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)

Tumores corticosuprarrenales pediátricos: imagen de adenomas y carcinomas / Adrenocortical tumors in children: imaging adenomas and carcinomas

Objetivo. El propósito de la revisión es mostrar las características de imagen que presentan los tumores corticosuprarrenales pediátricos (TCSP). Material y métodos. Se realiza una revisión retrospectiva de los pacientes diagnosticados de TCSP en nuestro hospital terciario en el periodo comprendido entre los años 2000 y 2010, desde el punto de vista radiológico y anatomopatológico. Se estudian las características radiológicas mediante ecografía, tomografía computarizada (TC) y resonancia magnética (RM), que ayudarán a orientar la lesión hacia benignidad o malignidad, y el seguimiento de imagen. Resultados Se presentan 8 TCSP: 5 carcinomas, 2 adenomas y un tumor borderline; se clasifican 2 en estadio I, uno en estadio II, 3 en estadio III y 2 en estadio IV. La radiología permitió el diagnóstico de carcinoma en estadio IV en 2 casos, dada la presencia de metástasis iniciales en un paciente y el gran tamaño y desestructuración tumoral en otro, desarrollando posteriormente metástasis. En los otros 6 casos el diagnóstico radiológico fue de aproximación respecto a su naturaleza de carcinoma o adenoma. Conclusiones. Los TCSP son raros en la infancia. Engloban las entidades de adenoma y carcinoma, siendo difíciles de diferenciar histológica y radiológicamente en ausencia de infiltración vascular y/o metástasis. En un paciente en edad pediátrica la combinación de una masa suprarrenal y signos clínicos de hiperfunción corticosuprarrenal es virtualmente diagnóstica de TCSP (AU)

Objective. This article aims to show the imaging characteristics of pediatric adrenocortical tumors. Material and methods. We review the imaging and histological findings in patients diagnosed with pediatric adrenocortical tumors at our tertiary hospital between 2000 and 2010. We analyze the findings at ultrasonography, computed tomography, and magnetic resonance imaging that can help orient the diagnosis toward benign or malignant lesions and guide imaging follow-up. Outcome. We found 8 adrenocortical tumors in children: 5 carcinomas, 2 adenomas, and 1 borderline tumor. Two tumors were classified as stage I, 1 as stage II, 3 as stage III, and 2 as stage IV. Imaging enabled the diagnosis of stage IV carcinoma in 2 cases, due to the presence of initial metastases in one patient and to size of the tumor and structural changes in the other, who later developed metastases. In the other 6 cases, the imaging studies oriented the diagnosis toward carcinoma or adenoma. Conclusions. Adrenocortical tumors are rare in children. Adrenocortical tumors include adenomas and carcinomas, and in the absence of vascular infiltration and/or metastases it is difficult to differentiate between the two types by imaging and histology. The combination of an adrenal mass and clinical signs of adrenocortical hyperfunction in a child is virtually diagnostic of an adrenocortical tumor (AU)

[Adrenocortical tumors in children: imaging adenomas and carcinomas]. / Tumores corticosuprarrenales pediátricos: imagen de adenomas y carcinomas.

OBJECTIVE: This article aims to show the imaging characteristics of pediatric adrenocortical tumors. MATERIAL AND METHODS: We review the imaging and histological findings in patients diagnosed with pediatric adrenocortical tumors at our tertiary hospital between 2000 and 2010. We analyze the findings at ultrasonography, computed tomography, and magnetic resonance imaging that can help orient the diagnosis toward benign or malignant lesions and guide imaging follow-up. OUTCOME: We found 8 adrenocortical tumors in children: 5 carcinomas, 2 adenomas, and 1 borderline tumor. Two tumors were classified as stage I, 1 as stage II, 3 as stage III, and 2 as stage IV. Imaging enabled the diagnosis of stage IV carcinoma in 2 cases, due to the presence of initial metastases in one patient and to size of the tumor and structural changes in the other, who later developed metastases. In the other 6 cases, the imaging studies oriented the diagnosis toward carcinoma or adenoma. CONCLUSIONS: Adrenocortical tumors are rare in children. Adrenocortical tumors include adenomas and carcinomas, and in the absence of vascular infiltration and/or metastases it is difficult to differentiate between the two types by imaging and histology. The combination of an adrenal mass and clinical signs of adrenocortical hyperfunction in a child is virtually diagnostic of an adrenocortical tumor.

