RESUMO
El liponeurocitoma supratentorial es un tumor excepcional, de predominio en varones jóvenes. Su localización habitual son los ventrículos laterales, siendo excepcional la afectación del iii ventrículo. Se define como un tumor neuronoglial, de bajo grado, con áreas de lipomatosis asociada. La manifestación clínica más frecuente es la derivada de la aparición de hidrocefalia e hipertensión intracraneal secundaria. Presentamos el primer caso descrito en España de esta estirpe tumoral y uno de los pocos presentados, hasta la fecha, en las bases bibliográficas. Se describe el caso de un varón de 33 años de edad, afectado de clínica sensitiva crural y cefalea de reciente inicio. El estudio radiológico mostró la presencia de una gran tumoración supratentorial, intraventricular, multiquística, heterogénea, con áreas de lipomatosis asociada. El paciente fue intervenido, consiguiéndose la exéresis tumoral completa. El diagnóstico definitivo fue de liponeurocitoma supratentorial. La presencia de una tumoración intraventricular supratentorial, con áreas de degeneración grasa, debe obligar a considerar, dentro del diagnóstico diferencial, este tipo de tumor. La resección tumoral completa se considera el tratamiento de elección
Supratentorial liponeurocytoma is a rare tumor, predominatly appearing in young males. It most commonly affects the lateral ventricles, with involvement of the third ventricle being exceptional. It is defined as a low-grade neuroglial tumor, with areas of associated lipomatosis. The most common clinical manifestation is that resulting from the presence of secondary intracranial hypertension and hydrocephalus. We present the first case reported in Spain of this tumor type and one of the few appearing in the literature so far. We report the case of a 33-year-old male patient, suffering from crural sensitive sympoms and recent onset headache. The radiographic study revealed the presence of a large supratentorial tumor; intraventricular, multicystic, heterogeneous and with areas of associated lipomatosis. The patient underwent surgery and complete tumor resection was achieved. The definitive diagnosis was of supratentorial liponeurocytoma. The presence of a supratentorial intraventricular tumor with areas of fatty degeneration should lead us to consider this type of tumor in the differential diagnosis. Complete tumor resection is considered to be the treatment of choice
Assuntos
Humanos , Masculino , Adulto , Neurocitoma/diagnóstico , Neoplasias do Ventrículo Cerebral/diagnóstico , Lipomatose/patologia , Neoplasias Supratentoriais/diagnóstico , Ventrículos Laterais/patologia , Diagnóstico Diferencial , Metaplasia/patologiaRESUMO
Supratentorial liponeurocytoma is a rare tumor, predominantly appearing in young males. It most commonly affects the lateral ventricles, with involvement of the third ventricle being exceptional. It is defined as a low-grade neuroglial tumor, with areas of associated lipomatosis. The most common clinical manifestation is that resulting from the presence of secondary intracranial hypertension and hydrocephalus. We present the first case reported in Spain of this tumor type and one of the few appearing in the literature so far. We report the case of a 33-year-old male patient, suffering from crural sensitive symptoms and recent onset headache. The radiographic study revealed the presence of a large supratentorial tumor; intraventricular, multicystic, heterogeneous and with areas of associated lipomatosis. The patient underwent surgery and complete tumor resection was achieved. The definitive diagnosis was of supratentorial liponeurocytoma. The presence of a supratentorial intraventricular tumor with areas of fatty degeneration should lead us to consider this type of tumor in the differential diagnosis. Complete tumor resection is considered to be the treatment of choice.
