ABSTRACT
Los tumores del tronco cerebral son infrecuentes en la población adulta. Las controversias surgen cuando se considera la necesidad de confirmar el diagnóstico histopatológico en esta área elocuente del cerebro, balanceando los beneficios de obtener un diagnóstico certero y las desventajas de los procedimientos invasivos. Existen escasas publicaciones acerca de su tratamiento quirúrgico en adultos, todas series pequeñas analizadas retrospectivamente. Presentamos nuestra experiencia con el propósito de contribuir al proceso de toma de decisiones. Diez de 13 pacientes fueron intervenidos. Las lesiones se clasificaron en focales (n:7), infiltrativa difusa (n:1), tectal (1) y exofítica (1). El estado neurológico según la escala Karnofsky Performance Status fue ≥ 70 en 6 casos y < 70 en 7. Las muestras fueron obtenidas mediante abordaje microquirúrgico directo o por biopsia estereotáctica. Los hallazgos histopatológicos fueron confirmados en todos los casos: astrocitoma pilocítico (n:1), glioma de bajo grado (n:1), glioblastoma (n:1), hemangioblastoma celular (n:1), subependimoma (n:1), disgerminoma (n:1), y lesiones pseudotumorales (n:4, 3 cavernomas, 1 pseudotumor inflamatorio). La amplia variedad de hallazgos patológicos en esta localización en adultos exige una precisa definición histopatológica, que no solo determina la terapéutica adecuada sino que también previene las consecuencias potencialmente catastróficoas de los tratamientos empíricos.
Brainstem tumors are uncommon beyond childhood. Controversies arise regarding the need of histological diagnosis in this eloquent area of the brain, weighting the benefits of a reliable diagnosis against the disadvantages of invasive procedures. There are scant publications about the surgical management of brainstem tumors in adults, all of them involving small retrospective cohorts. We are reporting our experience with the aim of contributing to the decision making process. Out of a series of 13 patients, 10 were approached surgically. According to Guillamo´s classification the lesions were: focal (n:7), diffuse infiltrative (n:1), tectal (n:1), and exophytic (n:1). According to the Karnofsky Performance Status scale, the neurological status was ≥ 70 in 6 cases and < 70 in 7. Histopathology was confirmed in all 10 treated cases and the samples were obtained by a direct microsurgical approach or by stereotactic biopsy. Histopathological findings were: pilocytic astrocytoma (n:1), low grade glioma (n:1), glioblastoma (n:1), cellular haemangioblastoma (n:1), subependimoma (n:1), pseudotumoral lesions (n:4; 3 cavernomas, 1 inflammatory pseudotumor), and disgerminoma (n:1). As a broad variety of pathologies could be found in this brain localization, an accurate histopathological definition can not only determine the adequate therapy, but also avoid the disastrous consequences of empiric treatments.
Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aged , Brain Stem Neoplasms/surgery , Brain Stem Neoplasms/pathology , Biopsy , Magnetic Resonance Imaging , Cerebral Angiography/methods , Retrospective Studies , Karnofsky Performance Status , Hemangioblastoma/diagnosis , Hemangioma, Cavernous, Central Nervous System/surgery , Hemangioma, Cavernous, Central Nervous System/diagnosis , Brain Stem Neoplasms/diagnosis , Glioma/diagnosis , Granuloma, Plasma Cell/diagnosisABSTRACT
El hemangioblastoma es un tumor benigno del sistema nervioso central que usualmente se originan en la fosa posterior, especialmente en el cerebelo. Existen alrededor de 114 casos reportados de ubicación supratentorial y no encontramos ningún caso de tipo extraaxial. Nosotros presentamos un caso de hemangioblastoma cerebral izquierdo, en relación con la fisura de Silvio, sólido, no relacionado con la enfermedad de Von Hippel-Lindau, revisamos la literatura relacionada con dicha entidad, y discutimos las características imagenológicas, macroscópicas e histológicas así como las dificultades en su diagnóstico.
