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Hemangioblastoma

Los LOS Neisseria hemangioblastomas Hemangioblastomas A benign tumor of the nervous system that may occur sporadically or in association with von Hippel-Lindau disease. It accounts for approximately 2% of intracranial tumors, arising most frequently in the cerebellar hemispheres and vermis. Histologically, the tumors are composed of multiple capillary and sinusoidal channels lined with endothelial cells and clusters of lipid-laden pseudoxanthoma cells. Usually solitary, these tumors can be multiple and may also occur in the brain stem, spinal cord, retina, and supratentorial compartment. Cerebellar hemangioblastomas usually present in the third decade with intracranial hypertension, and ataxia. Von Hippel-Lindau Disease son neoplasias vasculares del SNC. Los LOS Neisseria hemangioblastomas Hemangioblastomas A benign tumor of the nervous system that may occur sporadically or in association with von Hippel-Lindau disease. It accounts for approximately 2% of intracranial tumors, arising most frequently in the cerebellar hemispheres and vermis. Histologically, the tumors are composed of multiple capillary and sinusoidal channels lined with endothelial cells and clusters of lipid-laden pseudoxanthoma cells. Usually solitary, these tumors can be multiple and may also occur in the brain stem, spinal cord, retina, and supratentorial compartment. Cerebellar hemangioblastomas usually present in the third decade with intracranial hypertension, and ataxia. Von Hippel-Lindau Disease son raros y a menudo se asocian con la enfermedad de von Hippel-Lindau. La presentación más común es cefalea y, según el tamaño y la ubicación del tumor Tumor Inflammation, los LOS Neisseria pacientes pueden presentar déficits sensoriales y debilidad motora. La imagenología es el principal método diagnóstico y se requiere una evaluación histopatológica para el diagnóstico definitivo. La cirugía a menudo está indicada en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum el tratamiento de los LOS Neisseria hemangioblastomas Hemangioblastomas A benign tumor of the nervous system that may occur sporadically or in association with von Hippel-Lindau disease. It accounts for approximately 2% of intracranial tumors, arising most frequently in the cerebellar hemispheres and vermis. Histologically, the tumors are composed of multiple capillary and sinusoidal channels lined with endothelial cells and clusters of lipid-laden pseudoxanthoma cells. Usually solitary, these tumors can be multiple and may also occur in the brain stem, spinal cord, retina, and supratentorial compartment. Cerebellar hemangioblastomas usually present in the third decade with intracranial hypertension, and ataxia. Von Hippel-Lindau Disease, aunque según el tamaño, el número y la ubicación de los LOS Neisseria tumores, también se puede justificar la radioterapia. El pronóstico suele ser bueno en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum los LOS Neisseria hemangioblastomas Hemangioblastomas A benign tumor of the nervous system that may occur sporadically or in association with von Hippel-Lindau disease. It accounts for approximately 2% of intracranial tumors, arising most frequently in the cerebellar hemispheres and vermis. Histologically, the tumors are composed of multiple capillary and sinusoidal channels lined with endothelial cells and clusters of lipid-laden pseudoxanthoma cells. Usually solitary, these tumors can be multiple and may also occur in the brain stem, spinal cord, retina, and supratentorial compartment. Cerebellar hemangioblastomas usually present in the third decade with intracranial hypertension, and ataxia. Von Hippel-Lindau Disease solitarios, pero los LOS Neisseria tumores asociados con von Hippel-Lindau a menudo se asocian con un peor pronóstico y un mayor riesgo de recurrencia.

Last updated: Dec 15, 2025

Editorial responsibility: Stanley Oiseth, Lindsay Jones, Evelin Maza

Descripción General

Definición

Los LOS Neisseria hemangioblastomas Hemangioblastomas A benign tumor of the nervous system that may occur sporadically or in association with von Hippel-Lindau disease. It accounts for approximately 2% of intracranial tumors, arising most frequently in the cerebellar hemispheres and vermis. Histologically, the tumors are composed of multiple capillary and sinusoidal channels lined with endothelial cells and clusters of lipid-laden pseudoxanthoma cells. Usually solitary, these tumors can be multiple and may also occur in the brain stem, spinal cord, retina, and supratentorial compartment. Cerebellar hemangioblastomas usually present in the third decade with intracranial hypertension, and ataxia. Von Hippel-Lindau Disease son neoplasias del SNC raras, de crecimiento lento, benignas y altamente vascularizadas que tienen una alta asociación con la enfermedad de von Hippel-Lindau, que es una condición autosómica dominante caracterizada por una variedad de tumores benignos y malignos.

