ABSTRACT
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Subject(s)
Humans , Male , Female , Child , Adolescent , Adrenal Cortex Neoplasms/diagnostic imaging , Adrenal Cortex Neoplasms/diagnosis , Adrenal Cortex Neoplasms/etiology , Adrenal Cortex Neoplasms/drug therapy , HormonesABSTRACT
INTRODUCCIÓN: Los tumores de células germinales intracraneales son un grupo poco frecuente de tumores en niños. Comprenden un grupo heterogéneo de neoplasias, que aunque comparten un origen común, presentan comportamientos clínicos muy diferentes. PACIENTES Y MÉTODOS: Análisis retrospectivo de las características epidemiológicas e histológicas, las manifestaciones clínicas y la evolución de 20 pacientes diagnosticados de tumor de células germinales intracraneal en el Hospital Infantil Universitario Niño Jesús de Madrid durante los años 1994-2014. RESULTADOS: Se obtuvieron 20 pacientes: 14 niños y 6 niñas. La edad media fue de 11,1 años (rango 2-18 años). Se realizó confirmación histológica en el 95% de los pacientes. De los 20 pacientes, 14 fueron germinomas puros (70%) y 6 tumores de células germinales no germinomas (30%). Las localizaciones más frecuentes fueron pineal (45%) y supraselar (45%). Los síntomas más frecuentes en el momento del diagnóstico en los tumores de localización pineal fueron cefalea y vómitos (77,77%), seguido de alteraciones visuales (44,4%), y en los tumores de localización supraselar, polidipsia y poliuria (100%). En el momento del diagnóstico recibieron radioterapia el 90% de los pacientes y quimioterapia asociada a la radioterapia el 55%. Presentaron recaída tumoral 4 pacientes (20%), de los cuales 3 fallecieron. La supervivencia global fue del 80%, siendo un 85,7% para los germinomas y un 60% para los no germinomas. CONCLUSIÓN: El tipo histológico más frecuente fue el germinoma. Los tumores de células germinales son un grupo heterogéneo de tumores que conllevan un pronóstico diferente, por lo que un adecuado diagnóstico y estadificación es fundamental para planear el tratamiento
INTRODUCTION: Intracranial germ cell tumours are rare in children. They are a heterogeneous group of neoplasms that show different clinical manifestations despite having a common origin. PATIENTS AND METHODS: A retrospective analysis was carried out on the epidemiological and histological characteristics, clinical manifestations, and outcomes of 20 patients diagnosed with intracranial germ cell tumours in the Nino Jesús Children's Hospital of Madrid from 1994-2014. RESULTS: A total of 20 patients were identified: 14 boys and 6 girls. The mean age was 11.1 years (range 2-18 years). Histological confirmation of the diagnosis was obtained in 95% of the patients. Of the 20 patients, 14 were pure germinoma (70%) and 6 non-seminomatous germ cell tumours (30%). The most frequent locations were pineal (45%) and suprasellar (45%). The most frequent clinical symptoms in pineal tumours at diagnosis were headache and vomiting (77.77%), followed by visual disturbances (44.4%). In suprasellar tumours it was polydipsia and polyuria (100%). At diagnosis, 90% of the patients received radiotherapy, and 55% received chemotherapy combined with radiotherapy. There was a relapse in 4 patients (20%), and 3 of them died. Overall survival was 80%; 85.7% for pure germinomas and 60% for non-seminomatous germ cell tumours. CONCLUSIONS: The most common histological subtype was pure germinoma. Germ cell tumours include heterogeneous disease entities that have a variable prognosis. Thus, an accurate diagnosis is vital for patient counselling and treatment planning
Subject(s)
Humans , Male , Female , Child , Neoplasms, Germ Cell and Embryonal/congenital , Brain Neoplasms/pathology , Retrospective Studies , Central Nervous System/physiology , Yolk Sac/cytology , Magnetic Resonance Spectroscopy/methods , Hormone Replacement Therapy/methods , Neoplasms, Germ Cell and Embryonal/pathology , Brain Neoplasms/metabolism , Spain , Central Nervous System/pathology , Yolk Sac/physiology , Magnetic Resonance Spectroscopy/instrumentation , Hormone Replacement TherapyABSTRACT
INTRODUCTION: Intracranial germ cell tumours are rare in children. They are a heterogeneous group of neoplasms that show different clinical manifestations despite having a common origin. PATIENTS AND METHODS: A retrospective analysis was carried out on the epidemiological and histological characteristics, clinical manifestations, and outcomes of 20 patients diagnosed with intracranial germ cell tumours in the Niño Jesús Children's Hospital of Madrid from 1994-2014. RESULTS: A total of 20 patients were identified: 14 boys and 6 girls. The mean age was 11.1 years (range 2-18 years). Histological confirmation of the diagnosis was obtained in 95% of the patients. Of the 20 patients, 14 were pure germinoma (70%) and 6 non-seminomatous germ cell tumours (30%). The most frequent locations were pineal (45%) and suprasellar (45%). The most frequent clinical symptoms in pineal tumours at diagnosis were headache and vomiting (77.77%), followed by visual disturbances (44.4%). In suprasellar tumours it was polydipsia and polyuria (100%). At diagnosis, 90% of the patients received radiotherapy, and 55% received chemotherapy combined with radiotherapy. There was a relapse in 4 patients (20%), and 3 of them died. Overall survival was 80%; 85.7% for pure germinomas and 60% for non-seminomatous germ cell tumours. CONCLUSIONS: The most common histological subtype was pure germinoma. Germ cell tumours include heterogeneous disease entities that have a variable prognosis. Thus, an accurate diagnosis is vital for patient counselling and treatment planning.
