Enfermedad relacionada con IgG4 con afectación exclusiva nasosinusal: presentación de un caso clínico y revisión de la literatura / IgG4 related disease with exclusive sinonasal involvement: A case report and literature review

La enfermedad relacionada con IgG4 (ER-IgG4) es un proceso patológico que ha unificado un gran número de enfermedades consideradas como propias de distintos órganos cuya etiopatogenia se desconocía. Su diagnóstico se establece mediante criterios histológicos, radiológicos y serológicos. El tratamiento se basa en corticoides durante tiempo prolongado, reservándose el uso de rituximab para casos refractarios. Es extremadamente inusual que esta entidad asiente solo en cabeza y cuello sin afectación sistémica, por lo que hay pocos casos como el que presentamos descritos en la literatura. Exponemos el caso de una paciente con una tumoración orbitaria con afectación de senos paranasales, diagnosticada de ER-IgG4 nasosinusal. La precisión del diagnóstico propició el inicio precoz de tratamiento corticoideo. En la actualidad la paciente se encuentra asintomática

IgG4 related disease (igG4-RD) is a pathological process which integrates a large number of diseases of unknown pathogenesis, considered as being exclusive to many different organs. Diagnosis is established through histological, radiological and serological criteria. Treatment is based on long term corticosteroids; rituximab being used only in refractory cases. It is unusual for this entity to be found exclusively in the head and neck, without systemic involvement; there are only a few reported cases to date. We present a case of a nasosinusal IgG4-RD orbital tumor with paranasal sinus involvement. The accurate diagnosis made early onset corticosteroid treatment possible and the patient is currently asymptomatic

[IgG4 related disease with exclusive sinonasal involvement: A case report and literature review]. / Enfermedad relacionada con IgG4 con afectación exclusiva nasosinusal: presentación de un caso clínico y revisión de la literatura.

IgG4 related disease (igG4-RD) is a pathological process which integrates a large number of diseases of unknown pathogenesis, considered as being exclusive to many different organs. Diagnosis is established through histological, radiological and serological criteria. Treatment is based on long term corticosteroids; rituximab being used only in refractory cases. It is unusual for this entity to be found exclusively in the head and neck, without systemic involvement; there are only a few reported cases to date. We present a case of a nasosinusal IgG4-RD orbital tumor with paranasal sinus involvement. The accurate diagnosis made early onset corticosteroid treatment possible and the patient is currently asymptomatic.

Liponeurocitoma del ventrículo lateral izquierdo. Revisión del primer caso descrito en España y análisis de la bibliografía / Liponeurocytoma in the left lateral ventricle. Presentation of the first case reported in Spain and literature review

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

[Liponeurocytoma in the left lateral ventricle. Presentation of the first case reported in Spain and literature review]. / Liponeurocitoma del ventrículo lateral izquierdo. Revisión del primer caso descrito en España y análisis de la bibliografía.

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.