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: 438 relevant articles (15 outcomes, 35 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.	Meningioma (Meningiomas)
5.	Renal Cell Carcinoma (Grawitz Tumor)

Experts

1.	Chew, Emily Y: 11 articles (01/2015 - 01/2005)
2.	Chan, Chi-Chao: 8 articles (01/2015 - 01/2005)
3.	Lonser, Russell R: 8 articles (01/2015 - 08/2005)
4.	Zhuang, Zhengping: 8 articles (08/2014 - 01/2005)
5.	Wong, Wai T: 6 articles (12/2012 - 02/2007)
6.	Neumann, Hartmut P H: 5 articles (06/2013 - 02/2002)
7.	Oldfield, Edward H: 5 articles (04/2011 - 08/2005)
8.	Shields, Carol L: 4 articles (02/2015 - 01/2008)
9.	Shields, Jerry A: 4 articles (02/2015 - 01/2008)
10.	Shen, Defen: 4 articles (08/2014 - 01/2005)

Drugs and Biologics

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

1.	Retinaldehyde (Retinal)IBA 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. " 01/01/1992 - "This study supports the concept that retinal hemangioblastoma is composed of a proliferation of capillaries and glial cells." 01/01/2015 - "Our pathology findings were consistent with previously described features of retinal hemangioblastoma. " 01/01/2015 - "A large retinal hemangioblastoma was located at the posterior pole adjacent to the optic nerve head. " 12/01/2014 - "To report the results of treatments and therapeutic complications of retinal hemangioblastomas (RH). "
2.	bevacizumabFDA Link 01/01/2014 - "For a period of 12 months, therapy with bevacizumab achieved a clinical response and radiological stabilization in a patient with progressive multifocal central nervous system (CNS) hemangioblastoma. " 01/01/2010 - "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. " 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." 01/01/2012 - "Bevacizumab for the treatment of surgically unresectable cervical cord hemangioblastoma: a case report."
3.	Vascular Endothelial Growth Factor A (Vascular Endothelial Growth Factor)IBA 07/01/1998 - "In previous studies, the endothelial-specific mitogen vascular endothelial growth factor (VEGF) was shown to be up-regulated in the stromal cells, the putative neoplastic cells in 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). " 05/01/2010 - "The protein expressions of CXCR4 and VEGF were detected by SP immunohistochemical staining in 40 hemangioblastomas. "
4.	DNA (Deoxyribonucleic Acid)IBA 12/01/2000 - "To obtain a clearer understanding of the pathogenesis of this unusual case and its relationship to VHL syndrome, molecular analysis of the VHL gene was performed by use of complete sequence analysis and loss of heterozygosity studies on deoxyribonucleic acid derived from the patient's blood leukocytes and three separately resected hemangioblastomas. " 01/01/2014 - "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. " 12/01/2000 - "Loss of heterozygosity analysis of deoxyribonucleic acid from the three hemangioblastoma resections showed normal heterozygosity in the 3p25-26 region. " 01/01/1998 - "A similar screening in 18 patients with sporadic hemangioblastoma revealed 2 missense DNA variants. "
5.	Carbonic Anhydrases (Carbonic Anhydrase)IBA 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. "
6.	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. "
7.	Indocyanine Green (Cardio-Green)FDA LinkGeneric 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." 05/01/2015 - "To introduce a resourceful method, we present a case of spinal hemangioblastoma for which temporary arterial occlusion during near-infrared intraoperative indocyanine green (ICG) videoangiography gives useful assessment of the main and minor feeders easily. " 05/01/2015 - "Visualization of vascular structure of spinal hemangioblastoma using intraoperative indocyanine green videoangiography and temporary feeder occlusion." 09/01/2013 - "We report our experience using intraoperative indocyanine green (ICG) videoangiography during the surgical resection of spinal cord hemangioblastomas (SH) and evaluate the advantages and limitations of this technique. " 09/01/2013 - "Application of intraoperative indocyanine green videoangiography for resection of spinal cord hemangioblastoma: advantages and limitations."
8.	SU 5416 (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. "
9.	Vascular Endothelial Growth Factor Receptors (VEGF Receptors)IBA 03/15/1995 - "Our findings suggest that, in VHL disease-associated and sporadic hemangioblastomas, VEGF121 and VEGF165 are secreted by stromal cells and interact with the corresponding VEGF receptors expressed on tumor endothelial cells. " 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. " 03/15/1995 - "Northern blot and in situ hybridization analysis revealed significant up-regulation of VEGF and VEGF receptor expression in VHL disease-associated and sporadic hemangioblastomas compared to normal brain and tumor stromal cells as sites of abundant VEGF transcription. " 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. "
10.	ClusterinIBA 01/01/2007 - "Ocular specimens were evaluated by routine histology, immunohistochemistry for clusterin expression, and molecular detection of clusterin transcripts within ocular VHL hemangioblastomas compared with normal tissue from the same eye using microdissection and quantitative real-time PCR. " 01/01/2007 - "Marked decrease of clusterin immunoreactivity was detected in all retinal hemangioblastoma and the optic nerve hemangioblastoma, whereas positive clusterin reactivity of the vascular and glial components was similar to that of normal retina. " 01/01/2007 - "Further investigation of clusterin may provide better understanding of retinal hemangioblastoma associated with VHL disease." 01/01/2007 - "Clusterin shows possible important functions in tumor suppression by the VHL gene product (pVHL) and the potential to be a novel biomarker in retinal hemangioblastoma associated VHL disease. " 01/01/2007 - "Quantitative real-time PCR analysis confirmed the decrease of clusterin mRNA in the VHL associated retinal hemangioblastoma and optic nerve hemangioblastoma in five cases. "

