Linfoma no Hodgkin: excelentes resultados a expensas de elevada toxicidad del tratamiento / Non-Hodgkin lymphoma: Excellent results at the expense of the high toxicity of the treatment

Introducción : Los linfomas son la tercera neoplasia maligna en los niños y, dentro de ellos, los linfomas no Hodgkin (LNH) representan tan solo el 7% del cáncer en menores de 15 años. La quimioterapia constituye actualmente el tratamiento de elección. El objetivo de este estudio es analizar la toxicidad secundaria al tratamiento en pacientes pediátricos diagnosticados de LNH. Material y métodos: Estudio retrospectivo de pacientes diagnosticados de LNH de células B maduras tratados según el protocolo LMB 2001, desde enero del 2007 hasta febrero del 2014. Se recogieron los datos referentes al diagnóstico, el tratamiento y las toxicidades que desarrollaron durante el mismo. Resultados: Se diagnosticaron 20 LNH de células B maduras: 16 linfomas Burkitt, 2 linfomas difusos de células grandes y 2 leucemias maduras. Un 65% de los pacientes se clasificó al diagnóstico en un estadio de alto grado (III-IV). Los procesos infecciosos graves, la mielosupresión severa, las alteraciones hepáticas y la mucositis fueron las toxicidades más frecuentes. La supervivencia global fue del 95% (19/20). Un paciente falleció por causas no relacionadas con su enfermedad. Conclusión: La mayoría de los pacientes diagnosticados de LNH de células B maduras experimentan toxicidades grado III y IV durante el tratamiento, a pesar de lo cual la supervivencia es excelente (AU)

Introduction: Lymphomas are the third malignancy in children, and within them non-Hodgkin lymphoma (NHL) accounts for just 7% of cancers in children under 15 years old. Chemotherapy is currently the treatment of choice. The objective of this study is to analyze the toxicity caused by the treatment in pediatric patients diagnosed with NHL. Material and methods: A retrospective study was conducted on patients diagnosed with mature B-cell NHL, treated according to the LMB protocol 2001, from January 2007 to February 2014. Data concerning the diagnosis, treatment and toxicities that developed in the patients during the same period were collected. Results: A total of 20 mature B-cell NHL cases were diagnosed: 16 Burkitt lymphomas, 2 diffuse large cell lymphomas and 2 mature leukemias. Almost two-thirds (65%) of patients were classified in a high grade stage (III-IV) at diagnosis. Serious infectious processes, severe myelosuppression, liver abnormalities, and mucositis were the most frequent toxicities. Overall survival was 95% (19/20). One patient died of causes unrelated to the illness. Conclusion: Despite the excellent survival rate, most patients diagnosed with NHL mature B cells experience grade III and IV toxicities during treatment (AU)

Gliomas de bajo grado: revisión de 10 años / Low-grade gliomas: Review of 10 years

INTRODUCCIÓN: Los tumores del SNC son los tumores sólidos más frecuentes en la edad pediátrica. Dentro de ellos los gliomas de bajo grado constituyen el tipo más común de tumor del SNC en niños, representando hasta el 30-50% de los mismos. PACIENTES Y MÉTODOS: Análisis retrospectivo de las características epidemiológicas, manifestaciones clínicas, localización del tumor, histología, tipo de tratamiento si lo ha recibido, evolución y secuelas a largo plazo de 111 pacientes diagnosticados de glioma de bajo grado en el Hospital Infantil Universitario Niño Jesús de Madrid entre enero de 2002 y diciembre de 2011. RESULTADOS: De los 111 pacientes 57 eran niños y 54 niñas. La edad media fue de 7,26 años (intervalo 2 meses-19 anos). Los síntomas de presentación más frecuentes fueron la cefalea (27%) y los vómitos (19%). Las localizaciones más frecuentes fueron los hemisferios cerebrales (38%), seguido del tronco cerebral (27,4%) y del cerebelo (18,5%). Se realizó estudio histológico en 89 pacientes (80,18%), siendo el astrocitoma pilocítico el tipo histológico más frecuente. Se realizó biopsia diagnóstica en 20 pacientes (22,5%), resección parcial en 38 pacientes (42,7%) y resección total en 31 pacientes (34,8%). Recibieron quimioterapia 16 pacientes (14%) y radioterapia 18 pacientes (16%). La supervivencia global fue del 88,3%. Un paciente presentó secuelas auditivas, 5 pacientes presentaron secuelas visuales y 4 pacientes secuelas endocrinas. CONCLUSIONES: El tipo histológico más frecuente es el astrocitoma pilocítico. La supervivencia global fue del 88,3%. Solo el 9% de los pacientes presentaron algún tipo de secuela auditiva, visual o endocrinológica

