Solid-cystic supratentorial hemangioblastoma affecting the falx cerebri. Report of a case

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, pariet...

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Veröffentlicht in:Neurocirugía (Asturias, Spain) Spain), 2010-10, Vol.21 (5), p.401-404
Hauptverfasser: Elguezabal, A, Díaz, M L, Landeyro, J, Gené, M, Boutayeb, L, Escosa, M, Sirvent, J J
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container_issue 5
container_start_page 401
container_title Neurocirugía (Asturias, Spain)
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creator Elguezabal, A
Díaz, M L
Landeyro, J
Gené, M
Boutayeb, L
Escosa, M
Sirvent, J J
description 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). 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. 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.
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Report of a case</title><source>MEDLINE</source><source>Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals</source><source>Alma/SFX Local Collection</source><creator>Elguezabal, A ; Díaz, M L ; Landeyro, J ; Gené, M ; Boutayeb, L ; Escosa, M ; Sirvent, J J</creator><creatorcontrib>Elguezabal, A ; Díaz, M L ; Landeyro, J ; Gené, M ; Boutayeb, L ; Escosa, M ; Sirvent, J J</creatorcontrib><description>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). 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. 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. 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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. 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. 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The importance of a correct diagnosis of these histologically benign tumors, lies on the possible association with VHL syndrome and its complications.</abstract><cop>Spain</cop><pmid>21042692</pmid><tpages>4</tpages></addata></record>
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subjects Aged
Dura Mater - anatomy & histology
Dura Mater - pathology
Female
Hemangioblastoma - diagnosis
Hemangioblastoma - pathology
Humans
Magnetic Resonance Imaging
Supratentorial Neoplasms - diagnosis
Supratentorial Neoplasms - pathology
von Hippel-Lindau Disease - complications
von Hippel-Lindau Disease - diagnosis
von Hippel-Lindau Disease - pathology
title Solid-cystic supratentorial hemangioblastoma affecting the falx cerebri. Report of a case
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