Therapies and Procedures

1.	Particle Accelerators 01/01/2012 - "Results of this limited experience indicate linear accelerator-based radiosurgery is safe and effective for spinal cord hemangioblastomas. " 01/01/2012 - "To retrospectively review the efficacy and safety of image-guided linear accelerator-based radiosurgery for spinal hemangioblastomas. " 01/01/2012 - "Image-guided linear accelerator-based spinal radiosurgery for hemangioblastoma." 07/01/2014 - "The objective of this study was to analyze the clinical results of 14 patients with 56 intracranial hemangioblastomas treated with linear accelerator (linac)-based stereotactic radiosurgery (SRS) and radiotherapy (SRT) in the same institute. " 01/01/2012 - "Longer follow-up is necessary to confirm the durability of tumor control, but these initial results imply linear accelerator-based radiosurgery may represent a therapeutic alternative to surgery for selected patients with spinal hemangioblastomas."
2.	Lasers (Laser) 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." 12/01/2011 - "We evaluated 20 archival renal cell carcinomas (RCCs) and 20 hemangioblastomas (HBs) for biomarker expression levels using laser-scanning cytometry (LSC). " 07/01/2011 - "Nine patients with hemangioblastoma were given 1 g of 5-ALA orally before surgery, and a laser beam of 405 nm was focused on the tumor during resective surgery. " 04/01/1980 - "A surgical carbon dioxide laser unit (Laser) has been used in 26 cases of various brain tumors, including 10 meningioma (4 sphenoid ridge, 3 parasagittal, 1 falx, 1 olfactory, 1 posterior fossa); 12 glioma (7 glioblastoma, 5 astrocytoma); two metastatic brain tumors; 1 hemangioblastoma, and 1 AVM. "
3.	Craniotomy 11/01/1993 - "Craniotomy and resection is usually a safe and effective treatment for hemangioblastoma. " 01/01/2014 - "This video demonstrates the excision of a brainstem hemangioblastoma via a left retrosigmoid craniotomy under Stealth guidance. " 02/01/1985 - "He had undergone a craniotomy 2 years previously for the diagnosis of a cerebellar hemangioblastoma. " 03/01/1985 - "We describe a patient with a strongly positive family history of this disease who, at age 28, underwent craniotomy with removal of a cystic cerebellar hemangioblastoma; at age 48, he developed syringomyelia of the spinal cord, became quadriplegic, and had a progressive downhill course. " 04/01/2011 - "She underwent craniotomy for cerebellar hemangioblastoma at the age of 22 years and total pancreatectomy for multiple neuroendocrine tumors at the age of 24 years. "
4.	Light Coagulation 10/01/1987 - "[A case of bilateral papillary hemangioblastoma treated by photocoagulation]." 02/01/2005 - "Small retinal tumours are treated by photocoagulation, big hemangioblastomas by cryotherapy. " 01/01/2012 - "Laser photocoagulation performed 2 weeks after the intravitreal bevacizumab injection provided further regression of exudation and scarring of the hemangioblastomas. " 01/01/2007 - "Large retinal hemangioblastomas (1-3 disc diameters) and vasoproliferative tumors were resected surgically after laser photocoagulation in 4 patients. " 01/01/2012 - "To report a patient with retinal capillary hemangioblastomas associated with Von Hippel-Lindau syndrome who achieved long-term visual improvement with single intravitreal bevacizumab injection followed by laser photocoagulation. "
5.	Radiotherapy 07/01/2014 - "The clinical outcome of intracranial hemangioblastomas treated with linac-based stereotactic radiosurgery and radiotherapy." 01/01/2014 - "Antiangiogenic treatment has been explored in few patients with hemangioblastoma after failure of surgery and radiotherapy. " 12/01/2007 - "To assess the clinical outcomes and toxicity in patients receiving fractionated external beam radiotherapy (EBRT) for hemangioblastoma of the central nervous system, treated at two Canadian radiation oncology institutions. " 12/01/2007 - "Role of fractionated external beam radiotherapy in hemangioblastoma of the central nervous system." 10/01/1996 - "Six hemangioblastomas were treated with radiotherapy at a median margin dose of 25 Gy (20-35 Gy) before 1990 and the next five with a median of 10 Gy (5-19 Gy). "