Craneofaringioma ectópico y síndrome de Gardner: a propósito de un caso y revisión de la literatura / Ectopic craniopharyngioma and Gardner's syndrome: Case report and literature review

Introducción: El craneofaringioma representa alrededor del 3% de todos los tumores primarios del sistema nervioso central. Por lo general, se localiza en la región supraselar, sin embargo, puede tener una localización atípica. Caso clínico: Presentamos el caso de un varón de 29 años de edad que fue intervenido de un osteoma de mandíbula a los 19 años de edad, y a quien posteriormente se le diagnosticó síndrome de Gardner. Ingresó en nuestro hospital con una paresia facial derecha de un día de evolución acompañada de diplopía. La exploración neurológica confirmó la existencia de una paresia leve del VI y VII pares craneales derechos. La resonancia magnética cerebral mostró una lesión sólido-quística, redondeada, de contorno bien definido, de unos 2cm de diámetro que ocupaba el cuarto ventrículo. El paciente fue intervenido mediante una craneotomía fosa posterior, abordaje telovelar y extirpación completa del tumor implantado a nivel del techo del cuarto ventrículo. El estudio anatomopatológico definitivo informó de un craneofaringioma adamantinomatoso. Conclusión: El craneofaringioma es un tumor que puede aparecer en otra localización diferente de la región selar-supraselar, y por mecanismos etiopatogénicos hasta ahora desconocidos su localización atípica puede guardar relación con alguna forma de poliposis adenomatosa familiar como el síndrome de Gardner

Introduction: Craniopharyngioma accounts for around 3% of all primary tumours of the central nervous system. It is usually located in the suprasellar region, although it may also have an ectopic location. Case report: The case is presented on 29 year-old male who underwent surgery for a jaw osteoma when he was 19 years old and was subsequently diagnosed with Gardner's syndrome. He was admitted in our Hospital with right facial paresis and diplopia of one day onset. The examination showed mild right VII and VI cranial nerves paresis. Magnetic resonance imaging of the brain demonstrated a rounded solid and cystic lesion with well-defined contours of about 2cm in diameter filling the fourth ventricle. The patient underwent a posterior fossa craniotomy using a telovelar approach with complete removal of the tumour implanted at roof level of the fourth ventricle. The final histology of the tumour was reported as adamantinomatous craniopharyngioma. Conclusion: Craniopharyngioma may appear in another location other than the suprasellar region. Its atypical location may be related to Gardner syndrome by still unknown pathogenic mechanisms

[Ectopic craniopharyngioma and Gardner's syndrome: Case report and literature review]. / Craneofaringioma ectópico y síndrome de Gardner: a propósito de un caso y revisión de la literatura.

INTRODUCTION: Craniopharyngioma accounts for around 3% of all primary tumours of the central nervous system. It is usually located in the suprasellar region, although it may also have an ectopic location. CASE REPORT: The case is presented on 29 year-old male who underwent surgery for a jaw osteoma when he was 19 years old and was subsequently diagnosed with Gardner's syndrome. He was admitted in our Hospital with right facial paresis and diplopia of one day onset. The examination showed mild right VII and VI cranial nerves paresis. Magnetic resonance imaging of the brain demonstrated a rounded solid and cystic lesion with well-defined contours of about 2cm in diameter filling the fourth ventricle. The patient underwent a posterior fossa craniotomy using a telovelar approach with complete removal of the tumour implanted at roof level of the fourth ventricle. The final histology of the tumour was reported as adamantinomatous craniopharyngioma. CONCLUSION: Craniopharyngioma may appear in another location other than the suprasellar region. Its atypical location may be related to Gardner syndrome by still unknown pathogenic mechanisms.

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.