  • Los LOS Neisseria tumores se encuentran con mayor frecuencia en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum el cerebelo.
  • Los LOS Neisseria hemangioblastomas Hemangioblastomas A benign tumor of the nervous system that may occur sporadically or in association with von Hippel-Lindau disease. It accounts for approximately 2% of intracranial tumors, arising most frequently in the cerebellar hemispheres and vermis. Histologically, the tumors are composed of multiple capillary and sinusoidal channels lined with endothelial cells and clusters of lipid-laden pseudoxanthoma cells. Usually solitary, these tumors can be multiple and may also occur in the brain stem, spinal cord, retina, and supratentorial compartment. Cerebellar hemangioblastomas usually present in the third decade with intracranial hypertension, and ataxia. Von Hippel-Lindau Disease suelen encontrarse en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum el parénquima unido a la piamadre o cerca de ella, una parte de las meninges Meninges The brain and the spinal cord are enveloped by 3 overlapping layers of connective tissue called the meninges. The layers are, from the most external layer to the most internal layer, the dura mater, arachnoid mater, and pia mater. Between these layers are 3 potential spaces called the epidural, subdural, and subarachnoid spaces. Meninges: Anatomy (formadas por 3 capas fibrosas) que recubren el SNC:
    • Duramadre: la capa externa, que forma una cubierta de colágeno resistente
    • Aracnoides: capa intermedia que consta de epitelio escamoso simple con una malla suelta de colágeno y fibras elásticas
    • Piamadre: capa interna delgada en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum contacto con el cerebro y la médula espinal
Tabla: Clasificación de los LOS Neisseria tumores del sistema nervioso
Categorías Tumores específicos
Tumores neuroepiteliales en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum el SNC
  • Astrocitomas, incluido el glioblastoma multiforme Glioblastoma multiforme Glioblastoma multiforme is a high-grade astrocytoma, an aggressive brain tumor arising from astrocytes, with an unknown cause and a poorly understood link to risk factors. There are two main types: primary, a more aggressive form seen more commonly in older patients, and secondary, developing from lower-grade astrocytomas and seen more commonly in younger patients. Glioblastoma Multiforme
  • Oligodendroglioma Oligodendroglioma Oligodendrogliomas are malignant CNS tumors arising from neural glial cell precursors. Oligodendrogliomas often arise in the frontal lobes of the brain and have a generally favorable prognosis when compared to other gliomas. Oligodendrogliomas are the 3rd most common CNS tumor. The most frequent presenting symptom is a seizure. Oligodendroglioma
  • Ependimoma y tumores del plexo coroideo
  • Meduloblastomas (tumores embrionarios)
Tumores meníngeos
  • Meningiomas
  • Hemangioblastomas Hemangioblastomas A benign tumor of the nervous system that may occur sporadically or in association with von Hippel-Lindau disease. It accounts for approximately 2% of intracranial tumors, arising most frequently in the cerebellar hemispheres and vermis. Histologically, the tumors are composed of multiple capillary and sinusoidal channels lined with endothelial cells and clusters of lipid-laden pseudoxanthoma cells. Usually solitary, these tumors can be multiple and may also occur in the brain stem, spinal cord, retina, and supratentorial compartment. Cerebellar hemangioblastomas usually present in the third decade with intracranial hypertension, and ataxia. Von Hippel-Lindau Disease
Tumores de la región selar
  • Craneofaringioma
  • Adenoma hipofisario
  • Pinealoma/pinealoblastoma
Linfoma primario del SNC Linfoma primario del SNC
Metástasis al AL Amyloidosis cerebro (5 veces más común que los LOS Neisseria tumores cerebrales primarios) Más comúnmente surgen de:
  • Carcinomas de pulmón, mama y células renales
  • Melanoma Melanoma Melanoma is a malignant tumor arising from melanocytes, the melanin-producing cells of the epidermis. These tumors are most common in fair-skinned individuals with a history of excessive sun exposure and sunburns. Melanoma
Tumores periféricos
  • Schwannomas, incluidos los LOS Neisseria neuromas acústicos
  • Neuroblastoma Neuroblastoma Neuroblastoma is a malignancy that arises from the neural crest cell derivatives along the sympathetic chain (neuroblasts) and is most commonly located in the adrenal medulla. The tumor often presents in childhood with a flank mass that crosses the midline. Neuroblastoma

Epidemiología

  • Esporádico: 75%
  • Asociado a von Hippel-Lindau: 25%
  • Incidencia:
    • Extremadamente raro
    • 2,5% de todas las neoplasias intracraneales
    • 5% de los LOS Neisseria tumores de la médula espinal
  • Sexo: La incidencia es mayor en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum hombres que en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum mujeres (2:1).
  • Edad:
    • Los LOS Neisseria niños rara vez se ven afectados.
    • La edad máxima de incidencia es de 20–50 años.