Subject(s)
Humans , Female , Adult , Brain Neoplasms , Cerebral Aqueduct , Craniotomy , von Hippel-Lindau Disease/complications , Hemangioblastoma/surgery , Hemangioblastoma/diagnosis , Hemangioblastoma/etiology , Central Nervous System/pathology , Tomography, X-Ray Computed , Vascular NeoplasmsABSTRACT
An 18-year-old boy presented to us with bilateral retinal hemangioblastoma and von Hippel-Lindau disease with history of cerebral capillary hemangioblastoma and embryonic cell carcinoma of left testes. The vision in the right eye was already lost with development of secondary closed angle glaucoma, optic atrophy with subsequent development of bullous keratopathy. The multiple retinal angiomatous lesions in the seeing left eye were treated with various modalities like triple freeze thaw cryopexy, focal lasers and transpupillary thermo therapy in multiple sittings over a period of almost 20 years since detection. One particular angiomatous lesion in the left eye was showing resistance to all the above mentioned modalities and was finally successfully treated with verteporfin and photodynamic therapy to achieve complete regression without any post-treatment complication and with a sustained 20/20 vision till a follow-up of 15 months.
Subject(s)
Adolescent , Diagnosis, Differential , Follow-Up Studies , Hemangioblastoma/complications , Hemangioblastoma/diagnosis , Hemangioblastoma/drug therapy , Humans , Male , Photochemotherapy/methods , Photosensitizing Agents/therapeutic use , Porphyrins/therapeutic use , Retinal Neoplasms/complications , Retinal Neoplasms/diagnosis , Retinal Neoplasms/drug therapy , Time Factors , von Hippel-Lindau Disease/complications , von Hippel-Lindau Disease/diagnosisABSTRACT
We report a very rare case of hemangioblastomatosis that developed after surgical removal of a solitary cerebellar hemangioblastoma (HB). A 51-yr-old man presented with back pain 10 yr after undergoing surgery for cerebellar HB. Magnetic resonance imaging showed numerous mass lesions along the entire neuraxis accompanied by prominent leptomeningeal enhancement. Genomic DNA analysis showed no mutation in the von Hippel-Lindau (VHL) genes. A surgical specimen obtained from a lesion in the cauda equina showed pathological findings identical to those of the cerebellar HB that had been resected 10 yr earlier. External beam radiation therapy and radiosurgery were subsequently performed; however, the patient succumbed one year after receiving the diagnosis of hemangioblastomatosis. The reduction of tumor cell spillage during surgery and regular long-term follow-up are recommended for patients with HBs.
Subject(s)
Humans , Male , Middle Aged , Central Nervous System Neoplasms/diagnosis , Cerebellar Neoplasms/pathology , Hemangioblastoma/diagnosis , Magnetic Resonance Imaging , Recurrence , von Hippel-Lindau Disease/geneticsABSTRACT
Objetivo: Apresentar os achados associados à doença de von Hippel-Lindau em pacientes portadores de hemangioblastoma do sistema nervoso central, aparentemente esporádico, submetidos a rastreamento (screening)em um centro neurocirúrgico de referência.Métodos:Na base de dados do Instituto Nacional de Neurologia e Neurocirurgia da Cidade do México foram identificados todos os pacientes com diagnóstico histológico confirmado de hemangioblastomado sistema nervoso central entre janeiro de 1980 e agosto de 2000.Os arquivos médicos, radiológicos e patológicos desses pacientes foram revisados retrospectivamente. Destes, 25 pacientes assintomáticos concordaram em submeter-se ao rastreamento, por meio de exame físico, ultra-sonografia e tomografia axial computadorizada de abdômen,ressonância magnética cranioespinhal e exame oftalmológico.Resultados: 32 por cento dos pacientes apresentaram policistose, 16 por cento, hemangioblastomas múltiplos e 12 por cento, carcinomas viscerais. Segundo os critérios de Neumann, logrou-se estabelecer o diagnóstico clínico da doença de von Hippel-Lindau em 60 por cento dos casos. Conclusão:A presença de lesões assintomáticas nos portadores da doença de von Hippel-Lindau assinala a falta de sensibilidade do exame clínico, fazendo-se necessário o rastreamento de todo paciente portador de hemangioblastomas do sistema nervoso central.
Subject(s)
Adolescent , Adult , Middle Aged , von Hippel-Lindau Disease/complications , von Hippel-Lindau Disease/diagnosis , von Hippel-Lindau Disease/therapy , Hemangioblastoma/complications , Hemangioblastoma/diagnosis , Hemangioblastoma/therapy , Wounds and InjuriesABSTRACT
Angiomatous meningioma accounts for 2.1% of all meningiomas. It has features of a typical benign meningioma with many small or large vascular channels which may predominate over its meningothelial elements. We present here a series of three cases of angiomatous meningioma, which posed diagnostic difficulty to clinicians, radiologists, and pathologists. All the three cases showed a tumor entirely composed of thin-walled vascular channels and cells with bland morphology in the background. The diagnosis was confirmed by immunohistochemistry. We present series of three cases to highlight the histomorphological features of this uncommon variant of meningioma that could help in distinguishing it from hemangioblastoma and hemangiopericytoma.