INTRODUCTION: Central nervous system (CNS) tumors are the most common solid tumors in children. Among these, the low-grade gliomas are the most common type, accounting for up to 30-50% of them. PATIENTS AND METHODS: A retrospective analysis was carried out on the epidemiology, clinical characteristics, tumor location, histology, treatment, outcome and long-term sequelae of 111 patients diagnosed with low-grade glioma in the Nino˜ Jesús Children's Hospital of Madrid from January 2002 to December 2011. RESULTS: Of the 111 patients, there were 57 boys and 54 girls. The mean age was 7.26 years (range, 2 months - 19 years). The most common symptoms of presentation were headache (27%) and vomiting (19%). The most common locations were the cerebral hemispheres (38%), followed by the brainstem (27.4%), and cerebellum (18.5%). Histological examination was performed in 89 patients (80.18%). Pilocytic astrocytoma was the most common histological type. Diagnostic biopsy was performed in 20 patients (22.5%), partial resection in 38 patients (42.7%), and total resection in 31 patients (34.8%). Sixteen patients received chemotherapy (14%), and eighteen patients received radiotherapy (16%). Overall survival was 88.3%. Long term hearing, visual and endocrine sequelae were note in 1, 5, and 4 patients, respectively. CONCLUSIONS: The most common histological type is pilocytic astrocytoma. Overall survival was 88.3%. Only 9% of patients had some kind or auditory, visual or endocrine sequelae

[Non-Hodgkin lymphoma: Excellent results at the expense of the high toxicity of the treatment]. / Linfoma no Hodgkin: excelentes resultados a expensas de elevada toxicidad del tratamiento.

INTRODUCTION: Lymphomas are the third malignancy in children, and within them non-Hodgkin lymphoma (NHL) accounts for just 7% of cancers in children under 15 years old. Chemotherapy is currently the treatment of choice. The objective of this study is to analyze the toxicity caused by the treatment in pediatric patients diagnosed with NHL. MATERIAL AND METHODS: A retrospective study was conducted on patients diagnosed with mature B-cell NHL, treated according to the LMB protocol 2001, from January 2007 to February 2014. Data concerning the diagnosis, treatment and toxicities that developed in the patients during the same period were collected. RESULTS: A total of 20 mature B-cell NHL cases were diagnosed: 16 Burkitt lymphomas, 2 diffuse large cell lymphomas and 2 mature leukemias. Almost two-thirds (65%) of patients were classified in a high grade stage (iii-iv) at diagnosis. Serious infectious processes, severe myelosuppression, liver abnormalities, and mucositis were the most frequent toxicities. Overall survival was 95% (19/20). One patient died of causes unrelated to the illness. CONCLUSION: Despite the excellent survival rate, most patients diagnosed with NHL mature B cells experience grade iii and iv toxicities during treatment.

All children with malignant rhabdoid tumors should be treated initially with intensive chemotherapy.

Malignant rhabdoid tumors (MRT) of soft tissues are aggressive tumors, which can be detected in almost any part of the body. MRT are rare, and very few cases have been reported in the literature. Prognosis of these tumors is extremely poor despite intensive therapy. Some risk factors such as young age or disseminated disease are associated with an aggressive and almost always lethal clinical course. Some clinicians even recommend initial palliative care due to this outcome. We report a case of metastatic MRT in a 6-month-old child with excellent initial response to chemotherapy.

