[Solid-cystic supratentorial hemangioblastoma affecting the falx cerebri. Report of a case]. / Hemangioblastoma sólido-quístico supratentorial afectando la hoz del cerebro. Presentación de un caso.

INTRODUCTION: hemangioblastomas are benign neoplasias that are originated in the central nervous system and constitute between 1.5-2.5% of intracranial tumors. The majority of them are infratentorial, mainly affecting the cerebellum (76%). Supratentorial lessions are rare, being in these cases the frontal, parietal or temporal lobes the most common locations. Meningeal involvement is infrequent. Only eight cases have been reported in the literature. In 30% of the cases, these tumors are associated with von Hippel Lindau syndrome (VHL). CASE REPORT: 67 year old woman without any medical or family history. She presented with 4 month evolution neurological symptoms. The cranial MRI scan showed a solitary solid-cystic lesion on the right paramedian frontal lobe, in contact with the falx cerebri. The pathological analysis showed a cellular proliferation composed of polygonal cells with clear cytoplasm due to the presence of intracytoplasmic vacuoles and round or oval nucleus without cytologic atypia. These cells were accompanied by a rich vascular network of capillary type and blood extravasation. She was diagnosed of supratentorial hemangioblastoma. CONCLUSION: the preoperative diagnosis of these neoplasms is difficult because the clinical suspicion is low in supratentorial location. Imaging techniques are useful but definitive diagnosis is made through pathologic examination. The use of immunohistochemical techniques is helpful for the differential diagnosis with lesions that are more common in this region. The importance of a correct diagnosis of these histologically benign tumors, lies on the possible association with VHL syndrome and its complications.

[Neuro-Behçet: clinicopathological findings in an autopsy case]. / Neurobehçet: hallazgos clinicopatológicos de un caso de autopsia.

INTRODUCTION: Behçet's disease (BD) was first described by Hulusi Behçet in 1937 as a triad of oral aphthae, genital ulcers and uveitis. It affects most of tissues and organs without exception and has a prevalence of neurologic involvement between 5 and 30%. Histopathological research of autopsy cases is limited, even though the cause of death is confirmed in this study. CASE REPORT: A 30-years-old man without prior medical history of interest debuting with a BD with progressive neurological manifestations and fatal evolution. The autopsy showed, in gross examination, oral and scrotal ulcers. Moreover, the brain revealed a marked cerebral edema, opaque leptomeninges and hemorrhagic infarction of the left frontal lobule extended to the homolateral ventricular cavity. In the brain stem, multiples hemorrhagic infarctions and hemorrhage in the fourth ventricle. The microscopic examination showed a generalized perivascular lymphocytic and neutrophilic inflammation in small and medium vessels with intense diapedesis bleeding. This phenomenon was predominant in brain stem. CONCLUSIONS: The necropsy studies of patients with neuro-Behcet's disease are scarce. We report the clinicopathological findings in a patient with BD and neurological involvement, in which stands out a cerebral vascular affectation predominant in brain stem.

Hemangioblastoma sólido-quístico supratentorial afectando la hoz del cerebro. Presentación de un caso / Solid-cystic supratentorial hemangioblastoma affecting the falx cerebri. Report of a case

Introducción. Los hemangioblastomas son neoplasias benignas que se originan en el sistema nervioso central y constituyen entre un 1,5-2,5% de los tumores intracraneales. Mayoritariamente son de localización infratentorial, afectando principalmente al cerebelo (76%). Las lesiones supratentoriales son sumamente raras, siendo en estos casos la localización más habitual el lóbulo frontal, parietal o temporal. La afectación meníngea es excepcional, habiéndose descrito sólo ocho casos en la literatura. En un 30% de los casos, estos tumores se asocian al síndrome de von Hippel Lindau (VHL).Caso clínico. Mujer de 67 años sin antecedentes patológicos ni familiares de interés que se presentó conclínica neurológica de 4 meses de evolución. El estudio de resonancia magnética craneal demostró una lesión única sólido-quística frontal paramedial derecha, encontacto con la hoz del cerebro, que se orientó como meningioma. El estudio anatomo-patológico de la pieza quirúrgica objetivó una proliferación celular constituida por células poligonales con citoplasma claro debido a la presencia de vacuolas intracitoplasmáticas y núcleo redondo u oval sin atipia citológica. Estas células estaban acompañadas de una rica red vascular de tipocapilar, con anastomosis y extravasación sanguínea. Se diagnosticó de hemangioblastoma supratentorial. Discusión. El diagnóstico preoperatorio de estas neoplasias es difícil debido a que la sospecha clínica esbaja cuando se halla en localización supratentorial. Las técnicas de imagen son de utilidad, realizándose el diagnóstico definitivo a través del estudio anatomopatológico. El uso de técnicas de inmunohistoquímica es de gran ayuda para el diagnóstico diferencial con lesiones que habitualmente se localizan en esta región. La importancia de un diagnóstico correcto de estos tumores histológicamente benignos, radica, entre otras cosas,en la posible asociación con en síndrome de VHL y suscomplicaciones (AU)