Subject(s)
Adult , Diagnosis, Differential , Female , Hemangioblastoma/diagnosis , Hemangiopericytoma/diagnosis , Humans , Immunohistochemistry , Meningeal Neoplasms/pathology , Meningioma/diagnosis , Middle AgedABSTRACT
A rare case of hemangioblastoma located in the region of hippocampus is reported. A 27-year-old female presented with a single episode of generalized convulsion. The vascular and cherry red color hemangioblastoma was resected by a temporo-zygomatic approach. There has been no recurrence of tumor at a follow-up of 11 years.
Subject(s)
Adult , Female , Functional Laterality , Hemangioblastoma/diagnosis , Hippocampus , Humans , Seizures/etiologyABSTRACT
Haemangioblastoma is a rare benign vascular tumor commonly seen in the cerebellum. There is a striking histologic similarity between cellular variant of haemangioblastoma and metastatic renal cell carcinoma. We present a case of haemangioblastoma in a 32-year-old male. Histology revealed a highly vascular tumor composed of clear cells in a lobular arrangement.
Subject(s)
Adult , Carcinoma, Renal Cell/diagnosis , Cerebellar Neoplasms/diagnosis , Cerebellum/pathology , Diagnosis, Differential , Hemangioblastoma/diagnosis , Humans , MaleABSTRACT
La Enfermedad de Von Hippel-Lindau es un síndrome hereditario, autosómico dominante asociado a la mutación de un gen supresor tumoral localizado en cromosoma 3p25-26 que tiene riesgo genético esperado de desarrollar hemangioblastoma múltiple en cerebro, médula y retina, feocromocitoma, carcinoma renal de células claras, tumor del saco endolinfático, quistes renales, pancreáticos, hepáticos, de ligamentos anchos y epidídimo. Se presenta un caso clínico con hemangioblastoma de médula espinal cuyo diagnóstico se hizo en el curso de su primer embarazo. Fue operada del tumor después del parto y desarrolló otro embarazo posteriormente. Se discuten aspectos generales del hemangioblastoma del sistema nervioso central y de otras localizaciones y su relación con el embarazo y el parto. Se comunica la nueva clasificación propuesta del síndrome y los principios del manejo actual.
Subject(s)
Humans , Female , Pregnancy , Pregnancy Complications, Neoplastic/surgery , Pregnancy Complications, Neoplastic/diagnosis , Hemangioblastoma/congenital , Hemangioblastoma/diagnosis , Spinal Cord Neoplasms , von Hippel-Lindau Disease , Clinical Evolution , Pregnancy Outcome , Syndrome , von Hippel-Lindau DiseaseABSTRACT
El hemangioblastoma cerebeloso es un tumor raro que tiene una incidencia de 1 a 2 por ciento entre los tumores cerebelosos. Se presenta generalmente en hombres jóvenes, de edad media entre 30 y 40 años. Es un tumor formado por numerosos vasos sanguíneos de tipo capilar con células endoteliales, pericitos y células estromales claras vacuoladas. En el Hospital Juárez de México se estudiaron cuatro casos de hemangioblastomas cerebelosos, de los cuales dos eran hombres y dos mujeres todos ellos presentaron síndrome de hipertención endocraneana y síndrome cerebeloso. El diagnóstico radiológico se dio en tres casos de cisticercosis y uno de hemangioblastoma. A todos ellos se les operó inicialmente realizándose cirugía descompresiva. El diagnóstico histológico de hemangioblastoma se realizó en el estudio definitivo. Dicho diagnóstico es de por sí difíil, por lo que requiere tinciones especiales; en tanto, el diagnóstico diferencial debe hacerse con carcinoma renal o astrocitoma pilocítico
Subject(s)
Humans , Male , Female , Adult , Middle Aged , Cerebellar Diseases/diagnosis , Cerebellar Diseases/pathology , Hemangioblastoma/diagnosis , Hemangioblastoma/pathology , Hemangioblastoma/surgery , Tomography, X-Ray ComputedABSTRACT
Presentamos un caso inusual de hemangioblastomas supre e infratentoriales de diferente histología. Se describen las características y los estudios neuroradiológicos que permitieron identificar esta necesidad de considerar esta patología como uno de los diagnósticos diferenciales en los procesos tumorales del Sistema Nervioso Central