[Low-grade gliomas: review of 10 years]. / Gliomas de bajo grado: revisión de 10 años.

INTRODUCTION: Central nervous system (CNS) tumors are the most common solid tumors in children. Among these, the low-grade gliomas are the most common type, accounting for up to 30-50% of them. PATIENTS AND METHODS: A retrospective analysis was carried out on the epidemiology, clinical characteristics, tumor location, histology, treatment, outcome and long-term sequelae of 111 patients diagnosed with low-grade glioma in the Niño Jesús Children's Hospital of Madrid from January 2002 to December 2011. RESULTS: Of the 111 patients, there were 57 boys and 54 girls. The mean age was 7.26 years (range, 2 months - 19 years). The most common symptoms of presentation were headache (27%) and vomiting (19%). The most common locations were the cerebral hemispheres (38%), followed by the brainstem (27.4%), and cerebellum (18.5%). Histological examination was performed in 89 patients (80.18%). Pilocytic astrocytoma was the most common histological type. Diagnostic biopsy was performed in 20 patients (22.5%), partial resection in 38 patients (42.7%), and total resection in 31 patients (34.8%). Sixteen patients received chemotherapy (14%), and eighteen patients received radiotherapy (16%). Overall survival was 88.3%. Long term hearing, visual and endocrine sequelae were note in 1, 5, and 4 patients, respectively. CONCLUSIONS: The most common histological type is pilocytic astrocytoma. Overall survival was 88.3%. Only 9% of patients had some kind or auditory, visual or endocrine sequelae.

Irinotecán y temozolomida en tumores sólidos pediátricos en recaída o refractarios / Irinotecan plus temozolomide in refractory or relapsed pediatric solid tumors

Introducción: El pronóstico de los tumores sólidos pediátricos refractarios o en recaída es infausto, y actualmente no existen tratamientos estandarizados para este tipo de situaciones. La combinación irinotecán y temozolomida ha demostrado su utilidad en adultos como segunda línea de tratamiento en distintos tumores sólidos. En pacientes pediátricos ya ha demostrado su eficacia en sarcoma de Ewing, neuroblastoma o rabdomiosarcoma en recaída o refractario. Pacientes y métodos: Se ha realizado un estudio retrospectivo de 32 pacientes pediátricos con tumores sólidos en recaída o refractarios que fueron tratados con irinotecán y temozolomida desde septiembre de 2005 hasta junio de 2012 en el Servicio de Oncología del Hospital Infantil Niño Jesús. Se analizaron las características clínicas, el tratamiento realizado, la toxicidad presentada y la respuesta obtenida. Resultados: Treinta y dos pacientes recibieron un total de 180 ciclos. De 30 pacientes valorables, 10 (33%) presentaron respuesta positiva (2 remisión completa y 8 remisión parcial) y 8 (27%) mantuvieron enfermedad estable. El 94% de los pacientes que obtuvo respuesta lo hicieron en los 4 primeros ciclos. De los 180 ciclos analizados, solo 50 (28%) presentaron toxicidad y, de ellos, solo 15 (8%) fueron de grado III-IV . La toxicidad más frecuente fue la diarrea que apareció en 18 pacientes. Todos los pacientes recibieron el tratamiento de forma ambulatoria, excepto 3 que precisaron ingreso por síntomas relacionados con su enfermedad de base. Conclusión: La combinación irinotecán y temozolomida es bien tolerada y activa frente a tumores sólidos pediátricos refractarios al tratamiento o en recaída (AU)

