Pineal region hemangioblastoma in a patient with Von Hippel-Lindau disease

Pineal region hemangioblastoma in a patient with Von Hippel-Lindau disease.

CASE The patient is a 37 year-old female who was diagnosed with Von Hippel-Lindau disease at the age of 28 years. At presentation the patient had an abdominal sonogram showing pancreatic cysts and a single liver hemangioma, normal ophthalmologic examination and normal CT scan of the brain. One year later, she had an episode of vertigo and severe headache and a new CT scan of the brain showed an...

Meningeal Supratentorial Hemangioblastoma in a Patient with Von Hippel-Lindau Disease Mimicking Angioblastic Menigioma

Hemangioblastomas are sporadic tumors found in the cerebellum or spinal cord. Supratentorial hemangioblastomas are rare, and those with meningeal involvement are extremely rare and have been reported in only approximately 130 patients. Here, we report the case of a 51-year-old female patient with supratentorial meningeal hemangioblastoma detected 5 years after surgical resection of an infratent...

Von Hippel-Lindau disease.

Von Hippel-Lindau Disease

A germline mutation in the Von-Hippel Lindau (VHL) gene predisposes carriers to development of abundantly vascularised tumours in the retina, cerebellum, spine, kidney, adrenal gland and pancreas. Most VHL patients die from the consequences of cerebellar haemangioblastoma or renal cell carcinoma. The VHL gene is a tumour suppressor gene and is involved in angiogenesis by regulation of the activ...

[Von Hippel-Lindau disease].

In recent years advances have been made in the clinical and genetic aspects of von Hippel-Lindau disease (VHL). Retinal capillary hemangioma is the most common manifestation of VHL disease and, therefore, ophthalmologists are frequently involved in the care of patients with this disease. The incidence of VHL disease is approximately 1 in 40,000 live births. It is estimated that there are approx...