Etiología

  • Se desconoce la causa exacta del hemangioblastoma Hemangioblastoma Hemangioblastomas are vascular neoplasms of the CNS. Hemangioblastomas are rare and are often associated with von Hippel-Lindau disease (VHL). The most common presentation is a headache and, depending on the size and location of the tumor, patients may present with sensory deficits and motor weakness. Hemangioblastoma.
  • Se está considerando una mutación en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum el gen VHL debido a una alta asociación con von Hippel-Lindau.

Fisiopatología

Localización del tumor Tumor Inflammation

Los LOS Neisseria hemangioblastomas Hemangioblastomas A benign tumor of the nervous system that may occur sporadically or in association with von Hippel-Lindau disease. It accounts for approximately 2% of intracranial tumors, arising most frequently in the cerebellar hemispheres and vermis. Histologically, the tumors are composed of multiple capillary and sinusoidal channels lined with endothelial cells and clusters of lipid-laden pseudoxanthoma cells. Usually solitary, these tumors can be multiple and may also occur in the brain stem, spinal cord, retina, and supratentorial compartment. Cerebellar hemangioblastomas usually present in the third decade with intracranial hypertension, and ataxia. Von Hippel-Lindau Disease crecen adheridos a la piamadre (capa meníngea más interna)

  • Parénquima del cerebelo
  • Tronco encefálico
  • Médula espinal

Patogénesis

Aunque los LOS Neisseria hemangiomas esporádicos no tienen una patogénesis clara, se cree que los LOS Neisseria hemangiomas asociados con von Hippel-Lindau son causados por una mutación en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum el gen VHL. Hasta el 50% de los LOS Neisseria hemangioblastomas Hemangioblastomas A benign tumor of the nervous system that may occur sporadically or in association with von Hippel-Lindau disease. It accounts for approximately 2% of intracranial tumors, arising most frequently in the cerebellar hemispheres and vermis. Histologically, the tumors are composed of multiple capillary and sinusoidal channels lined with endothelial cells and clusters of lipid-laden pseudoxanthoma cells. Usually solitary, these tumors can be multiple and may also occur in the brain stem, spinal cord, retina, and supratentorial compartment. Cerebellar hemangioblastomas usually present in the third decade with intracranial hypertension, and ataxia. Von Hippel-Lindau Disease esporádicos también tienen mutaciones o deleciones en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum el gen VHL.

  • Gen VHL:
    • Gen supresor de tumores encontrado en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum el cromosoma 3p
    • Responsable de la inhibición del factor 2α inducible por hipoxia (HIF-2α) por degradación proteasomal mediada por ubiquitina
  • HIF-2α:
    • Parte de un complejo de proteína de factor de transcripción más grande llamado HIF HIF Hypoxia-inducible factor 1, alpha subunit is a basic helix-loop-helix transcription factor that is regulated by oxygen availability and is targeted for degradation by VHL tumor suppressor protein. Von Hippel-Lindau Disease, que participa en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum la regulación de la capacidad del cuerpo para adaptarse a los LOS Neisseria cambios en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum los LOS Neisseria niveles de oxígeno.
    • HIF HIF Hypoxia-inducible factor 1, alpha subunit is a basic helix-loop-helix transcription factor that is regulated by oxygen availability and is targeted for degradation by VHL tumor suppressor protein. Von Hippel-Lindau Disease puede inducir la expresión de más de 70 genes Genes A category of nucleic acid sequences that function as units of heredity and which code for the basic instructions for the development, reproduction, and maintenance of organisms. DNA Types and Structure diana, que incluyen:
      • Factor de crecimiento endotelial vascular (VEGF, por sus siglas en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum inglés)
      • Factor de crecimiento derivado de plaquetas (PDGF, por sus siglas en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum inglés)
      • Eritropoyetina
      • Factor de crecimiento transformante α (TGF-α)
  • Cuando hay suficiente oxígeno disponible, la proteína VHL ayuda a suprimir el HIF HIF Hypoxia-inducible factor 1, alpha subunit is a basic helix-loop-helix transcription factor that is regulated by oxygen availability and is targeted for degradation by VHL tumor suppressor protein. Von Hippel-Lindau Disease.
  • Un gen VHL disfuncional (causado por mutación o deleción) puede conducir a la acumulación de HIF-α debido a la incapacidad de degradar HIF-2α.
  • La estabilización de HIF-2α le permite inducir la expresión de sus genes Genes A category of nucleic acid sequences that function as units of heredity and which code for the basic instructions for the development, reproduction, and maintenance of organisms. DNA Types and Structure diana → ↑ factores angiogénicos → crecimiento tumoral

Desarrollo de síntomas

El desarrollo de los LOS Neisseria síntomas es causado por:

  • Compresión directa del tumor Tumor Inflammation sobre las estructuras neurales
  • Sangrado peritumoral
  • Complicaciones paraneoplásicas