Introduction: The prognosis of refractory or relapsed pediatric solid tumors is very poor, and there is no standard treatment for this condition. The combination of irinotecan and temozolomide has proved useful in adults as a second-line treatment of different solid tumors. In pediatric patients, this combination has been effective in Ewing's sarcoma, neuroblastoma, and relapsed or refractory rhabdomyosarcoma. Patients and methods: A retrospective study was conducted on 32 pediatric patients with refractory or relapsed solid tumors, who were treated with irinotecan and temozolomide in the Oncology Department at Children's Hospital Niño Jesus from September 2005 to June 2012. The clinical characteristics, treatment performed, toxicity and outcome, were analyzed. Results: Thirty-two patients received a total of 180 cycles. Of the 30 evaluable patients, 10 (33%) had a positive response (2 complete remission and 8 partial remission), and in 8 (27%) the disease remained stable. Almost all (94%) of the patients achieved a response in the first four cycles. Of the 180 cycles analyzed, only 50 (28%) had toxicity, and of these only 15 (8%) were grade III-IV . The most common toxicity was diarrhea appearing in 18 patients. All patients received ambulatory treatment, except three of them who required hospitalization due to symptoms of their underlying disease. Conclusion: The combination of irinotecan and temozolomide is well tolerated and active against pediatric refractory or relapsed solid tumors (AU)

[Irinotecan plus temozolomide in refractory or relapsed pediatric solid tumors]. / Irinotecán y temozolomida en tumores sólidos pediátricos en recaída o refractarios.

INTRODUCTION: The prognosis of refractory or relapsed pediatric solid tumors is very poor, and there is no standard treatment for this condition. The combination of irinotecan and temozolomide has proved useful in adults as a second-line treatment of different solid tumors. In pediatric patients, this combination has been effective in Ewing's sarcoma, neuroblastoma, and relapsed or refractory rhabdomyosarcoma. PATIENTS AND METHODS: A retrospective study was conducted on 32 pediatric patients with refractory or relapsed solid tumors, who were treated with irinotecan and temozolomide in the Oncology Department at Children's Hospital Niño Jesus from September 2005 to June 2012. The clinical characteristics, treatment performed, toxicity and outcome, were analyzed. RESULTS: Thirty-two patients received a total of 180 cycles. Of the 30 evaluable patients, 10 (33%) had a positive response (2 complete remission and 8 partial remission), and in 8 (27%) the disease remained stable. Almost all (94%) of the patients achieved a response in the first four cycles. Of the 180 cycles analyzed, only 50 (28%) had toxicity, and of these only 15 (8%) were grade iii-iv. The most common toxicity was diarrhea appearing in 18 patients. All patients received ambulatory treatment, except three of them who required hospitalization due to symptoms of their underlying disease. CONCLUSION: The combination of irinotecan and temozolomide is well tolerated and active against pediatric refractory or relapsed solid tumors.

Candidiasis hepatoesplénica en pacientes hemato-oncológicos pediátricos / Hepatosplenic candidiasis in paediatric haematology-oncology patients

Introducción: En las últimas décadas, la supervivencia en las enfermedades hemato-oncológicas infantiles ha aumentado gracias a la mejora de los tratamientos, con regímenes quimioterápicos más agresivos. Esto ha conducido a un aumento en la incidencia de infecciones graves, con la consecuente morbimortalidad. La candidiasis hepatoesplénica es una infección diseminada por Candida que suele afectar más frecuentemente al hígado y bazo, aunque pueden verse involucrados otros órganos. Pacientes y métodos: Se ha realizado un estudio retrospectivo de 13 pacientes pediátricos diagnosticados de candidiasis hepatoesplénica desde enero de 2002 a febrero de 2010 en el Servicio de Hemato-Oncología pediátrica del Hospital Niño Jesús de Madrid siguiendo los criterios propuestos por la EORTC/MSG (European Organization for Research and Treatment of Cancer and Mycoses Study Group) revisados en el 2008. Se analizaron las características clínicas, los métodos diagnósticos, el tratamiento realizado y la evolución posterior. Resultados: El síntoma más frecuente de presentación de la candidiasis hepatoesplénica en nuestra serie fue la fiebre persistente, hasta en un 84,6% de los casos, en casi todos, asociada a algún otro síntoma. Los factores de riesgo para desarrollarla son bastante inespecíficos y estaban presentes hasta en un 92,3% de nuestros pacientes. El diagnóstico de infección probada, que se realiza mediante histología o cultivos, normalmente es difícil de obtener y sólo se logró obtener en nuestro caso en un 23,1%. La mayoría de las veces, como en nuestra serie, sólo se llega a un diagnóstico de sospecha. Las pruebas de laboratorio no tienen utilidad en la población pediátrica y tan sólo en un paciente se apreció aumento de la fosfatasa alcalina al diagnóstico. Este resultado difiere del comportamiento de este marcador biológico en los adultos. Aunque suelen ser necesarios tratamientos prolongados para la resolución de las lesiones, la supervivencia es muy alta, en nuestra serie un 100%, con un tratamiento adecuado. Conclusión: La mortalidad de la candidiasis hepatoesplénica es baja si se realiza un tratamiento precoz y adecuado (AU)

