2,338 results match your criteria Hemangioblastoma


Von Hippel-Lindau Disease: Current Challenges and Future Prospects.

Onco Targets Ther 2020 16;13:5669-5690. Epub 2020 Jun 16.

Department of Pathology, Indiana University-Purdue University, Indianapolis, IN, USA.

Understanding of molecular mechanisms of tumor growth has an increasing impact on the development of diagnostics and targeted therapy of human neoplasia. In this review, we summarize the current knowledge on molecular mechanisms and their clinical implications in von Hippel-Lindau (VHL) disease. This autosomal dominant tumor syndrome usually manifests in young adulthood and predisposes affected patients to the development of benign and malignant tumors of different organ systems mainly including the nervous system and internal organs. Read More

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http://dx.doi.org/10.2147/OTT.S190753DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7305855PMC

Primary and secondary retinal capillary haemangioma in Mexico.

Arch Soc Esp Oftalmol 2020 Jun 26. Epub 2020 Jun 26.

Departamento de Retina y Vítreo, Fundación Hospital Nuestra Señora de la Luz, IAP, Ciudad de México, México.

A series is presented of 16 cases of retinal capillary haemangioma from consecutive patients at an ophthalmology teaching hospital in Mexico City. There were 7 primary haemangioblastomas, and 9 due to von Hippel-Lindau disease. All cases associated with this disease already had systemic manifestations, such as cerebellar, medullary and renal tumours. Read More

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http://dx.doi.org/10.1016/j.oftal.2020.05.024DOI Listing

Retinal Artery Macroaneurysm Associated With Retinal Hemangioblastoma.

Ophthalmic Surg Lasers Imaging Retina 2020 Jun;51(6):365-366

A 34-year-old Caucasian woman with a past medical history of hypertension presented with acute floaters in the right eye (OD) for 1 week. Best-corrected visual acuity (VA) was 20/20 in both eyes. Fundus examination OD (Figure 1) revealed a retinal hemangioblastoma (RH) in the temporal midperiphery with a prominent feeding artery and draining vein. Read More

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http://dx.doi.org/10.3928/23258160-20200603-07DOI Listing

Juxtapapillary Capillary Retinal Angioma and Epiretinal Membrane Management in a Monocular Patient with Von Hippel-Lindau Syndrome.

Retin Cases Brief Rep 2020 Jun 10. Epub 2020 Jun 10.

University Hospitals Eye Institute, Case Western Reserve University Cleveland, Ohio.

Purpose: To report a case of von Hippel-Lindau (VHL) syndrome in a monocular patient with progressive vision loss due to Juxtapapillary Capillary Retinal Angioma and epiretinal membrane (ERM).

Patient: We describe a 37-year-old white male patient with von Hippel-Lindau (VHL) syndrome who presented for retinal evaluation. The right eye was blind with NLP vision. Read More

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http://dx.doi.org/10.1097/ICB.0000000000001021DOI Listing

Cerebral Hemangioblastoma Without Von Hippel-Lindau Syndrome: A Report of 6 Cases.

Int J Surg Pathol 2020 Jun 14:1066896920933998. Epub 2020 Jun 14.

Department of Neurosurgery, The First Hospital of Jilin University, Changchun, China.

. Hemangioblastoma occurs mainly in the cerebellum and rarely in the cerebrum. . Read More

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http://dx.doi.org/10.1177/1066896920933998DOI Listing

Intramedullary Masses of the Spinal Cord: Radiologic-Pathologic Correlation.

Radiographics 2020 Jul-Aug;40(4):1125-1145. Epub 2020 Jun 12.

From the Department of Radiology, Uniformed Services University of the Health Sciences, 4301 Jones Bridge Rd, Bethesda, MD 20814 (R.Y.S.); and Department of Radiology, Mayo Clinic, Rochester, Minn (K.K.K.).

Spinal cord tumors are a challenge for patients and neurosurgeons because of the high risk of neurologic deficits from the disease process and surgical interventions. Spinal cord tumors are uncommon, and approximately 2%-3% of primary intra-axial tumors of the central nervous system occur in the spinal cord. Primary intra-axial tumors are usually derived from neuroepithelial tissue, especially glial cells. Read More

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http://dx.doi.org/10.1148/rg.2020190196DOI Listing

Hemangioblastoma and von Hippel-Lindau disease: genetic background, spectrum of disease, and neurosurgical treatment.