Hemangioblastomas are benign neoplasias that are originated in the central nervous systemand constitute between 1.5-2.5% of intracranial tumors.The majority of them are infratentorial, mainly affectingthe cerebellum (76%). Supratentorial lessions are rare,being in these cases the frontal, parietal or temporallobes the most common locations. Meningeal involvementis in frequent. Only eight cases have been reported in the literature. In 30% of the cases, these tumors areas sociated with von Hippel Lindau syndrome (VHL).Case report. 67 year old woman without any medicalor family history. She presented with 4 month evolution neurological symptoms. The cranial MRI scan showed a solitary solid-cystic lesion on the right paramedianfrontal lobe, in contact with the falx cerebri. The pathological analysis showed a cellular proliferation composed of polygonal cells with clear cytoplasm due to the presence of intracytoplasmic vacuoles and round oroval nucleus without cytologic atypia. These cells wereaccompanied by a rich vascular network of capillarytype and blood extravasation. She was diagnosed ofsupratentorial hemangioblastoma. Conclusion. The preoperative diagnosis of these neoplasmsis difficult because the clinical suspicion is low in supratentorial location. Imaging techniques are useful but definitive diagnosis is made through pathologic examination. The use of immunohistochemical techniquesis helpful for the differential diagnosis with lesions that are more common in this region. The importance of acorrect diagnosis of these histologically benign tumors,lies on the possible association with VHL syndrome and its complications (AU)

Neurobehçet: hallazgos clinicopatológicos de un caso de autopsia / Neuro-behçet: Clinicopathological findings in an autopsy case

Introducción. La enfermedad de Behçet (EB) la describió por primera vez en 1937 Hulusi Behçet como la tríada deúlceras orales, úlceras genitales y uveítis. Involucra a casi todos los tejidos y órganos sin excepción, y la tasa de afectación del sistema nervioso central es del 5 al 30%. Los estudios histopatológicos de casos de necropsias son escasos, a pesar de que la causa de muerte se constata en este estudio. Caso clínico. Varón de 30 años que comenzó con EB con afectación neurológicade evolución progresiva y fatal. El estudio autópsico mostró en el examen externo úlceras orales y escrotales. El encéfalo presentaba marcado edema cerebral, meninges opacas y un infarto hemorrágico frontal izquierdo con extensión hacia el ventrículo lateral homólogo, y el tronco del encéfalo, infartos hemorrágicos confluentes y hemorragia del cuarto ventrículo. En el examen de los órganos restantes destacaban múltiples infartos pulmonares bilaterales. El estudio histológico del encéfalo mostró, de forma generalizada, predominante en el tronco del encéfalo, vasos de pequeño y mediano calibre rodeados por una corona de linfocitos y neutrófilos, con intensa extravasación eritrocitaria perivascular. Conclusiones. Los estudios denecropsias de pacientes con neurobehçet son escasos. Presentamos los hallazgos clínicos y patológicos de un paciente con EB con afectación neurológica, en el que destaca una afectación vascular cerebral predominante en el tronco del encéfalo

Introduction. Behçet’s disease (BD) was first described by Hulusi Behçet in 1937 as a triad of oral aphthae, genital ulcers and uveitis. It affects most of tissues and organs without exception and has a prevalence of neurologic involvement between 5 and 30%. Histopathological research of autopsy cases is limited, even though the cause of death is confirmed in thisstudy. Case report. A 30-years-old man without prior medical history of interest debuting with a BD with progressive neurological manifestations and fatal evolution. The autopsy showed, in gross examination, oral and scrotal ulcers. Moreover, the brain revealed a marked cerebral edema, opaque leptomeninges and hemorrhagic infarction of the left frontal lobuleextended to the homolateral ventricular cavity. In the brain stem, multiples hemorrhagic infarctions and hemorrhage in the fourth ventricle. The microscopic examination showed a generalized perivascular lymphocytic and neutrophilic inflammationin small and medium vessels with intense diapedesis bleeding. This phenomenon was predominant in brain stem. Conclusions. The necropsy studies of patients with neuro-Behçet’s disease are scarce. We report the clinicopathological findings in a patient with BD and neurological involvement, in which stands out a cerebral vascular affectation predominant in brain stem