Presentación Clínica y Complicaciones

Presentación Clínica

Los LOS Neisseria síntomas dependen de la ubicación y la progresión del tumor Tumor Inflammation. Los LOS Neisseria síntomas más comunes que se observan son:

  • Cefalea: más común
  • Dolor Dolor Inflammation local (común en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum lesiones de la médula espinal)
  • Ataxia Ataxia Impairment of the ability to perform smoothly coordinated voluntary movements. This condition may affect the limbs, trunk, eyes, pharynx, larynx, and other structures. Ataxia may result from impaired sensory or motor function. Sensory ataxia may result from posterior column injury or peripheral nerve diseases. Motor ataxia may be associated with cerebellar diseases; cerebral cortex diseases; thalamic diseases; basal ganglia diseases; injury to the red nucleus; and other conditions. Ataxia-telangiectasia cerebelosa y descoordinación
  • Aumento de la presión intracraneal (PIC) debido a hidrocefalia obstructiva
  • Disfunción del nervio oculomotor
  • Debilidad motora
  • Déficits sensoriales

Hemangioblastomas Hemangioblastomas A benign tumor of the nervous system that may occur sporadically or in association with von Hippel-Lindau disease. It accounts for approximately 2% of intracranial tumors, arising most frequently in the cerebellar hemispheres and vermis. Histologically, the tumors are composed of multiple capillary and sinusoidal channels lined with endothelial cells and clusters of lipid-laden pseudoxanthoma cells. Usually solitary, these tumors can be multiple and may also occur in the brain stem, spinal cord, retina, and supratentorial compartment. Cerebellar hemangioblastomas usually present in the third decade with intracranial hypertension, and ataxia. Von Hippel-Lindau Disease esporádicos versus asociados a von Hippel-Lindau

Tabla: Presentaciones clínicas típicas de hemangioblastomas Hemangioblastomas A benign tumor of the nervous system that may occur sporadically or in association with von Hippel-Lindau disease. It accounts for approximately 2% of intracranial tumors, arising most frequently in the cerebellar hemispheres and vermis. Histologically, the tumors are composed of multiple capillary and sinusoidal channels lined with endothelial cells and clusters of lipid-laden pseudoxanthoma cells. Usually solitary, these tumors can be multiple and may also occur in the brain stem, spinal cord, retina, and supratentorial compartment. Cerebellar hemangioblastomas usually present in the third decade with intracranial hypertension, and ataxia. Von Hippel-Lindau Disease esporádicos versus asociados a Von Hippel-Lindau
Hemangioblastoma Hemangioblastoma Hemangioblastomas are vascular neoplasms of the CNS. Hemangioblastomas are rare and are often associated with von Hippel-Lindau disease (VHL). The most common presentation is a headache and, depending on the size and location of the tumor, patients may present with sensory deficits and motor weakness. Hemangioblastoma esporádico Hemangioblastoma Hemangioblastoma Hemangioblastomas are vascular neoplasms of the CNS. Hemangioblastomas are rare and are often associated with von Hippel-Lindau disease (VHL). The most common presentation is a headache and, depending on the size and location of the tumor, patients may present with sensory deficits and motor weakness. Hemangioblastoma asociado a von Hippel-Lindau
Se presenta como tumor Tumor Inflammation solitario Múltiples tumores a lo largo del neuroeje
Generalmente ocurre en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum la 3ra o 4ta década de la vida Diagnosticado a una edad más temprana (2da década)
Comúnmente se presenta como un tumor Tumor Inflammation aislado en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum el cerebelo El 50% de los LOS Neisseria tumores se localizan en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum la médula espinal, el 40% en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum el cerebelo y el 10% en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum el tronco encefálico.

Complicaciones

Las complicaciones suelen ocurrir por aumento del tamaño del tumor Tumor Inflammation (> 1,5 cm), provocando compresión, o por hemorragia espontánea:

  • Síntomas de aumento de la PIC/hidrocefalia obstructiva rápida
  • Estado mental alterado
  • Policitemia por producción ectópica de eritropoyetina (síndrome paraneoplásico)
  • Cuadriplejia

Diagnóstico

Si bien la principal herramienta diagnóstica es la imagenología, el estándar de oro del diagnóstico requiere la histopatología de una muestra de biopsia.

Imagenología

Se deben obtener imágenes de todo el eje neural para descartar lesiones múltiples, que son comunes en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum los LOS Neisseria casos de von Hippel-Lindau.