Childs Nerv Syst 2020 Jun 7. Epub 2020 Jun 7.

Department of Neurosurgery, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Breisacher Str. 64, 79106, Freiburg, Germany.

Introduction: Hemangioblastomas are rare, histologically benign, highly vascularized tumors of the brain, the spinal cord, and the retina, occurring sporadically or associated with the autosomal dominant inherited von Hippel-Lindau (VHL) disease. Children or adults with VHL disease have one of > 300 known germline mutations of the VHL gene located on chromosome 3. They are prone to develop hemangioblastomas, extremely rarely starting at age 6, rarely at age 12-18, and, typically and almost all, as adults. Read More

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http://dx.doi.org/10.1007/s00381-020-04712-5DOI Listing

Cerebellar hemangioblastoma during pregnancy: Management options and review of literature.

Surg Neurol Int 2020 23;11:123. Epub 2020 May 23.

Department of Neurosurgery, All India Institute of Medical Science, Raipur, Chhattisgarh, India.

Background: Symptomatic cerebellar hemangioblastomas are extremely rare in pregnant women and the ideal management is not well established. In the present article, we aimed to report a case of large cerebellar hemangioblastoma complicated by pregnancy and managed successfully by surgical resection. In addition, we also discuss management options and review of the current literature pertaining to this pathology. Read More

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http://dx.doi.org/10.25259/SNI_203_2020DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7265369PMC

CLN5 in heterozygosis may protect against the development of tumors in a VHL patient.

Orphanet J Rare Dis 2020 Jun 2;15(1):132. Epub 2020 Jun 2.

Centro de Investigaciones Biológicas Margarita Salas, Consejo Superior de Investigaciones Científicas (CSIC), Madrid, Spain.

Von Hippel-Lindau syndrome (VHL) is a rare disease of dominant inheritance that increases susceptibility to tumor development, with a complete penetrance at the age of 60. In this report, we present the unprecedented case of a VHL carrier who remains healthy at 72. Under the course of this study, it was discovered that this patient carries a mutation for a second rare disease, Neuronal Ceroid Lipofuscinosis (NCL or CNL). Read More

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http://dx.doi.org/10.1186/s13023-020-01410-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7268215PMC

Stereotactic radiosurgery for central nervous system hemangioblastoma in von Hippel-Lindau disease: A systematic review and meta-analysis.

Clin Neurol Neurosurg 2020 May 15;195:105912. Epub 2020 May 15.

Department of Urology, Peking University First Hospital, Beijing, China; Institute of Urology, Peking University, Beijing, China; National Urological Cancer Center, Beijing, China. Electronic address:

Von Hippel-Lindau (VHL) disease is a dominantly inherited disorder marked by multiorgan tumors, such as central nervous system benign hemangioblastomas (CHB). Stereotactic radiosurgery (SRS) has also been used to treat CHB for a long time. The purpose of this meta-analysis is to provide a long-term outcome of SRS for VHL-associated CHB by reviewing published studies. Read More

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http://dx.doi.org/10.1016/j.clineuro.2020.105912DOI Listing

Freiburg Neuropathology Case Conference : A Hearing-Impaired Patient Presenting with Lower Limb Ataxia and Progressive Personality Changes.

Clin Neuroradiol 2020 Jun;30(2):413-418

Department of Neuropathology, Medical Centre, University of Freiburg, Freiburg, Germany.

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http://dx.doi.org/10.1007/s00062-020-00912-3DOI Listing

Intramedullary spinal cord tumours - a single Centre, 10-year review of clinical and pathological outcomes.

Br J Neurosurg 2020 May 22:1-4. Epub 2020 May 22.

Department of Neurosurgery, Leeds General Infirmary, Leeds, UK.

Intramedullary spinal cord tumours are relatively rare tumours of the central nervous system. Surgical outcomes are affected by many variables, including pre-operative neurological function, tumour histology and extent of resection. Emphasis remains on surgical treatment due to limited adjunctive therapeutic options and poor drug penetration. Read More

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http://dx.doi.org/10.1080/02688697.2020.1765973DOI Listing

Longitudinal Swept Source OCT Angiography of Juxtapapillary Retinal Capillary Hemangioblastoma.

Ophthalmol Retina 2020 May 8. Epub 2020 May 8.

Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, Florida. Electronic address:

In this case report, SS-OCTA identified the key diagnostic features of JRCH seen with multimodal imaging. Serial SS-OCTA imaging showed transient decreases in vascular congestion and exudation after intravitreal anti-VEGF injections. SS-OCTA may be the sole imaging modality needed for the diagnosis of JRCH, an important entity that is commonly misdiagnosed as disc edema or choroidal neovascularization. Read More

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http://dx.doi.org/10.1016/j.oret.2020.05.002DOI Listing

Retinal capillary hemangioblastomatosis and renal tumor in Von Hippel-Lindau disease.

Am J Ophthalmol Case Rep 2020 Jun 23;18:100718. Epub 2020 Apr 23.

Roski Eye Institute, Department of Ophthalmology, Keck School of Medicine, University of Southern California, 1450 San Pablo St, Los Angeles, CA, USA.

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http://dx.doi.org/10.1016/j.ajoc.2020.100718DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7210392PMC

Spinal paraganglioma as unusual finding in von Hippel-Lindau disease.

J Clin Neurosci 2020 Jul 7;77:217-221. Epub 2020 May 7.

Department of Neurosurgery, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Germany.

A 20-year-old patient with a history of von Hippel-Lindau disease reported on thoracic back pain radiating to the left shoulder for 10 weeks. Magnetic resonance imaging revealed a progressive contrast-enhancing tumor (14 × 21 × 28 mm) compressing the spinal cord and extending into the left neural foramen at T5/6. After embolization of supplying vessels, the tumor was completely resected via hemilaminectomy of T5. Read More

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http://dx.doi.org/10.1016/j.jocn.2020.05.025DOI Listing

Molecular Advances in Central Nervous System Mesenchymal Tumors.

Surg Pathol Clin 2020 Jun;13(2):291-303

Center for Skull Base and Pituitary Surgery, Department of Neurosurgery, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA. Electronic address:

Mesenchymal tumors of the central nervous system (CNS) comprise an array of neoplasms that may arise from or secondarily affect the CNS and its immediate surroundings. This review focuses on meningiomas and solitary fibrous tumors, the most common primary CNS mesenchymal tumors, and discusses recent advances in unveiling the molecular landscapes of these neoplasms. An effort is made to underscore those molecular findings most relevant to tumor diagnostics and prognostication from a practical perspective. Read More

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http://dx.doi.org/10.1016/j.path.2020.02.002DOI Listing

Role of VHL-JAK-STAT signaling pathway in central nervous system hemangioblastoma associated with von Hippel-Lindau disease.

J Neurooncol 2020 May 30;148(1):29-38. Epub 2020 Apr 30.

Department of Urology, Yokohama City University Graduate School of Medicine, Yokohama, Kanagawa, Japan.

Introduction: Central nervous system hemangioblastoma is a benign tumor associated with or without von Hippel-Lindau (VHL) disease which is an autosomal dominant hereditary disease that results from a germline mutation in the VHL gene. A main axis of signaling pathways in central nervous system hemangioblastoma is VHL-HIF signaling pathway. Here, we propose an alternative VHL-JAK-STAT signaling pathway in hemangioblastoma and discuss the role. Read More

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http://dx.doi.org/10.1007/s11060-020-03506-8DOI Listing

Safety and efficacy of surgical treatment for brainstem hemangioblastoma: a meta-analysis.

Neurosurg Rev 2020 Apr 30. Epub 2020 Apr 30.

Department of Neurosurgery, Peking University First Hospital, Peking University, No. 8 Xishiku Street, Xicheng District, Beijing, China.

Brainstem hemangioblastomas are benign, highly vascular tumors located in the mesencephalon, pons, and medulla oblongata. Although surgical resection is currently considered the main therapeutic option for symptomatic lesions, evidence supporting the application of microsurgery has not been systematically assessed. This meta-analysis aims to evaluate the safety and efficacy of surgical treatment for brainstem hemangioblastomas. Read More

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http://dx.doi.org/10.1007/s10143-020-01305-3DOI Listing

Pearls and Pitfalls in Interpretation of 68Ga-DOTATOC PET Imaging.

Clin Nucl Med 2020 Jun;45(6):e279-e280

From the Departments of Clinical Physiology, Nuclear Medicine, and PET.

