BACKGROUND AND PURPOSE: Cerebellar hemangioblastomas (HBs) are traditionally classified into different morphologic types: cystic and. Hemangioblastomas (capilliary hemangioblastomas) are tumors of the central nervous system that originate from the vascular system usually during middle- age. The occurrence of cerebral hemangioblastoma in a patient with on Hippel-Lindau disease is very rare. In , Rochat described a cerebral hemangioblastoma.

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Supratentorial hemangioblastoma is a rare form gemangioblastoma hemangioblastoma; little information is available regarding prognosis, treatment, and clinical characteristics, because the available literature is primarily composed of case reports and small case series.

Therefore, we performed a systematic review of the literature to analyze clinical characteristics, disease progression, and surgical outcomes with respect to survival for supratentorial hemangioblastomas. A total of articles met the search criteria, which combined for a total of patients. On the basis of our analysis of the literature on published cases of supratentorial hemangioblastoma, gross-total resection appears to be superior to other treatment modalities in extending PFS.

Hemangioblastomas are uncommon tumors of the central nervous system CNS that were first described by von Hippel in Supratentorial hemangioblastomas are exceedingly rare. Because of the rarity of hemangioblastomas in this location, the literature is primarily composed of case reports and small series. Little information is available regarding the clinical features of these tumors, particularly in comparison with lesions found in other locations.

No consensus is currently available regarding appropriate management approaches for supratentorial hemangioblastomas or whether supratentorial hemangioblastoma is part hemangioblastooma the stigmata of VHL.

To supplement the current understanding of the clinical significance and management of supratentorial hemangioblastoma, we systematically reviewed the published literature to determine what is known about the presentation and clinical course in patients with this disease.

Where possible, we performed statistical analysis to determine the efficacy of various treatment approaches using the existing body of published patient data. A comprehensive systematic review of the English-language literature hemangiolbastoma performed. A total of articles was identified, of which had patient data regarding supratentorial hemangioblastomas.

All references that contained individual patient data or purely supratentorial aggregated data sets of either histologically confirmed tumors or tumors presenting in the context of confirmed diagnosis of VHL disease were included in our analysis.

Patients with tumors unrelated to hemangioblastoma or VHL disease were excluded from all analyses.

Data from individual and aggregated case series were extracted from each article. The median largest tumor dimension and median tumor volume were not reportable or analyzable in our analysis, hemangiobblastoma studies did not consistently report either value. Patients were considered to have VHL disease if the study reported that they met clinical diagnostic criteria or genetic testing requirements.

Cerebral Hemangioblastoma Occurring in a Patient with von Hippel-Lindau Disease

A clinical diagnosis was made if patients met either of the following 2 criteria: Kaplan-Meier analysis was used to generate time to progression curves. Differences in time to progression were analyzed using the log-rank test. Five percent of patients underwent postsurgical treatment with fractionated radiotherapy.


Follow-up ranged from 0 to months in these studies. All patients receiving fractionated RT as the only treatment modality had presumed hemangioblastoma based on diagnosis of VHL disease. Rate of progression-free survival among patients with gross-total resection GTR and radiation therapy RT of tumors. Because of limitations of the available data on survival, survival analysis provided no significant results for specific treatment modalities. There was a trend toward significance in the association between presence of cysts and PFS; patients with cystic tumors were less likely to present with progression than were those with solid tumors 5-year PFS: When stratified by age, sex, and cystic composition, the association remained significant.

No other significant association was found between tumor location and VHL disease. CNS hemangioblastomas, especially multiple hemangioblastomas, are the most common manifestation of VHL disease.

Our principle goal was to identify features of these tumors that would not be immediately obvious by reading isolated case reports. Of more importance, among 14 tumors with documented serial imaging, 13 tumors showed growth, suggesting that these tumors show high propensity for growth.

Hemangioblastoma is currently considered to be a grade I meningeal neoplasm of uncertain origin according to the latest World Health Organization classification of CNS tumors. Thus, surgery has been the mainstay treatment for CNS hemangioblastomas with good outcomes. In series by Jagannathan et al. Solid tumors seem to have a higher rate of recurrence. Moreover, hemangioblastomas in patients with VHL have high propensity for tumor growth Asymptomatic lesions ceerebral be carefully followed up with a low threshold for surgical resection with development of new symptoms given low risks involved with surgery.

In one study by Jagannathan et al. As a retrospective study, this review is limited by the data available and may reflect source study biases. The diverse range of available data limits hemangioblatoma number of variables that can be analyzed and controlled.

Variables of possible interest, such as tumor volume and histological variants, were reported inconsistently and could not be analyzed. In conclusion, we report patient, tumor, and treatment characteristics for previously published cases of supratentorial hemangioblastomas.