  • RM de contraste:
    • La RM realzada con gadolinio es el método de imagenología más sensible para diagnosticar el hemangioblastoma.
    • Rasgos característicos:
      • Nódulo realzado asociado a un quiste (60% de los tumores)
      • Tumor sólido realzado (40%)
    • T1 muestra un nódulo hipointenso a isointenso
    • T2 muestra un nódulo hiperintenso
    • Cualquier quiste presente tiene la misma densidad que el LCR.
  • TC:
    • No es el método preferido de diagnóstico, ya que los tumores pequeños pueden quedar ocultos por los artefactos óseos
    • Una TC sin contraste muestra un nódulo isointenso.
    • Con contraste: intenso realce homogéneo de los nódulos
    • La TC también se utiliza como adyuvante de la angiografía cuando el paciente no puede someterse a una RM.
  • Angiografía cerebral y espinal:
    • Se observa un elevado “blush” tumoral vascular con arterias agrandadas y venas dilatadas.
    • Puede detectar el suministro de sangre al AL Amyloidosis tumor Tumor Inflammation → ayuda al AL Amyloidosis cirujano en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum la resección adecuada del tumor Tumor Inflammation
hemangioblastoma

Imágenes radiográficas de hemangioblastomas:
A: RM axial potenciada en T1 con contraste que muestra hemangioblastoma cerebeloso con nódulo mural que realza con contraste y quiste peritumoral
B: RM sagital potenciada en T1 con contraste que revela hemangioblastoma medular con contraste y edema vasogénico circundante
C: RM sagital, realzada con contraste, potenciada en T1 con hemangioblastoma posterior/dorsal realzado con contraste con siringe asociada

Imagen: “Hemangioblastoma” por Department of Neurological Surgery, Ohio State University Wexner Medical Center , Columbus, OH , USA. Licencia: CC BY 4.0

Examen oftalmológico

  • Los LOS Neisseria hemangioblastomas Hemangioblastomas A benign tumor of the nervous system that may occur sporadically or in association with von Hippel-Lindau disease. It accounts for approximately 2% of intracranial tumors, arising most frequently in the cerebellar hemispheres and vermis. Histologically, the tumors are composed of multiple capillary and sinusoidal channels lined with endothelial cells and clusters of lipid-laden pseudoxanthoma cells. Usually solitary, these tumors can be multiple and may also occur in the brain stem, spinal cord, retina, and supratentorial compartment. Cerebellar hemangioblastomas usually present in the third decade with intracranial hypertension, and ataxia. Von Hippel-Lindau Disease tienen una alta asociación con von Hippel-Lindau, que se asocia con hemangioblastomas Hemangioblastomas A benign tumor of the nervous system that may occur sporadically or in association with von Hippel-Lindau disease. It accounts for approximately 2% of intracranial tumors, arising most frequently in the cerebellar hemispheres and vermis. Histologically, the tumors are composed of multiple capillary and sinusoidal channels lined with endothelial cells and clusters of lipid-laden pseudoxanthoma cells. Usually solitary, these tumors can be multiple and may also occur in the brain stem, spinal cord, retina, and supratentorial compartment. Cerebellar hemangioblastomas usually present in the third decade with intracranial hypertension, and ataxia. Von Hippel-Lindau Disease retinianos.
  • Se puede realizar una angiografía retiniana para descartar la presencia de un hemangioblastoma Hemangioblastoma Hemangioblastomas are vascular neoplasms of the CNS. Hemangioblastomas are rare and are often associated with von Hippel-Lindau disease (VHL). The most common presentation is a headache and, depending on the size and location of the tumor, patients may present with sensory deficits and motor weakness. Hemangioblastoma retiniano.
Enfermedad de von Hippel-Lindau con maculopatía exudativa

Angiografía con fluoresceína del ojo derecho que muestra un hemangioblastoma retiniano superior temporal con la arteria alimentadora y la vena de drenaje

Imagen: “Fluorescein angiogram of right eye” por Vitreoretinal Division, Department of Ophthalmology, Al-Hussein Hospital, King Hussein Medical Center, Amman, Jordan. Licencia: CC BY 2.0

Histopatología

  • Estándar de oro para el diagnóstico
  • Examen macroscópico: nódulos rojos muy vascularizados bien delimitados
  • Examinación microscópica:
    • Amplias redes vasculares con capilares estructuralmente normales
    • Células estromales neoplásicas:
      • Núcleos hipercromáticos, pleomórficos
      • Tasa mitótica baja
      • Sin atipia
      • Fibras de Rosenthal
  • Pueden aparecer 2 componentes celulares distintos en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum el mismo tumor Tumor Inflammation:
    • Tipo 1: pequeñas células endoteliales perivasculares con núcleos hipercromáticos y citoplasma escaso
    • Tipo 2: citoplasma vacuolado y rico en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum lípidos
Hemangioblastoma del nervio óptico

Microfotografía H&E de un hemangioblastoma del nervio óptico (ubicación inusual) que revela un tumor marcadamente vascular con células estromales lipidizadas (×200)