DOTA-D-Phe-Tyr-octreotide labeled with Ga (Ga-DOTATOC) is the commonly used PET tracer for imaging meningioma because of its high affinity to somatostatin receptor subtype 2 (SSTR2) and an established imaging modality for planning radiation and radionuclide therapy. However, SSTR2 is not an exclusive marker for meningioma, and not all meningiomas express high levels of SSTR2. The SSTR2 expression has been reported in other intracranial tumors, for example, glioma, pituitary adenoma, medullablastoma, primitive neuroectodermal tumors, and hemangioblastoma leading to a significant risk of misinterpretation of PET/CT findings. Read More

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http://dx.doi.org/10.1097/RLU.0000000000003012DOI Listing

Detection of VHL deletion by fluorescence in situ hybridization in extraneuraxial hemangioblastoma of soft tissue.

Pathol Int 2020 Apr 17. Epub 2020 Apr 17.

Department of Surgical Pathology, School of Medicine, Sapporo Medical University, Hokkaido, Japan.

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http://dx.doi.org/10.1111/pin.12935DOI Listing

Novel genetic characterisation and phenotype correlation in von Hippel-Lindau (VHL) disease based on the Elongin C binding site: a large retrospective study.

J Med Genet 2020 Apr 17. Epub 2020 Apr 17.

Department of Urology, Peking University First Hospital, Beijing, Beijing, China

Background: Von Hippel-Lindau (VHL) disease is an autosomal dominant genetic tumour syndrome resulting from mutations in the gene lineage, and its prognosis is generally poor. This study aimed to provide a more valuable genotype-phenotype correlation based on the Elongin C binding site in VHL disease.

Methods: This study included 553 patients (194 families) who were diagnosed with VHL disease in our centre from September 2010 to February 2019. Read More

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http://dx.doi.org/10.1136/jmedgenet-2019-106336DOI Listing
April 2020
6.335 Impact Factor

Percutaneous embolization of sporadic lumbar nerve root haemangioblastoma under local anaesthesia.

BJR Case Rep 2020 Mar 12;6(1):20190037. Epub 2020 Feb 12.

Department of Imaging and Interventional Radiology, Prince of Wales Hospital, Hong Kong, China.

Pre-operative embolization of spinal tumours are mainly performed using a transarterial approach. Percutaneous embolization of spinal tumours are undertaken much less frequently, though its use has been reported in hypervascular spinal metastases and spinal paraganglioma. We present a patient in whom pre-operative percutaneous embolization has been performed to a recurrent lumbar nerve root haemangioblastoma that had previously been embolized using a transarterial approach. Read More

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http://dx.doi.org/10.1259/bjrcr.20190037DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7068088PMC

Glaucoma as the presenting sign of intraocular tumors: beware of the masquerading sign.

Int Ophthalmol 2020 Jul 20;40(7):1789-1795. Epub 2020 Mar 20.

Ophthalmic Plastic Surgery and Ocular Oncology Services, The Operation Eyesight Universal Institute for Eye Cancer, LV Prasad Eye Institute, Hyderabad, 500034, India.

Purpose: To discuss the clinical presentation and management of intraocular tumors masquerading as primary glaucoma or non-tumor-related secondary glaucoma.

Methods: Retrospective chart review.

Results: Ten patients with unsuspected intraocular tumor were referred to glaucoma clinic with a diagnosis of primary glaucoma or non-tumor-related secondary glaucoma. Read More

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http://dx.doi.org/10.1007/s10792-020-01348-xDOI Listing

Establishment and Characterization of Cell Lines from Primary Culture of Hemangioblastoma Stromal Cells.

Neurol India 2020 Mar-Apr;68(2):383-388

Department of Neurosurgery, West China Hospital of Sichuan University, Chengdu, Sichuan, China.

Context: A well-established cell line of hemangioblastomas (HBs) is still lacking.

Aim: This study aims to explore a stable way to establish primary cell lines of HB stromal cells and investigate the morphological and molecular features of these cells.

Patients And Methods: Specimens of HBs from 13 patients were collected for establishment of primary cell lines of stromal cells. Read More

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http://dx.doi.org/10.4103/0028-3886.280643DOI Listing

[The role of SMARCE1 in the diagnosis of clear cell meningioma].

Zhonghua Bing Li Xue Za Zhi 2020 Mar;49(3):234-238

Department of Pathology, the First Affiliated Hospital of Fujian Medical University, Fuzhou 350005, China.

