| Hemangioblastomas are histologically benign tumors that occur exclusively within the neuraxis, most commonly in the posterior fossa. They are typically cystic tumors located in the cerebellum. Excision of the vascular mural nodules leads to cure. brain stem lesions are rarely reported. Surgical extirpation of a solid brain stem hemangioblastoma is relatively risky and requires precise microsurgical techniques. We present a woman with a hemangioblastoma embedded in the medulla oblongata. This 33-year-old woman presented with occipital headaches and sensory ataxia. Complete and detailed preoperative imaging studies were followed by successful microsurgical excision of the lesion. The patient recovered completely within 2 weeks after the operation except for mild paresthesia of the legs. Preoperative magnetic resonance imaging and cerebral angiography provided important information regarding the nature, location, and blood supply of this lesion, which facilitated its total removal. The importance of intraoperative identification and control of the feeding artery of the tumor is emphasized. ( info) |
2/173. hemangioblastoma mimicking tentorial meningioma: preoperative embolization of the meningeal arterial blood supply--case report. A 72-year-old male presented with a primary hemangioblastoma of the posterior fossa with unusual dural attachment and meningeal arterial blood supply from the external carotid artery and marginal tentorial artery. Preoperative embolization facilitated complete resection of the tumor with no resultant neurological deficit. hemangioblastoma must be included in the differential diagnosis of tumors with dural involvement. Preoperative embolization is very useful in such tumors. ( info) |
| A case of cerebellar hemangioblastoma with a coexistent arterial aneurysm on the feeding artery of the tumor is reported. The patient presented with an acute onset of headache, loss of consciousness, and left-sided hemiparesis due to a posterior fossa hemorrhage found adjacent to a hemangioblastoma. Four-vessel angiography revealed an aneurysm on the anterior inferior cerebellar artery (AICA), which was the main feeding vessel of the hemangioblastoma. Successful total excision of the hemangioblastoma and clipping of the AICA aneurysm achieved in a one-stage operation was demonstrated on postoperative angiography. ( info) |
4/173. Extradural spinal hemangioblastomas: report of two cases. Two cases of predominantly extraspinally extra- and intradural spinal cord hemangioblastomas in two patients each with and without von Hippel-Lindau-disease are reported. Preoperative MRI and angiographic findings are presented and the differential diagnosis is discussed. The surgical procedure is described and the literature of hemangioblastomas in this rare localization is reviewed. Improvements in both radiologic diagnosis and microsurgical techniques, and consequent screening programs will enhance life expectancy in patients afflicted with von hippel-lindau disease. ( info) |
5/173. Postpartum cerebellar herniation in von Hippel-Lindau syndrome. PURPOSE: To describe exacerbation during pregnancy of cerebellar hemangioblastoma in von Hippel-Lindau syndrome. METHOD: Case-report. A 21-year-old woman with von Hippel-Lindau syndrome was found on routine ocular examination to have severe papilledema 1 week after giving birth. RESULTS: Immediate magnetic resonance imaging disclosed a large cerebellar cyst from hemangioblastoma causing cerebellar tonsillar herniation. Immediate neurosurgical intervention was life saving. CONCLUSION: Worsening of intracranial hemangioblastoma during pregnancy in cases of von Hippel-Lindau syndrome should be realized and periodic neurologic and ophthalmologic observation is warranted. ( info) |
6/173. Multiple spinal "miliary" hemangioblastomas in von Hippel-Lindau (vHL) disease without cerebellar involvement. A case report and review of the literature. We report on a 57-year-old male presenting with radicular pain in the nerve roots of L5 and S1 on the right side and dysuria. magnetic resonance imaging (MRI) of the lumbar spine showed multiple (up to 20) small, intradural enhancing nodules attached to the cauda equina down to the sacrum, the largest 1 cm in diameter at the level Th12/L1 compressing the conus. Additionally, small nodules in the cervico-thoracal region adjacent to the cord, but no cerebellar or cerebral abnormalities, were detected in a consecutive MRI of the remaining neuroaxis. The histology of a resected lesion at Th12/L1 revealed hemangioblastoma of the reticular type. Together with a history of left eye enucleation performed 17 years ago for angiomatosis of the retina and the immunohistochemical detection of von Hippel-Lindau (vHL) protein within the removed spinal hemangioblastoma, a diagnosis of vHL disease was established. family history and screening for visceral manifestations of vHL disease were negative. In contrast to cerebellar or solitary spinal hemangioblastomas, multiple spinal hemangioblastomas without cerebellar involvement in vHL represent unusual manifestations. Unlike the case for solitary lesions in non-syndromic patients, a surgical cure does not seem feasible in this case. The role of treatment modalities is discussed. ( info) |
| A third ventricle tumor, in addition to a recurrent cerebellar hemangioblastoma, was found in a 47-year-old woman on follow-up magnetic resonance imaging (MRI) 5 years after operation of the cerebellar tumor. On MRI, the tumor was hypo- to isointense on T1-weighted images and hyperintense on T2-weighted images compared with the normal gray matter, and was strongly enhanced with gadolinium. The tumor was first treated with fractionated conventional external-beam radiation (5120 cGy in 16 fractions over a 4-week period), resulting in a slight decrease in size of the tumor. For a definite diagnosis and mass reduction, surgery was performed using an interhemispheric translamina terminalis approach, resulting in a partial removal of the tumor due to profuse bleeding. Histological diagnosis was hemangioblastoma. Hemangioblastomas of the third ventricle are extremely rare and have not been specifically discussed. We describe the detailed clinicopathological features of the present case together with the possible explanation for the development of this tumor in this rare location. ( info) |
| von hippel-lindau disease is an autosomal dominant disorder caused by a mutation of VHL gene. The incidence of the disease is one in 36,000 and its clinical manifestation is a familial occurrence of hemangioblastoma of the central nervous system and retina, renal cell cancer and pheochromocytoma. Cerebellar hemangioblastoma is the most frequent or sometimes the only abnormality observed in this syndrome. We present a family with von hippel-lindau disease in which four first degree relatives had a cerebellar hemangioblastoma. This neoplasm caused the death of two brothers aged 27 and 24 years old, respectively and their mother aged 62. The third son of this family was affected ten years ago, at the age of 30. The healthy family members are counselled in Oncological Genetic Outpatient Unit in Gdansk. ( info) |
| von Hippel-Lindau (VHL) disease is an autosomal dominant familial cancer syndrome predisposing to the development of retinal and central nervous system haemangioblastomas, pheochromocytomas, renal and pancreatic cancer. In the course of a molecular analysis conducted to detect germline mutations of this gene in von Hippel-Lindau patients and individuals affected by sporadic tumors, we have identified a case of somatic mosaicism in the asymptomatic mother of a VHL patient who was subsequently diagnosed with pheochromocytoma. This is the first report providing molecular evidence of somatic mosaicism in von hippel-lindau disease. mosaicism could provide some genetic explanation for the clinical heterogeneity and variable severity of the VHL phenotype, and should be considered, as a possible event when evaluating sporadic cases of VHL or patients with isolated VHL-related tumors. Hum Mutat 15:114, 2000. ( info) |
10/173. Metastasis of renal carcinoma to a cerebellar hemangioblastoma in a patient with von Hippel Lindau disease: a case report. We report a case of metastatic renal carcinoma in a multirecurrent cerebellar haemangioblastoma (HBL) in an adult patient with von Hippel Lindau (VHL) disease. To our knowledge, only two cases of metastases to intracranial HBLs have been reported. ( info) |