Because of the relative rarity of this tumor, this study aims to accurately describe outcome and tumor location characteristics using a data set that would be difficult to accumulate at a single center treating this tumor.

Oxford University Press is a department of the University of Oxford. It furthers the University’s objective of excellence in research, scholarship, and education by publishing worldwide. Sign In or Create an Account. Close mobile search navigation Article navigation. Department of Neurological Surgery S. Abstract Supratentorial hemangioblastoma is a rare form of hemangioblastoma; little information is available regarding prognosis, treatment, and clinical characteristics, because the available literature is primarily composed of case reports and small case series.

Hmangioblastoma large Download slide. Rate of progression-free survival among patients with cystic tumors and solid tumors. Intradural extramedullary hemangioblastoma differentiated by MR images hemangioblastomma the cervical spine: Central nervous system capillary haemangioblastoma: Surgical management of cerebellar hemangioblastomas in patients with von Hippel-Lindau disease.

Hemangioblastomas of the cerbral nervous system in von Hippel-Lindau syndrome and sporadic disease. Natural history of supratentorial hemangioblastomas in von Hippel-Lindau disease.


Supratentorial capillary hemangioblastoma presenting with fatal spontaneous intracerebral hemorrhage. Progressive peritumoral edema defining the optic cerebeal and nemangioblastoma in reversible visual loss.

Csrebral nervous system haemangioblastoma: Aggressive leptomeningeal hemangioblastomatosis of the central nervous system in a patient with von Hippel-Lindau disease. Pituitary hemangioblastoma in a patient with von Hippel-Lindau disease. Glial fibrillary acidic protein in stromal cells of some capillary hemangioblastomas: Report of a case and review of the literature. Unusual, rod-shaped cytoplasmic inclusions Hemangioblastoam bodies in a cerebellar hemangioblastoma. Diagnosis of von Hippel-Lindau disease in a patient with blindness resulting from bilateral optic nerve hemangioblastomas.

Suprasellar hemangioblastoma in a patient with von Hippel-Lindau disease confirmed by germline mutation study: Inhibin alpha distinguishes hemangioblastoma from clear cell renal cell carcinoma. Cerebral hemangioblastoma occurring in a patient with von Hippel-Lindau disease.

Case study and ultrastructural characteristics. Congenital cystic hemangioblastomas of the cerebral hemisphere in a neonate without alteration in the VHL gene. Immunoreactivity of CD10 and inhibin alpha in differentiating hemangioblastoma of central nervous system from metastatic clear cell renal cell carcinoma. Congenital supratentorial cystic hemangioblastoma. Case report and review of the literature. Astrocytes in hemangioblastomas of the central nervous system and their relationship to stromal cells.

Congenital supratentorial hemangioblastoma as an unusual cause of simultaneous supra- and infratentorial intracranial hemorrhage: Resection of suprasellar tumors by using a modified transsphenoidal approach.

Haemangioblastoma (central nervous system) | Radiology Reference Article |

Report of four cases. Expression of vascular endothelial growth factor in von Hippel-Lindau syndrome-associated papillary cystadenoma of the epididymis. Dynamic MR imaging of neurohypophyseal germ cell tumors for differential diagnosis of infundibular diseases. Spontaneous regression of two putative supratentorial haemangioblastomas in one patient.

Clinical course of retrobulbar hemangioblastomas in von Hippel-Lindau disease.

Pathology Outlines – Hemangioblastoma

Supratentorial hemangioblastoma associated with Von Hippel Lindau disease: Long-term prognosis of hemangioblastomas of the central nervous system: Immunohistochemical study of hemangioblastoma with special reference to its cytogenesis.

Stereotactic radiosurgical treatment of cranial and spinal hemangioblastomas. Hemangioblastomas of the central nervous system. A year study with special reference to von Hippel-Lindau syndrome. Hemangioblastoma of hippocampus without von Hippel-Lindau disease: An unusual case of a highly progressive supratentorial capillary haemangioblastoma – histopathological considerations.

Report of two cases and review of the literature. Hemangioblastoma of the lateral ventricle: Successful surgical removal of an asymptomatic optic nerve hemangioblastoma in von Hippel-Lindau disease. Cellular and reticular variants of hemangioblastoma differ in their cytogenetic profiles. Report of a rare case and review of literature.

Symptomatic intrasellar hemangioblastoma in a child treated with subtotal resection and adjuvant radiosurgery. Supratentorial haemangioblastoma not associated with Von Hippel Lindau complex or polycythaemia: Supratentorial dural-based hemangioblastoma not associated with von Hippel Lindau complex. Multiple intracerebral haemangioblastomas in identical twins with von Hippel-Lindau disease—a clinical and molecular study.