To analyze the expression of SMARCE1 in clear cell meningioma (CCM), and evaluate the role of SMARCE1 in the differential diagnosis in morphologically similar diseases. Thirteen samples/11 cases of CCMs were collected from the First Affiliated Hospital of Fujian Medical University, Shandong Provincial Hospital, Xuanwu Hospital of Capital Medical University and Thaihe Hospital of Hubei Province from January 2000 to December 2018, as well as 17 cases of meningiomas with clear-cell-like morphology, 782 cases of other types of meningiomas and other intracranial tumors with clear-like morphology. A tissue microarray was made using these cases, on which immunohistochemical/histochemical staining of SMARCE1, SSTR2, EMA, Ki-67, p53, PAS and D-PAS were performed. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0529-5807.2020.03.005DOI Listing

Novel and recurrent germline mutations in the VHL gene in 5 Arab patients with Von Hippel-Lindau disease.

Cancer Genet 2020 May 6;243:1-6. Epub 2020 Mar 6.

Genomics, Ontario Institute for Cancer Research, Toronto, Canada. Electronic address:

Inherited germline mutations in the VHL gene cause predisposition to Von Hippel-Lindau (VHL) disease. Patients exhibit benign and cancerous lesions in multiple tissues, including hemangioblastomas, clear cell renal cell carcinoma, cysts in kidneys and pancreas, and pheochromocytomas. Although pathogenic germline mutations in the VHL gene have been widely described in different populations, only a single mutation was previously reported in a family from mixed Arab-Persian ethnicity. Read More

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http://dx.doi.org/10.1016/j.cancergen.2020.02.006DOI Listing

Preoperative embolization of suprasellar hemangioblastoma supplied by artery of foramen rotundum: a case report and review of the literature.

Br J Neurosurg 2020 Mar 12:1-3. Epub 2020 Mar 12.

Department of Neurosurgery, Kyungpook National University Hospital, Daegu, Republic of Korea.

We present the case of 69-year-old woman who underwent preoperative embolization of a suprasellar hemangioblastoma supplied by the artery of foramen rotundum. To our best knowledge, this is the first such report in English. We review the literature focusing on feeding arteries of sellar and suprasellar hemangioblastomas. Read More

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http://dx.doi.org/10.1080/02688697.2020.1738341DOI Listing

Surgical Management of Intramedullary Spinal Cord Tumors.

Neurosurg Clin N Am 2020 Apr 31;31(2):237-249. Epub 2020 Jan 31.

Department of Neurological Surgery, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, NY 10065, USA; Department of Neurological Surgery, Weill Cornell Medical College, 525 E. 68th St, New York, NY 10065, USA.

Intramedullary spinal cord tumors (IMSCT) comprise a rare subset of CNS tumors that have distinct management strategies based on histopathology. These tumors often present challenges in regards to optimal timing for surgery, invasiveness, and recurrence. Advances in microsurgical techniques and technological adjuncts have improved extent of resection and outcomes with IMSCT. Read More

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http://dx.doi.org/10.1016/j.nec.2019.12.004DOI Listing

Hemangioblastoma of the Cerebellopontine Angle Evaluated with Pseudocontinuous Arterial Spin Labeling.

Magn Reson Med Sci 2020 Mar 4. Epub 2020 Mar 4.

Department of Radiology, Mie University School of Medicine.

Hemangioblastomas of the cerebellopontine angle (CPA) that emerge extra-axially from the peripheral nervous system are extremely rare. We report a case of hemangioblastoma of the CPA evaluated by pseudocontinuous arterial spin labeling (pCASL). The high rate of tumor blood flow determined using pCASL provided additional useful information for the differential diagnosis of the CPA tumors in this patient. Read More

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http://dx.doi.org/10.2463/mrms.ici.2020-0011DOI Listing

von Hippel-Lindau Syndrome: Genetic Study of Case With a Rare Pathogenic Variant With Optic Nerve Hemangioblastoma, a Rare Phenotypic Expression.

Front Oncol 2020 14;10:139. Epub 2020 Feb 14.

