protocol for the treatment of retinoblastoma.

PURPOSE: To describe the treatment of retinoblastoma at a single institution using a prospective protocol based on histopathologic staging. PATIENTS AND METHODS: We included 116 consecutive patients (101 eligible, 46 bilateral) from August 1987 to December 1993. Treatment was enucleation or conservative therapy for intraocular disease (stage I patients). Stage II patients (orbital or postlaminar invasion) received vincristine, cyclophosphamide, and doxorubicin for 57 weeks. Patients with orbital mass and extension beyond the cut end of the optic nerve also received orbital radiotherapy (45 Gy). The latter received intrathecal therapy. In those with CNS (stage III) or hematogenous metastasis (stage IV), cisplatin and etoposide were added along with cranial (in patients with a CNS mass and prophylactically in stage IV) or craniospinal (in patients with positive CSF) radiotherapy. RESULTS: The median follow-up time was 39 months (range, 12 to 84). The overall survival rate was 0.84. Survival rates according to stage were as follows: stage I probability of overall survival [pOS] = 0.97) (alive/total), 59 of 60; stage II (pOS = 0.85) including patients with scattered episcleral cells, three of three; orbital mass, one of one; postlaminar invasion up to and beyond the cut end of optic nerve, 10 of 11 and 11 of 14, respectively; of stage III (pOS = 0), zero of six; and stage IV (pOS = 0.50), three of six. Only those patients with preauricular adenopathy as the only metastatic site survived in the latter group. Acute toxicity was mild. CONCLUSION: Chemotherapy is not warranted to prevent systemic metastasis for intraocular disease. Patients with extraocular orbital disease and had a good outcome with this therapy. Patients with metastatic disease fared poorly, except for those with isolated malignant preauricular adenopathy.

Treatment of retinoblastoma. Results obtained from a prospective study of 51 patients.

During 4 years, 51 patients with retinoblastoma were studied (unilateral 32, and bilateral 19). Treatment consisted of enucleation of those eyes which showed massive involvement followed by radiotherapy when there was optic nerve and/or orbit involvement. Systemic chemotherapy was administered to all patients who underwent nonsurgical treatment and when the histologic examination showed choroidal optic nerve head and/or optic nerve involvement or residual tumor after enucleation. When there was compromise of the central nervous system intrathecal chemotherapy was administered. Nonsurgical treatment consisted of radiotherapy and/or light coagulation. Survival was 90.6% for unilateral cases and 84.2% for the bilateral. The median follow-up was 31 months and 29 months, respectively. Of 19 conservated eyeballs, 16 preserve useful vision (63%).

Comparison of two consecutive trials for treatment of childhood non-Hodgkin's lymphoma.

Two consecutive trials for the treatment of childhood non-Hodgkin's lymphoma were evaluated, carried out by the same cooperative groups. Group A: From June, 1973 to December, 1975, 50 evaluable patients under 16 years of age participated in a study that included vincristine and prednisone plus surgery and/or radiotherapy as induction. This was followed by 2400 rad of cranial radiotherapy plus 5 doses of intrathecal methotrexate-dexamethasone and anti-leukemia (6-mercaptopurine, methotrexate, cyclophosphamide) or anti-lymphoma (cyclophosphamide, vincristine, procarbazine, and prednisone) maintenance treatment. Group B: From January, 1976 to December, 1980, 55 evaluable patients participated in a consecutive study that added Adriamycin (doxorubicin) and cyclophosphamide to the former induction regimen. Central nervous system (CNS) prevention was performed with 5 doses of intrathecal methotrexate-dexamethasone. Maintenance treatment was the same. Prognostic factors as stage and primary site were comparable in both groups. A total of 33 (66%) of 50 children of Group A and 48 (87%) of 55 children of Group B achieved complete remission (P less than 0.005). Disease-free survival at 60 months was 27% in Group A and 49% in Group B; for Stage I-II, 30% in Group A and 85% in Group B (P less than 0.025); for Stage III-IV 28% in Group A and 36% in Group B (not significant). In Group A, 9.1% and in Group B, 8.3% had primary CNS relapse. Both maintenance schedules had the same relapse rate. It was concluded that: (1) the addition of Adriamycin and cyclophosphamide to vincristine-prednisone in Group B produces a higher rate of complete remission in Stage III-IV, a higher rate of disease-free survival in Stage I-II, and a higher survival rate in all stages; (2) CNS prevention with intrathecal methotrexate-dexamethasone is equally effective as cranial radiation plus intrathecal methotrexate-dexamethasone; and (3) anti-leukemia and anti-lymphoma maintenance are equally effective in the context of this study.

[Association of Wilms' tumor, pseudohermaphroditism and glomerular nephropathy. Partial and complete forms of the syndrome]. / Asociación de tumor de Wilms, pseudohermafroditismo y nefropatía glomerular. Formas incompletas y completa del síndrome.

Four patients, followed at the Buenos Aires Cildren's Hospital are reported: one with a nephrotic syndrome and male pseudohermaphroditism, the second with nephrotic syndrome, male pseudohermaphroditism and Wilms' tumor, the third with pseudohermaphroditism and Wilms' tumor and the fourth with a nephrotic syndrome and Wilms' tumor. The cases presenting nephrotic syndrome, all had an early onset and a rapid and fatal course leading to death in renal failure or secondary to a related bacterial infection. The renal histology in all of the cases with nephrotic syndrome, was very similar: diffuse involvement of all glomeruli consisting in a severe increase of the mesangial matrix, with scarce mesengial proliferation.

Non-Hodgkin's lymphoma in children: an analysis of 122 cases from Argentina.

One hundred twenty two children with non-Hodgkin's lymphoma were studied from January 1966 to December 1975. The first group (1966-1972) did not receive an uniform treatment. The second group (1973-1975) entered in a G.A.T.L.A. protocol consisting of: vincristine-prednisone plus surgery and/or radiotherapy as induction treatment, craniocervical radiotherapy and intrathecal methotrexate as CNS preventive treatment and anti-leukemia (6-mercaptopurine, methotrexate and vincristine-prednisone pulses) or anti-lymphoma (COPP) treatment as maintenance, in a randomized trial. Comparison of survival of the two groups are as follows: series 1966-1972, 22% and 20% at 12 and 24 months of evolution, respectively, and series 1973-1975, 33% and 26% at 12 and 24 months, respectively. After 2 years of complete remission we have not seen any relapse. We conclude that 1) this disease is highly malignant and must be treated with more intensive chemotherapeutic treatment, and 2) there is no difference between antileukemia or anti-lymphoma maintenance treatment, as yet.