Hiperplasia nodular focal: diagnóstico a considerar ante una masa hepática / Focal nodular hyperplasia: A diagnosis to consider in a hepatic mass

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Seguimiento a largo plazo del paciente onco-hematológico pediátrico / Long-term follow-up of the pediatric oncology-hematology patient

Se describen las características clínicas, diagnósticas, terapéuticas y de investigación en el paciente oncológico pediátrico. Se revisan los efectos secundarios, su detección y prevención (AU)

We describe the clinical, diagnostic, therapeutic and research characteristics in the pediatric oncologic patient. Side eHects, their detection and prevention are reviewed (AU)

Afectación del sistema nervioso central por enfermedad de Hodgkin en un paciente con histiocitosis de células de Langerhans / Central nervous system involvement of Hodgkin’s disease in a child with Langerhans cell histiocytosis

La histiocitosis de células de Langerhans (HCL) es una enfermedad poco frecuente, que en muy pocos casos se ha descrito asociada a algunas enfermedades malignas, como la enfermedad de Hodgkin (EH). La afectación del sistema nervioso central (SNC) por la EH se presenta con baja frecuencia, generalmente limitada a los casos de enfermedad avanzada y recurrente. Describimos el caso de un niño que desarrolló estas dos raras circunstancias: por un lado, la coexistencia de la EH y la HCL y, por otro, la recaída de la EH en el SNC (AU)

Langerhans cell histiocytosis (LCH) is a rare disease in children and in a few cases has been described associated to some malignancies like Hodgkin’s disease (HD). Central nervous system (CNS) involvement in HD has been observed in few cases with systemic and recurrent diseases. We report a case of a child who developed these two rare clinical conditions, the coexistence of HD and LCH and the relapse of HD in CNS (AU)

Linfoma anaplásico de células grandes endobronquial en la infancia / Endobronchial anaplastic large cell lymphoma in childhood

El linfoma anaplásico de células grandes (LACG) es una enfermedad poco frecuente en la infancia. Su localización más habitual es ganglionar o cutánea; la presentación endobronquial es extremadamente rara. A continuación se presenta el caso clínico de un varón de 10 años, en el que se llegó al diagnóstico de LACG endobronquial casualmente, al realizarle estudios por una lesión ósea benigna, debido a la ausencia de síntomas respiratorios inicialmente (AU)

Anaplastic large cell lymphoma is a very rare disease in childhood. The most common location of this lymphoma is lymph node and skin, with endobronchial involvement being extremely rare. We report a case of a 10-year-old boy diagnosed by chance with an endobronchial anaplastic large cell lymphoma, while he was being investigated for a benign bone disease, due to the initial absence of respiratory symptoms (AU)

[Endobronchial anaplastic large cell lymphoma in childhood]. / Linfoma anaplásico de células grandes endobronquial en la infancia.

Anaplastic large cell lymphoma is a very rare disease in childhood. The most common location of this lymphoma is lymph node and skin, with endobronchial involvement being extremely rare. We report a case of a 10-year-old boy diagnosed by chance with an endobronchial anaplastic large cell lymphoma, while he was being investigated for a a benign bone disease, due to the initial absence of respiratory symptoms.

[Malignant thymoma associated with severe aplastic anaemia]. / Timoma maligno asociado a aplasia medular grave.

Malignant thymoma is a very rare neoplasm in paediatric patients; it is usually associated with para-neoplastic syndromes, the most frequent is myasthenia gravis; some haematological abnormalities may also be present, such as pure red cell aplasia or aplastic anaemia. We report a 12-year-old boy suffering from a very large thymoma, treated with multiple chemotherapy, and who developed a severe aplastic anaemia after surgery. He had a poor response to immunosuppressive treatment and later developed massive pulmonary bleeding as a complication.

Timoma maligno asociado a aplasia medular grave / Malignant thymoma associated with severe aplastic anaemia

El timoma maligno es una neoplasia rara en sujetos pediátricos y generalmente se asocia con síndromes paraneoplásicos; el más típico es la miastenia grave. También se describen alteraciones hematológicas, como la aplasia pura de serie roja o la aplasia medular completa. A continuación se presenta el caso de un varón de 12 años con un timoma maligno de gran tamaño tratado con poliquimioterapia. El sujeto desarrolló una aplasia medular grave después de la cirugía, con mala respuesta al tratamiento inmunosupresor, que evolucionó a hemorragia pulmonar masiva (AU)

Malignant thymoma is a very rare neoplasm in paediatric patients; it is usually associated with para-neoplastic syndromes, the most frequent is myasthenia gravis; some haematological abnormalities may also be present, such as pure red cell aplasia or aplastic anaemia. We report a 12-year-old boy suffering from a very large thymoma, treated with multiple chemotherapy, and who developed a severe aplastic anaemia after surgery. He had a poor response to immunosuppressive treatment and later developed massive pulmonary bleeding as a complication (AU)

Successful treatment with vincristine in PHACES syndrome.

We report a case of PHACES syndrome witch was effectively treated using vincristine. A female infant was referred at 8 months of age for evaluation with a segmental mandibular haemangioma with rapid growing and facial disfigurement. The infant was initially placed on oral prednisone with clinical response but she developed obstructive sleep apnoea. Vincristine was started at about 24 months and continued for 4 months with marked decrease in the size of the haemangioma. The only side effect was constipation.

Successful treatment with vincristine in PHACES syndrome

We report a case of PHACES syndrome witch was effectively treated using vincristine. A female infant was referred at 8 months of age for evaluation with a segmental mandibular haemangioma with rapid growing and facial disfigurement. The infant was initially placed on oral prednisone with clinical response but she developed obstructive sleep apnoea. Vincristine was started at about 24 months and continued for 4 months with marked decrease in the size of the haemangioma. The only side effect was constipation (AU)

Coriocarcinoma mediastínico y metástasis cerebrales. Presentación en un niño con síndrome de Klinefelter / Mediastinal choriocarcinoma with cerebral metastasis. A case report in a child with Klinefelter's syndrome

Propósito: Es importante comunicar los casos de tumores no seminomatosos mediastínicos en la infancia para protocolizar el tratamiento de las metástasis cerebrales. Material y métodos: varón de 13 años con coriocarcinoma en mediastino anterior, múltiples nódulos pulmonares y tac de cráneo normal. Inició tratamiento quimioterápico con excelente respuesta clínica y radiológica. Previo al segundo ciclo de quimioterapia presentó rápida instauración de coma neurológico, muerte por metástasis y hemorragia masiva. Conclusiones: El desarrollo de metástasis cerebrales no detectables al diagnóstico, obligan a discutir la idoneidad de un estudio de extensión intracraneal más exhaustivo, y la conveniencia profiláctica o terapéutica diferente para la diseminación cerebral del coriocarcinoma (AU)

[Hepatic tumors]. / Tumores hepáticos.

This article show our group of liver neoplasm. We studied the presentation way in the benign and malignant tumors. Moreover we compared the therapeutics results between both groups. In our population was more frequent the malignant neoplasm. The first symptom was more frequent the malignant neoplasm. The first symptom was abdominal mass but with more general complaints in the last one. We checked the increase survival with therapeutics as ADR or CDDP.