Hemangioblastoma

A benign tumor of the nervous system that may occur sporadically or in association with VON HIPPEL-LINDAU DISEASE. It accounts for approximately 2% of intracranial tumors, arising most frequently in the cerebellar hemispheres and vermis. Histologically, the tumors are composed of multiple capillary and sinusoidal channels lined with endothelial cells and clusters of lipid-laden pseudoxanthoma cells. Usually solitary, these tumors can be multiple and may also occur in the brain stem, spinal cord, retina, and supratentorial compartment. Cerebellar hemangioblastomas usually present in the third decade with INTRACRANIAL HYPERTENSION, and ataxia. (From DeVita et al., Cancer: Principles and Practice of Oncology, 5th ed, pp2071-2)

Also Known As:

Networked: 744 relevant articles (28 outcomes, 51 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Neoplasms (Cancer)
2.	Hemangioblastoma
3.	von Hippel-Lindau Disease (von Hippel Lindau Disease)
4.	Renal Cell Carcinoma (Grawitz Tumor)
5.	Cysts

Experts

1.	Chew, Emily Y: 18 articles (12/2021 - 09/2005)
2.	Oldfield, Edward H: 13 articles (04/2018 - 04/2002)
3.	Zhuang, Zhengping: 13 articles (01/2018 - 04/2002)
4.	Lonser, Russell R: 13 articles (10/2016 - 08/2005)
5.	Shields, Carol L: 9 articles (01/2022 - 01/2008)
6.	Wong, Wai T: 9 articles (12/2021 - 02/2007)
7.	Chan, Chi-Chao: 9 articles (01/2018 - 09/2005)
8.	Shields, Jerry A: 8 articles (01/2022 - 01/2008)
9.	Linehan, W Marston: 6 articles (12/2021 - 08/2006)
10.	Gläsker, Sven: 6 articles (10/2020 - 04/2006)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Hemangioblastoma:

1.	Retinaldehyde (Retinal)IBA 11/01/2019 - "This study reports on improved detection of retinal hemangioblastomas with widefield FA compared to widefield fundus images. " 03/01/2022 - "Final visual acuity improved from counting fingers to 20/50 with total retinal reattachment and regression of the retinal capillary hemangioblastoma. " 01/01/2019 - "The primary endpoint of the study was the number and size of retinal hemangioblastomas. " 01/01/2018 - "In the first national study in which almost all participants were genetically tested, vHL was the underlying cause of retinal hemangioblastoma in 84% of cases; more often than previously reported. " 08/16/2013 - "A number of studies have shown coexistence of pro-angiogenic and stem cell markers in 'tumorlet-like stromal cells' in the retinal and optic nerve hemangioblastomas, leading to suggestions that hemangioblastomas originate from developmentally arrested stem cells or embryonic progenitors. "
2.	Vascular Endothelial Growth Factor A (Vascular Endothelial Growth Factor)IBA 01/01/2022 - "We found no evidence for VEGF secretion from CNS hemangioblastomas in vHL in circulating blood. " 12/01/2019 - "Vascular endothelial growth factor inhibition continuity is a good therapeutic option, which improves outcomes of von Hippel-Lindau-related hemangioblastomas." 01/01/2014 - "The VHL-HIF-1 regulated genes VEGF and CAIX are expressed in hemangioblastomas but significant HIG-2 expression is not observed. " 05/01/2010 - "CXCR4 and VEGF may collaborate to induce angiogenesis in hemangioblastomas." 05/01/2010 - "The expression rates of CXCR4 and VEGF were found positive obviously in hemangioblastomas compared with the normal cerebellar tissues (P < 0.01). "
3.	Bevacizumab (Avastin)FDA Link 10/21/2010 - "Due to the short-term nature of these studies, we attempted long-term bevacizumab treatment over 60 months in a monocular subject with progressive visual loss due to a VHL associated macular and optic nerve hemangioblastoma. " 12/01/2022 - "Intra-arterial Bevacizumab for Posterior Fossa Hemangioblastoma." 01/01/2020 - "Cervicomedullary hemangioblastoma treated with bevacizumab." 01/01/2014 - "We present the first histopathological follow-up study of a bevacizumab-responsive hemangioblastoma that eventually progressed. " 01/01/2014 - "Neuropathological characteristics of progression after prolonged response to bevacizumab in multifocal hemangioblastoma."
4.	Indocyanine Green (Cardio-Green)FDA LinkGeneric 01/01/2021 - "Efficacy of Intra-arterial Indocyanine Green Videoangiography in Hemangioblastoma Surgery: A Case Report." 04/01/2012 - "Efficacy of indocyanine green videography and real-time evaluation by FLOW 800 in the resection of a spinal cord hemangioblastoma in a child: case report." 02/12/2021 - "Utility of indocyanine green in the detection of radiologically silent hemangioblastomas: case report." 01/01/2021 - "Intravenous indocyanine green (ICG) videoangiography is reportedly useful for vascular neurosurgery, and for treating hemangioblastoma due to its high vascularity. " 01/01/2021 - "Second-Window Indocyanine Green for Visualization of Hemangioblastoma: A Case Report With Two-Dimensional Operative Video."
5.	DNA (Deoxyribonucleic Acid)IBA 06/01/2023 - "The present study aimed to determine genomic changes in sporadic intracranial hemangioblastoma (HBL), and the mutation patterns were analyzed using next-generation DNA sequencing (NGS). " 06/01/2023 - "Supratentorial Sporadic Hemangioblastoma: A Case Report With Mutation Profiling Using Next-Generation DNA Sequencing." 10/01/2016 - "27 patients with a hemangioblastoma were included for analysis of immunohistochemistry of tissue, MRI and DNA. " 12/24/2014 - "We performed deep-coverage DNA sequencing on 32 sporadic hemangioblastomas (whole exome discovery cohort n = 10, validation n = 22), followed by analysis of clonality, copy number alteration, and somatic mutation. " 10/01/2004 - "DNA losses at 6q are frequent alterations in hemangioblastomas, as shown by comparative genomic hybridization. "
6.	Ranibizumab (Lucentis)FDA Link 11/01/2008 - "To evaluate the effect of intravitreal ranibizumab on retinal capillary hemangioblastomas (RCHs) associated with von Hippel-Lindau (VHL) disease that are not amenable or responsive to standard therapy. " 04/01/2008 - "In a single case decision a patient with active retinal hemangioblastomas due to VHL disease received repeated intravitreal injections of 0.5 mg ranibizumab. " 11/01/2008 - "Intravitreal ranibizumab therapy for retinal capillary hemangioblastoma related to von Hippel-Lindau disease." 08/01/2015 - "Oral propranolol and intravitreal ranibizumab for refractory serous macular detachment secondary to retinal capillary hemangioblastoma." 10/21/2010 - "Several short-term case series using ranibizumab (Lucentis, mAb fragment of bevacizumab with stronger affinity for VEGF-A) have shown it has promising but minimal success on most VHL-related hemangioblastomas [8, 9]. "
7.	Protons (Proton)IBA 03/01/2015 - "The purpose of this study was to evaluate the usefulness of dynamic susceptibility-weighted contrast-enhanced perfusion-weighted imaging (DSC-PWI) and proton MR spectroscopic imaging in the differentiation of hemangioblastomas and PAs. " 03/01/2015 - "Four patients with hemangioblastomas and 10 PA patients also underwent proton MR spectroscopy. " 05/01/2010 - "However, further investigations are required to establish the typical characteristics of proton MRS of hemangioblastoma." 05/01/2010 - "These unique results of proton MRS can play an important role in the differential diagnosis of intracranial hemangioblastoma. " 05/01/2010 - "Combined with the absence of the necrotic component on magnetic resonance imaging, this Lip peak on proton MRS could be the characteristic pattern of hemangioblastoma. "
8.	Carbonic Anhydrase IXIBA 07/01/2015 - "Specific immunohistochemical pattern of carbonic anhydrase IX is helpful for the diagnosis of CNS hemangioblastoma." 01/01/2014 - "Carbonic anhydrase IX staining in particular may be a helpful marker of hemangioblastomas but does not aid in the distinction from CCRCC." 10/01/2010 - "The diffuse strong carbonic anhydrase IX expression suggested that soft-tissue hemangioblastoma may share with central nervous system hemangioblastoma the tumorigenic mechanism involving the von Hippel-Lindau gene product and hypoxia-inducible factor. " 07/01/2015 - "Carbonic anhydrase IX with cytokeratin is thus a highly sensitive and specific marker combination for hemangioblastomas. " 07/01/2015 - "The pattern of strong, diffuse expression of carbonic anhydrase IX with membranous accentuation in combination with keratin negativity was considered diagnostic for hemangioblastomas. "
9.	belzutifanIBA 11/01/2023 - "Belzutifan appears to be an effective and safe treatment for CNS hemangioblastoma in VHL patients. " 11/01/2023 - "The outcome of central nervous system hemangioblastomas in Von Hippel-Lindau (VHL) disease treated with belzutifan: a single-institution retrospective experience." 08/01/2023 - "We showed that belzutifan is safe and well-tolerated with strong disease response for CNS hemangioblastoma in adults with VHL, supporting continued use of belzutifan in this patient population. " 08/01/2023 - "Our objective was to report on our clinical experience with belzutifan in adult patients with VHL-associated CNS hemangioblastoma. " 08/01/2023 - "Belzutifan in adults with VHL-associated central nervous system hemangioblastoma: a single-center experience."
10.	Semaxinib (SU5416)IBA 05/20/2005 - "Case 2. Hemangioblastomas: diagnosis of von Hippel-Lindau disease and antiangiogenic treatment with SU5416." 02/01/2004 - "Antiangiogenic treatment with interferon (IFN) alpha-2a and IFN alpha-2b and with SU 5416 has been reported to be effective and well tolerated in several patients with previously progressive angioblastomas and hemangioblastomas. " 09/01/2002 - "We report rapid, extensive, and durable recovery of visual function after systemic administration of the VEGF receptor inhibitor SU5416 to a patient with VHL syndrome and optic nerve head hemangioblastoma. " 09/01/2002 - "To present a case of rapid and durable recovery of visual function in a patient with von Hippel-Lindau syndrome and optic nerve head hemangioblastoma after systemic treatment with the vascular endothelial growth factor (VEGF) receptor inhibitor SU5416. " 05/15/2002 - "We observed, after 3 to 4 months of treatment, secondary paradoxical polycythemia in 3 VHL patients with CNS or retinal hemangioblastomas treated by the anti-VEGF receptor SU5416. "