Imagen: “Optic nerve
hemangioblastoma” por Department of Surgery, Division of Neurosurgery
, University of Alabama at Birmingham, Birmingham, AL
35294, USA. Licencia: CC BY 3.0

Tratamiento y Pronóstico

La resección quirúrgica es el enfoque principal para tratar los LOS Neisseria hemangioblastomas Hemangioblastomas A benign tumor of the nervous system that may occur sporadically or in association with von Hippel-Lindau disease. It accounts for approximately 2% of intracranial tumors, arising most frequently in the cerebellar hemispheres and vermis. Histologically, the tumors are composed of multiple capillary and sinusoidal channels lined with endothelial cells and clusters of lipid-laden pseudoxanthoma cells. Usually solitary, these tumors can be multiple and may also occur in the brain stem, spinal cord, retina, and supratentorial compartment. Cerebellar hemangioblastomas usually present in the third decade with intracranial hypertension, and ataxia. Von Hippel-Lindau Disease. Las terapias adyuvantes comúnmente se requieren en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum los LOS Neisseria hemangioblastomas Hemangioblastomas A benign tumor of the nervous system that may occur sporadically or in association with von Hippel-Lindau disease. It accounts for approximately 2% of intracranial tumors, arising most frequently in the cerebellar hemispheres and vermis. Histologically, the tumors are composed of multiple capillary and sinusoidal channels lined with endothelial cells and clusters of lipid-laden pseudoxanthoma cells. Usually solitary, these tumors can be multiple and may also occur in the brain stem, spinal cord, retina, and supratentorial compartment. Cerebellar hemangioblastomas usually present in the third decade with intracranial hypertension, and ataxia. Von Hippel-Lindau Disease asociados con von Hippel-Lindau, y las opciones incluyen radioterapia, embolización endovascular y terapia antiangiogénica.

Cirugía

La cirugía es el enfoque definitivo primario para tratar los LOS Neisseria hemangioblastomas Hemangioblastomas A benign tumor of the nervous system that may occur sporadically or in association with von Hippel-Lindau disease. It accounts for approximately 2% of intracranial tumors, arising most frequently in the cerebellar hemispheres and vermis. Histologically, the tumors are composed of multiple capillary and sinusoidal channels lined with endothelial cells and clusters of lipid-laden pseudoxanthoma cells. Usually solitary, these tumors can be multiple and may also occur in the brain stem, spinal cord, retina, and supratentorial compartment. Cerebellar hemangioblastomas usually present in the third decade with intracranial hypertension, and ataxia. Von Hippel-Lindau Disease, ya que son tumores benignos no invasivos.

  • Los LOS Neisseria tumores suelen estar bien delimitados de las estructuras circundantes, pero son muy vascularizados y se localizan en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum áreas neurológicamente sensibles.
  • La angiografía preoperatoria es útil para identificar las arterias de alimentación.
  • Necesidad de terapia adyuvante:
    • Generalmente, no se requiere en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum tumores únicos esporádicos
    • Típicamente requerida en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum tumores múltiples y/o asociados a von Hippel-Lindau

Otras modalidades terapéuticas

  • Radioterapia:
    • La radioterapia postoperatoria reduce la recurrencia de los LOS Neisseria hemangioblastomas Hemangioblastomas A benign tumor of the nervous system that may occur sporadically or in association with von Hippel-Lindau disease. It accounts for approximately 2% of intracranial tumors, arising most frequently in the cerebellar hemispheres and vermis. Histologically, the tumors are composed of multiple capillary and sinusoidal channels lined with endothelial cells and clusters of lipid-laden pseudoxanthoma cells. Usually solitary, these tumors can be multiple and may also occur in the brain stem, spinal cord, retina, and supratentorial compartment. Cerebellar hemangioblastomas usually present in the third decade with intracranial hypertension, and ataxia. Von Hippel-Lindau Disease.
    • Indicaciones:
      • Lesiones inaccesibles quirúrgicamente
      • Múltiples lesiones
  • Embolización endovascular:
    • Objetivo: disminuir la vascularización del tumor Tumor Inflammation y reducir las complicaciones intraoperatorias de la hemorragia
    • Los LOS Neisseria tumores grandes se pueden reducir considerablemente para facilitar la resección.
  • Inhibidores angiogénicos: bevacizumab Bevacizumab An anti-vegf humanized murine monoclonal antibody. It inhibits vegf receptors and helps to prevent pathologic angiogenesis. Targeted and Other Nontraditional Antineoplastic Therapy
    • Mecanismo de acción: un anticuerpo monoclonal que se une e inhibe los LOS Neisseria efectos del factor de crecimiento endotelial vascular → ↓ angiogénesis
    • Usos: pacientes con Von Hippel-Lindau con tumores que no responden a la cirugía o a la radiación, o que no son susceptibles de ellas
  • Inhibidor de HIF-2ɑ (belzutifan):
    • Para individuos con enfermedad de von Hippel-Lindau conocida
    • Se utiliza para retrasar la cirugía en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum aquellos con tumores de rápido crecimiento
    • También es beneficioso para individuos con tumores refractarios o recurrentes