Introduction: In the last few decades, the survival of children with haematology-oncological malignancies has increased due to more aggressive chemotherapy regimens. This has led to an increase of fungal infections causing significant morbidity and mortality in these patients. Hepatosplenic candidiasis is a disseminated candida infection that affects most commonly the liver and spleen, although other organs may be involved. Patients and methods: We performed a retrospective study of 13 paediatric patients diagnosed with hepatosplenic candidiasis from January 2002 to February 2010 in our paediatric haematology-oncology department following the criteria proposed by the EORTC/MSG (European Organization for Research and Treatment of Cancer and Mycoses study group) updated in2008. We analysed the clinical characteristics, diagnostic methods, treatment and outcome. Results: The most common symptom of presentation of hepatosplenic candidiasis in our series was persistent fever, up 84.6% of cases, almost all associated with other symptoms. Risk factors for development are non-specific and were present in 92.3% of our patients. The diagnosis of proven infection, which is made by histology or culture, is usually difficult to obtain and in our series it was obtained only in 23.1% of cases. As in our series, diagnosis is often presumptive. Laboratory tests are not useful in the paediatric population and only in one patient we observed an increase in alkaline phosphatase at diagnosis. This result differs from the behaviour of this biological marker in adults. Although prolonged treatment is often necessary for the resolution of the lesions, survival is high with appropriate treatment, 100% in our series. Conclusions: The mortality of hepatosplenic candidiasis is low with early and adequate treatment (AU)

[Hepatosplenic candidiasis in paediatric haematology-oncology patients]. / Candidiasis hepatoesplénica en pacientes hemato-oncológicos pediátricos.

INTRODUCTION: In the last few decades, the survival of children with haematology-oncological malignancies has increased due to more aggressive chemotherapy regimens. This has led to an increase of fungal infections causing significant morbidity and mortality in these patients. Hepatosplenic candidiasis is a disseminated candida infection that affects most commonly the liver and spleen, although other organs may be involved. PATIENTS AND METHODS: We performed a retrospective study of 13 paediatric patients diagnosed with hepatosplenic candidiasis from January 2002 to February 2010 in our paediatric haematology-oncology department following the criteria proposed by the EORTC/MSG (European Organization for Research and Treatment of Cancer and Mycoses study group) updated in 2008. We analysed the clinical characteristics, diagnostic methods, treatment and outcome. RESULTS: The most common symptom of presentation of hepatosplenic candidiasis in our series was persistent fever, up 84.6% of cases, almost all associated with other symptoms. Risk factors for development are non-specific and were present in 92.3% of our patients. The diagnosis of proven infection, which is made by histology or culture, is usually difficult to obtain and in our series it was obtained only in 23.1% of cases. As in our series, diagnosis is often presumptive. Laboratory tests are not useful in the paediatric population and only in one patient we observed an increase in alkaline phosphatase at diagnosis. This result differs from the behaviour of this biological marker in adults. Although prolonged treatment is often necessary for the resolution of the lesions, survival is high with appropriate treatment, 100% in our series. CONCLUSIONS: The mortality of hepatosplenic candidiasis is low with early and adequate treatment.