Department of Surgery, Faculdade De Medicina Do ABC, Santo André, Brazil.

von Hippel-Lindau syndrome (VHLS) is a rare, autosomal dominant genetic disease with high penetrance and variable phenotypic expression caused by variants in the gene. VHLS is associated with the presence of vascular tumors, often hemangioblastoma of the central nervous system, retina, or spinal cord and, less frequently, pancreatic cystic neoplasm, pancreatic neuroendocrine tumor, clear cell carcinoma of the kidney, endolymphatic sac tumor, pheochromocytoma, and paraganglioma. The authors report a case of a patient with VHLS with a rare pathogenic variant in the gene and with an optic nerve hemangioblastoma, a rare phenotypic expression. Read More

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http://dx.doi.org/10.3389/fonc.2020.00139DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7033541PMC
February 2020

Machine Learning Decision Tree Models for Differentiation of Posterior Fossa Tumors Using Diffusion Histogram Analysis and Structural MRI Findings.

Front Oncol 2020 7;10:71. Epub 2020 Feb 7.

Department of Radiology and Biomedical Imaging, University of California, San Francisco, San Francisco, CA, United States.

We applied machine learning algorithms for differentiation of posterior fossa tumors using apparent diffusion coefficient (ADC) histogram analysis and structural MRI findings. A total of 256 patients with intra-axial posterior fossa tumors were identified, of whom 248 were included in machine learning analysis, with at least 6 representative subjects per each tumor pathology. The ADC histograms of solid components of tumors, structural MRI findings, and patients' age were applied to construct decision models using Classification and Regression Tree analysis. Read More

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http://dx.doi.org/10.3389/fonc.2020.00071DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7018938PMC
February 2020

Case report: a synonymous VHL mutation (c.414A > G, p.Pro138Pro) causes pathogenic familial hemangioblastoma through dysregulated splicing.

BMC Med Genet 2020 02 27;21(1):42. Epub 2020 Feb 27.

Boler-Parseghian Center for Rare and Neglected Diseases, University of Notre Dame, Notre Dame, IN, 46556, USA.

Background: von Hippel-Lindau (VHL) disease is a familial neoplasia syndrome that results from the germline mutation of VHL. Pathogenic VHL mutations include deletion, frameshift, nonsense and missense mutations. Synonymous mutations are expected to be phenotypically silent and their role in VHL disease remains poorly understood. Read More

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http://dx.doi.org/10.1186/s12881-020-0976-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7045488PMC
February 2020
2.083 Impact Factor

Von Hippel-Lindau Disease and the Eye.

J Ophthalmic Vis Res 2020 Jan-Mar;15(1):78-94. Epub 2020 Feb 2.

Ophthalmic Epidemiology Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Retinal hemangioblastoma (also referred to as retinal capillary hemangioma) is a benign lesion originating from the endothelial and glial components of the neurosensory retina and optic nerve head. Historically known as a manifestation of the von Hippel-Lindau (VHL) disease, it can be seen as an isolated finding or in association with some rare ocular conditions. In addition to characteristic ophthalmoscopic features, results of numerous ancillary tests including angiography, ultrasound, optical coherence tomography, and genetic tests may support the diagnosis and differentiate it from similar conditions. Read More

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http://dx.doi.org/10.18502/jovr.v15i1.5950DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7001024PMC
February 2020

Quantitative assessment and clinical relevance of VEGFRs-positive tumor cells in refractory brain tumors.

Exp Mol Pathol 2020 Jun 21;114:104408. Epub 2020 Feb 21.

Department of Neurosurgery, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan. Electronic address:

Vascular endothelial growth factor (VEGF)/VEGF receptor (VEGFR)1 and 2 signaling is a potent activator of tumor angiogenesis. Although the expressions of VEGFR1 and VEGFR2 were initially thought to be limited to the endothelial cells, it is now known that both the receptors are expressed in tumor cells. This is the first study wherein VEGFRs-positive tumor cells are quantitatively evaluated for brain tumors with upregulated VEGF/VEGFR signaling. Read More

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http://dx.doi.org/10.1016/j.yexmp.2020.104408DOI Listing

Clinically Actionable Insights into Initial and Matched Recurrent Glioblastomas to Inform Novel Treatment Approaches.

J Oncol 2019 31;2019:4878547. Epub 2019 Dec 31.

Brain Tumour Research Centre, University of Bristol, Bristol, UK.