Pronóstico

El pronóstico es bueno en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum la mayoría de los LOS Neisseria hemangioblastomas Hemangioblastomas A benign tumor of the nervous system that may occur sporadically or in association with von Hippel-Lindau disease. It accounts for approximately 2% of intracranial tumors, arising most frequently in the cerebellar hemispheres and vermis. Histologically, the tumors are composed of multiple capillary and sinusoidal channels lined with endothelial cells and clusters of lipid-laden pseudoxanthoma cells. Usually solitary, these tumors can be multiple and may also occur in the brain stem, spinal cord, retina, and supratentorial compartment. Cerebellar hemangioblastomas usually present in the third decade with intracranial hypertension, and ataxia. Von Hippel-Lindau Disease tratados:

  • La resección quirúrgica limpia (con márgenes quirúrgicos negativos), especialmente en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum tumores esporádicos solitarios, asegura un buen pronóstico.
  • La detección e intervención temprana es favorable.
  • Hemangioblastomas Hemangioblastomas A benign tumor of the nervous system that may occur sporadically or in association with von Hippel-Lindau disease. It accounts for approximately 2% of intracranial tumors, arising most frequently in the cerebellar hemispheres and vermis. Histologically, the tumors are composed of multiple capillary and sinusoidal channels lined with endothelial cells and clusters of lipid-laden pseudoxanthoma cells. Usually solitary, these tumors can be multiple and may also occur in the brain stem, spinal cord, retina, and supratentorial compartment. Cerebellar hemangioblastomas usually present in the third decade with intracranial hypertension, and ataxia. Von Hippel-Lindau Disease asociados con von Hippel-Lindau:
    • Mayor probabilidad de recurrencia que los LOS Neisseria tumores esporádicos
    • Peor pronóstico
    • Mayor asociación con déficits neurológicos