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)

Diagnóstico diferido de hernia diafragmática congénita derecha tras sepsis por «Streptococcus» del grupo B / Delayed diagnosis of right-sided congenital diaphragmatic hernia following group B streptococcal sepsis

Se presenta el caso de un recién nacido atendido en el Servicio de Pediatría del Hospital Regional Universitario «Carlos Haya» por sepsis y enfermedad respiratoria, que desarrolló coagulopatía y shock en los primeros días, por lo que se le instauró antibioticoterapia y ventilación mecánica. La bronconeumonía inicial cursó con derrame pleural e imágenes persistentemente alteradas del hemidiafragma derecho. Al final y mediante ecografía, se estableció el diagnóstico de hernia diafragmática congénita derecha. Una vez superado el problema infeccioso, fue intervenido quirúrgicamente con éxito

We present the case of a newborn, treated in our unit for sepsis and respiratory disease. In his first few days of life, he developed coagulopathy and shock, requiring antibiotics and mechanical ventilation. The initial bronchopneumonia was associated with pleural effusion, and the imaging studies repeatedly revealed a defect in right hemidiaphragm that proved to be a congenital diaphragmatic hernia on ultrasound. Once the infection resolved, surgical repair was successfully performed

[Deep venous thrombosis: an early manifestation of Behçet's disease in childhood]. / Trombosis venosa profunda como manifestación precoz de la enfermedad de Behçet en la infancia.

Behcet's disease is a multisystemic vasculitis of unknown origin whose classical triad is oral and genital ulcers and uveitis. Deep venous thrombosis may sometimes be the first manifestation of this disease but is very rare in children. We report the case of a 14-year-old boy with deep venous thrombosis of the leg and a history of severe aphthous gingivostomatitis, perianal ulcers, Henoch-Schonlein purpura, ecchymotic lesions on the lower limbs and intermittent swelling of the left ankle. Complementary tests ruled out the main causes of thrombosis in children. HLA-B5 and B-51 study proved positive, which, together with the patient's history, suggested a diagnosis of Behcet's disease. This disease usually presents around the third decade of life and is very unusual in children. This case is exceptional because thrombosis as an early manifestation of Behcet's disease is very rare in children and because it led to the diagnosis of Behcet's disease in our patient.

Trombosis venosa profunda como manifestación precoz de la enfermedad de Behçet en la infancia / Deep venous thrombosis: an early manifestation of Behçet´s disease in childhood

La enfermedad de Behçet es una vasculitis multisistémica de origen desconocido cuya tríada clásica son aftas orales, úlceras genitales y uveítis. En ocasiones, la trombosis venosa profunda puede ser la primera manifestación, pero es muy rara en niños. Se expone el caso de un varón de 14 años con trombosis venosa profunda del miembro inferior, con antecedentes de gingivostomatitis aftosas graves, úlceras perianales, púrpura de Schönlein-Henoch, lesiones equimóticas en miembros inferiores y tumefacción intermitente del tobillo izquierdo. Mediante pruebas complementarias se descartaron las principales causas de trombosis en niños. La positividad de los antígenos de histocompatibilidad B5 (HLA-B5) y B51, junto con las múltiples manifestaciones previas del paciente llevaron al diagnóstico de la enfermedad de Behçet. Esta se presenta generalmente en torno a la tercera década de la vida, y es poco frecuente en niños. Lo particular de este caso es que la trombosis como manifestación precoz en niños es excepcional y permitió diagnosticar la enfermedad (AU)

Anemia intensa en la primera infancia / Severe anemia in early childhood

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