Therapies and Procedures

1.	Lasers (Laser) 09/18/2023 - "Intraoperative confocal laser endomicroscopy for interpretation of a sellar hemangioblastoma: illustrative case." 01/01/2020 - "Laser treatment should be considered as the primary treatment option for patients with capillary hemangioblastomas with and without exudation and can be combined with intravitreal antiangiogenics if exudation is significant. " 01/01/1993 - "We report on eight patients with spinal hemangioblastoma treated in our clinic since the installation of magnetic resonance imaging and laser-assisted microneurosurgery." 07/01/1990 - "It is concluded that microsurgical removal guided by MRI imaging and aided by the application of laser energy is the method of choice in the treatment of spinal hemangioblastomas." 09/01/2017 - "Six hemangioblastomas in the peripheral retina were treated with laser photocoagulation. "
2.	Light Coagulation 10/01/1987 - "[A case of bilateral papillary hemangioblastoma treated by photocoagulation]." 09/01/2017 - "Six hemangioblastomas in the peripheral retina were treated with laser photocoagulation. " 12/01/2018 - "Twin A was treated with laser photocoagulation to the larger hemangioblastoma in the left eye, and the asymptomatic juxtapapillary tumor was observed. " 03/01/2022 - "Vitrectomy, endolaser photocoagulation to tears adjacent to the original hemangioblastoma lesion, and silicone oil exchange was performed to repair the detachment. " 02/01/2005 - "Small retinal tumours are treated by photocoagulation, big hemangioblastomas by cryotherapy. "
3.	Therapeutics 10/01/2022 - "Bibliometric Analysis of the 100 Most Influential Hemangioblastoma Research Articles Illustrates Progress in Clinical Management and Room for Growth in Targeted Therapies." 01/01/2016 - "Surgical therapy for cerebellar hemangioblastoma was performed, and histological examination was consistent in cerebellar hemangioblastoma. " 09/29/2014 - "An effective therapy for multifocal central nervous system hemangioblastoma (CNS HB) is needed. " 05/01/2014 - "Unpredictable growth of hemangioblastomas compromises assessment of nonsurgical therapies. " 04/01/2013 - "Mechanisms, indications and results of salvage systemic therapy for sporadic and von Hippel-Lindau related hemangioblastomas of the central nervous system."
4.	Radiotherapy 01/01/2020 - "The standard treatment for cerebellar hemangioblastoma is complete microsurgical removal, but our results show a high level of efficacy for fractional photon radiotherapy after partial surgery of this benign tumour." 01/01/2022 - "Stereotactic radiotherapy for spinal hemangioblastoma - disease control and volume analysis in long-term follow up." 01/01/2021 - "Herein, we report a case of hemangioblastoma in which leptomeningeal dissemination occurred 2 years after hypofractionated radiation therapy (39 Gy/13 fractions). " 01/01/2020 - "Since the surgical removal was partial the patient was referred to our training where he received external radiotherapy on his hemangioblastoma of the posterior brain fossa. " 01/01/2020 - "A solitary hemangioblastoma of the posterior brain fossa: the role of radiotherapy."
5.	Laminectomy 01/01/2019 - "Laminectomy and partial resection of hemangioblastoma are safe and effective treatments to resolve impingement of the spinal cord. " 04/01/2011 - "The authors included consecutive patients enrolled in a prospective VHL disease natural history study who underwent cervical laminectomy(s) for spinal cord hemangioblastoma resection. " 04/01/2011 - "Whereas patients with VHL disease will often require multiple laminectomies for cervical spinal cord hemangioblastoma resection, a limited number of patients (12%) will develop clinical instability. " 07/01/1978 - "Laminectomy was performed at levels of L2 through L4 and a hemangioblastoma of the cauda equina was totally excised at L3 level. " 01/01/1978 - "In a 37 year old male patient a solitary hemangioblastoma of the cauda equina was demonstrated by myelography and totally removed by laminectomy. "