Diagnóstico Diferencial

  • Meningioma Meningioma Meningiomas are slow-growing tumors that arise from the meninges of the brain and spinal cord. The vast majority are benign. These tumors commonly occur in individuals with a history of high doses of skull radiation, head trauma, and neurofibromatosis 2. Meningioma: tumores que surgen de las meninges Meninges The brain and the spinal cord are enveloped by 3 overlapping layers of connective tissue called the meninges. The layers are, from the most external layer to the most internal layer, the dura mater, arachnoid mater, and pia mater. Between these layers are 3 potential spaces called the epidural, subdural, and subarachnoid spaces. Meninges: Anatomy del cerebro y la médula espinal. Los LOS Neisseria meningiomas suelen ser asintomáticos, pero pueden presentarse con cefalea, convulsiones y alteraciones visuales. El diagnóstico se realiza mediante RM y biopsia. Los LOS Neisseria casos asintomáticos generalmente se mantienen bajo observación, mientras que los LOS Neisseria pacientes sintomáticos se tratan quirúrgicamente o con radiación. A diferencia de los LOS Neisseria hemangioblastomas Hemangioblastomas A benign tumor of the nervous system that may occur sporadically or in association with von Hippel-Lindau disease. It accounts for approximately 2% of intracranial tumors, arising most frequently in the cerebellar hemispheres and vermis. Histologically, the tumors are composed of multiple capillary and sinusoidal channels lined with endothelial cells and clusters of lipid-laden pseudoxanthoma cells. Usually solitary, these tumors can be multiple and may also occur in the brain stem, spinal cord, retina, and supratentorial compartment. Cerebellar hemangioblastomas usually present in the third decade with intracranial hypertension, and ataxia. Von Hippel-Lindau Disease, los LOS Neisseria meningiomas siempre están cerca de las meninges Meninges The brain and the spinal cord are enveloped by 3 overlapping layers of connective tissue called the meninges. The layers are, from the most external layer to the most internal layer, the dura mater, arachnoid mater, and pia mater. Between these layers are 3 potential spaces called the epidural, subdural, and subarachnoid spaces. Meninges: Anatomy y, a menudo, tienen datos de inserción de la duramadre (e.g., signo de la cola dural).
  • Glioblastoma multiforme Glioblastoma multiforme Glioblastoma multiforme is a high-grade astrocytoma, an aggressive brain tumor arising from astrocytes, with an unknown cause and a poorly understood link to risk factors. There are two main types: primary, a more aggressive form seen more commonly in older patients, and secondary, developing from lower-grade astrocytomas and seen more commonly in younger patients. Glioblastoma Multiforme: astrocitoma de grado IV según la World Health Organization (WHO) rápidamente progresivo que surge de los LOS Neisseria astrocitos (células gliales en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum el cerebro) y se presenta clínicamente como cefalea, náuseas, somnolencia, visión borrosa, cambios de personalidad y convulsiones. La imagenología, la presentación clínica y la biopsia son los LOS Neisseria pilares del diagnóstico. El tratamiento incluye radioterapia, quimioterapia y escisión quirúrgica. El pronóstico es malo, incluso con tratamiento. A diferencia del hemangioblastoma Hemangioblastoma Hemangioblastomas are vascular neoplasms of the CNS. Hemangioblastomas are rare and are often associated with von Hippel-Lindau disease (VHL). The most common presentation is a headache and, depending on the size and location of the tumor, patients may present with sensory deficits and motor weakness. Hemangioblastoma, el glioblastoma multiforme Glioblastoma multiforme Glioblastoma multiforme is a high-grade astrocytoma, an aggressive brain tumor arising from astrocytes, with an unknown cause and a poorly understood link to risk factors. There are two main types: primary, a more aggressive form seen more commonly in older patients, and secondary, developing from lower-grade astrocytomas and seen more commonly in younger patients. Glioblastoma Multiforme no se asocia con Von Hippel-Lindau.
  • Oligodendroglioma Oligodendroglioma Oligodendrogliomas are malignant CNS tumors arising from neural glial cell precursors. Oligodendrogliomas often arise in the frontal lobes of the brain and have a generally favorable prognosis when compared to other gliomas. Oligodendrogliomas are the 3rd most common CNS tumor. The most frequent presenting symptom is a seizure. Oligodendroglioma: tumor Tumor Inflammation del SNC que surge de los LOS Neisseria oligodendrocitos. El oligodendroglioma Oligodendroglioma Oligodendrogliomas are malignant CNS tumors arising from neural glial cell precursors. Oligodendrogliomas often arise in the frontal lobes of the brain and have a generally favorable prognosis when compared to other gliomas. Oligodendrogliomas are the 3rd most common CNS tumor. The most frequent presenting symptom is a seizure. Oligodendroglioma puede presentarse con déficits neurológicos focales, convulsiones y cambios de personalidad según la ubicación exacta. El diagnóstico se realiza mediante imagenología de RM y biopsia. Los LOS Neisseria oligodendrogliomas suelen desarrollarse en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum los LOS Neisseria hemisferios cerebrales, normalmente en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum el lóbulo frontal Frontal The bone that forms the frontal aspect of the skull. Its flat part forms the forehead, articulating inferiorly with the nasal bone and the cheek bone on each side of the face. Skull: Anatomy, y rara vez se encuentran en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum el área infratentorial o en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum la médula espinal. El tratamiento implica la resección quirúrgica posiblemente acompañada de radiación y/o quimioterapia.
  • Aneurisma cerebral: debilidad en EN Erythema nodosum is an immune-mediated panniculitis (inflammation of the subcutaneous fat) caused by a type IV (delayed-type) hypersensitivity reaction. It commonly manifests in young women as tender, erythematous nodules on the shins. Erythema Nodosum la pared de un vaso sanguíneo que irriga el cerebro que sobresale y corre el riesgo de romperse. Un aneurisma no roto suele ser asintomático y la ruptura de un aneurisma provoca celafea intensa y repentina. Los LOS Neisseria aneurismas cerebrales se diagnostican con TC, angiografía o ultrasonido. El tratamiento depende de la ubicación y generalmente se trata con un injerto de stent endovascular.

Referencias

  1. Wong, E.T., Joseph, J., Wu, J.K. Hemangioblastoma. UpToDate. Retrieved May 20, 2021, from https://www.uptodate.com/contents/hemangioblastoma
  2. Slavin, K.V. (2017). Hemangioblastoma. Medscape. Retrieved May 20, 2021, from https://emedicine.medscape.com/article/250670-overview
  3. Pernick, N. (2021). CNS tumors—other tumors—hemangioblastoma. Pathology Outlines. Retrieved May 20, 2021, from https://www.pathologyoutlines.com/topic/cnstumorhemangioblastoma.html
  4. Kim W.Y., Kaelin W.G. (2004). Role of VHL gene mutation in human cancer. J Clin Oncol. Retrieved June 1, 2021, from https://pubmed.ncbi.nlm.nih.gov/15611513/
  5. Dengler, V. L., Galbraith, M., Espinosa, J. M. (2014). Transcriptional regulation by hypoxia inducible factors. Critical reviews in biochemistry and molecular biology, 49(1):1–15. Retrieved June 1, 2021, from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4342852/

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