Natural history of supratentorial hemangioblastomas in von Hippel-Lindau disease.
ABSTRACT Supratentorial hemangioblastomas are rare lesions, occurring either sporadically or in von Hippel-Lindau disease.
Following recent advances in our understanding of the natural history of von Hippel-Lindau-associated cerebellar and spinal hemangioblastomas, we conducted a study of the natural history of supratentorial hemangioblastomas in von Hippel-Lindau disease.
We reviewed a series of 18 supratentorial hemangioblastomas in 13 patients with von Hippel-Lindau disease. Clinical, genetic, and serial imaging data and operative records were analyzed.
Hemangioblastomas were most commonly seen in the temporal lobe. Only 6 tumors had a cyst at diagnosis or during follow-up, and only 6 patients had associated symptoms at presentation or during follow-up. The most frequent clinical presentations were intracranial hypertension and visual loss. Of 14 tumors with documented serial imaging, 13 demonstrated tumor growth. Rates and patterns of tumor growth were unique to each patient. The mechanism of cyst formation described in other locations was also demonstrated in the supratentorial region. Patterns of peritumoral edema and rate of cyst formation seemed to be influenced by the presence of anatomic barriers. Germline VHL mutation was identified in all patients, but no specific genotype-phenotype correlation was found, although a familial predisposition is suggested.
This series illustrates the wide variation in tumor locations, patterns of growth, and edema progression seen in supratentorial hemangioblastomas and adds to our knowledge of the natural history of hemangioblastomas.
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ABSTRACT: Supratentorial hemangioblastomas (HBs) are rare, and pituitary stalk HBs are extremely uncommon; therefore, pituitary stalk evaluation is often overlooked. Herein, we report the development of pituitary stalk HB over a 20-year period and the importance of regular long-term follow up for patients with HBs.Journal of Korean Neurosurgical Society 05/2013; 53(5):297-9. · 0.56 Impact Factor
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ABSTRACT: BACKGROUND: Haemangioblastomas are benign vascular tumours that may appear sporadically or in von Hippel-Lindau disease. Despite their higher incidence, sporadic haemangioblastomas have been less studied than syndromic ones. In this article, we evaluate the specific features, outcome and quality of life of patients with intracranial sporadic haemangioblastomas (ISHs) operated on in our institution. METHODS: Between 1998 and 2010, 38 patients harbouring 38 ISHs were operated on in our department. Their clinical, biological, radiological and surgical features were retrospectively reviewed. All patients were contacted for a quality-of-life (QOL) survey assessed by the Short Form 36 questionnaire (SF36). The mean duration of follow-up was 40 months (13-108 months). RESULTS: ISH represented 0.9 % of primary intracranial neoplasms treated in our centre during this period. Patients comprised 23 men and 15 women with a mean age of 47 years. None had polycythaemia. Cerebellar locations accounted for 79 % of ISHs, and brainstem ISH with involvement of the floor of the fourth ventricle represented 11 % of ISHs. At last follow-up, two patients harbouring solid medulla oblongata haemangioblastoma had died following severe bulbar syndrome and five patients had died of unrelated causes. One patient had multiple surgeries for three recurrences. Tumoral control was achieved in all cases at last follow-up. Results of the SF-36 questionnaire were as follows: median physical functioning score 100 (range 0-100), median physical problems score 100 (range 0-100), median bodily pain score 100 (range 45-100), median social functioning score 100 (range 25-100), median general mental health score 84 (range 40-92), median emotional problems score 100 (range 0-100), median vitality score 70 (range 35-80) and median general health perceptions score 70 (range 35-100). Mean QOL scores were similar to the general healthy population. CONCLUSION: Surgery of ISH provides good QOL and tumoral control except for those located in the medulla oblongata. We recommend considering a careful multimodal therapeutic approach, including radiosurgery for these specific locations.Acta Neurochirurgica 04/2013; · 1.55 Impact Factor
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ABSTRACT: 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 infratentorial hemangioblastoma associated with von Hippel-Lindau disease. Patients with von Hippel-Lindau syndrome are at risk for developing multiple hemangioblastomas, with new tumor formation and growth and possible meningeal infiltration. Regular lifelong follow-up in at-risk patients is recommended and should include the differential diagnosis of dural-based tumors such as angioblastic meningioma and metastatic renal cell carcinoma.Journal of Korean Neurosurgical Society 11/2013; 54(5):415-9. · 0.56 Impact Factor