Hemangioblastoma cerebeloso y trombocitopenia: reporte de caso / Cerebellar hemangioblastoma and thrombocytopenia: report of one case

The association between vascular tumors and thrombocytopenia is rare. Kasabach-Merritt Syndrome is seen in childhood and is characterized by hemangiomas and thrombocytopenia. A 42 years-old man with a cerebellar hemangioblastoma and thrombocytopenia, admitted with a subarachnoid hemorrhage is reported. The patient was operated and required a splenectomy to manage the thrombocytopenia. After the splenectomy the patient developed a subdural hematoma that was operated. Despite the surgical treatment, the patient died.

Verteporfin photodynamic therapy of retinal capillary hemangioblastoma in von Hippel-Lindau disease.

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.

Malignant Glioma Arising at the Site of an Excised Cerebellar Hemangioblastoma after Irradiation in a von Hippel-Lindau Disease Patient

We describe herein a malignant glioma arising at the site of the resected hemangioblastoma after irradiation in a patient with von Hippel-Lindau disease (VHL). The patient was a 25 year-old male with multiple hemangioblastomas at the cerebellum and spinal cord, multiple pancreatic cysts and a renal cell carcinoma; he was diagnosed as having VHL disease. The largest hemangioblastoma at the right cerebellar hemisphere was completely removed, and he received high-dose irradiation postoperatively. The tumor recurred at the same site 7 years later, which was a malignant glioma with no evidence of hemangioblastoma. The malignant glioma showed molecular genetic profiles of radiation-induced tumors because of its diffuse p53 immunostaining and the loss of p16 immunoreactivity. The genetic study to find the loss of heterozygosity (LOH) of VHL gene revealed that only the cerebellar hemangioblastoma showed allelic losses for the gene. To the best of our knowledge, this report is the first to show a malignant glioma that developed in a patient with VHL disease after radiation therapy at the site of an excised hemangioblastoma. This report also suggests that radiation therapy should be performed very carefully in VHL patients with hemangioblastomas.

Hemangioblastomas aparentemente esporádicos e doença de von Hippel-Lindau não suspeita / Apparently sporadic hemangioblastomas and not suspected von Hippel-Lindau disease

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.

Intracranial hemangioblastomas: an institutional experience.

BACKGROUND AND AIMS: We present our Institutional experience with intracranial hemangioblastomas. SETTINGS AND DESIGN: A retrospective study. MATERIALS AND METHODS: This study included all patients of intracranial hemangioblastomas admitted in our institution over a period of 11 years from January 1992 through June 2003. RESULTS: There were a total of 69 patients (45 males and 24 females). The average age at presentation was 34.5 years. The tumor was located in the cerebellar hemispheres, vermian and brainstem regions in 42 (60%) patients, 19 (28%) patients and 8 (12%) patients, respectively. Hydrocephalus was seen in 48 (69%) patients. Thirty-three patients underwent CSF diversion procedures prior to surgery on the tumor. All except one underwent definitive surgery. The mortality was 8 (11%). Sixty eight patients underwent surgery on the tumor. The follow-up ranged from 1 month to 11 years. Fifteen patients developed recurrent lesions. CONCLUSION: Lifelong surveillance is necessary in cases with hemangioblastomas to identify recurrences especially in those patients having VHL syndrome.

El mareo como síntoma de los tumores encefálicos. Estudio de 60 casos / Dizziness as a symptom of encephalic tumors. A review of 60 cases

Se procedió a estudiar la incidencia de manifestaciones de mareos en una muestra de 60 casos de pacientes de ambos sexos, con edades entre 6 y 76 años, con tumores encefálicos de diversa localización e histopatología. La ubicación de los tumores se estableció por métodos radiológicos y se confirmaron tras la intervención quirúrgica. Se estudió especialmente la relación del síntoma mareo con la ubicación de los tumores encefálicos. Comprobamos que el mareo aparece asociado especialmente a los tumores de fosa posterior. En los casos de tumores localizados en hemisferios cerebrales el mareo se manifiesta con menor incidencia. Estos casos se ubican en áreas próximas a los centros vestibulares corticales