Glioblastoma is the most common primary adult brain tumour, and despite optimal treatment, the median survival is 12-15 months. Patients with matched recurrent glioblastomas were investigated to try to find actionable mutations. Tumours were profiled using a validated DNA-based gene panel. Read More

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http://dx.doi.org/10.1155/2019/4878547DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7012245PMC
December 2019

A Large Solitary Hemangioblastoma of the Lateral Ventricles: A Case Report and Literature Review.

Iran J Med Sci 2020 Jan;45(1):67-72

Department of Neurosurgery, School of Medicine, Iran University of Medical Sciences, Tehran, Iran.

Hemangioblastoma (HB) in the supratentorial region of the brain is rare and only a few cases are reported on intraventricular HB. HB of the lateral ventricles is even rarer. We present a case of a 30-year-old man with generalized tonic clonic seizures. Read More

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http://dx.doi.org/10.30476/ijms.2019.81095DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6983274PMC
January 2020

Effect of Laryngeal Suspension and Upper Esophageal Sphincter Myotomy for Severe Dysphagia Due to Brainstem Disease.

Ann Otol Rhinol Laryngol 2020 Jul 10;129(7):689-694. Epub 2020 Feb 10.

Department of Otolaryngology, School of Medicine, Fujita Health University, Toyoake, Aichi, Japan.

Objectives: Surgical procedure is considered in patients with severe dysphagia when conservative treatment fails. This study aimed to evaluate laryngeal suspension (LS) and upper esophageal sphincter (UES) myotomy for treating severe dysphagia due to brain disease.

Methods: Fourteen patients underwent LS and UES myotomy, with a median follow-up of 5 years and 6 months when conservative treatment failed. Read More

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http://dx.doi.org/10.1177/0003489420904741DOI Listing

Cystic-solid hemangioblastoma at the cerebellopontine angle: A case report.

Medicine (Baltimore) 2020 Jan;99(3):e18871

Department of Neurosurgery, West China Hospital, Sichuan University, Chengdu, Sichuan, P. R. China.

Rationale: Hemangioblastomas (HMGs) originating from the cerebellopontine angle (CPA) are extremely uncommon. Nevertheless, the cystic-solid form of this lesion at the above location is even rarer.

Patient Concerns: We present a 31-years old male with a right ear hearing loss of 3 months duration. Read More

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http://dx.doi.org/10.1097/MD.0000000000018871DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7220448PMC
January 2020
5.723 Impact Factor

Treatment of Capillary Hemangioblastoma in Von Hippel-Lindau Disease: Case Report and Online Survey of Treatment Preferences among Experts.

Case Rep Ophthalmol 2020 Jan-Apr;11(1):37-47. Epub 2020 Jan 10.

Retina and Vitreous Department, Asociación para Evitar la Ceguera en México IAP, Hospital Dr. Luis Sánchez Bulnes, Mexico City, Mexico.

This case report aims to describe the diagnosis, treatment, and evolution of bilateral, asymmetrical retinal capillary hemangioblastomas treated with argon laser and intravitreal anti-vascular endothelial growth factor and also reports the results of an online survey of treatment preferences among retina and vitreous specialists. A previously healthy 23-year-old female presented to our Retina Department complaining of progressive visual loss in her right eye. Visual acuity at admission was 20/300 in her right eye and 20/20 in her left eye. Read More

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http://dx.doi.org/10.1159/000504430DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6984148PMC
January 2020

Adherence of US Insurance Payer Policies to the American Society of Radiation Oncology Stereotactic Radiosurgery Model Policy.

Pract Radiat Oncol 2020 Jan 28. Epub 2020 Jan 28.

Department of Radiation Oncology, Washington University School of Medicine, St Louis, Missouri. Electronic address:

Purpose: The medical necessity of stereotactic radiosurgery (SRS) is nonuniform across insurance policies. The American Society for Radiation Oncology (ASTRO) created a model policy based on the consensus of the radiation oncology community to communicate medically necessary indications for SRS. We compared the current insurance policies for SRS with those of the ASTRO model policy. Read More

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http://dx.doi.org/10.1016/j.prro.2020.01.006DOI Listing
January 2020

Retinal haemangioblastomas in von Hippel-Lindau germline mutation carriers: progression, complications and treatment outcome.

Acta Ophthalmol 2020 Jan 30. Epub 2020 Jan 30.

Department of Ophthalmology, Erasmus Medical Centre, Rotterdam, the Netherlands.

Purpose: Evaluation of phenotype and treatment outcome of retinal haemangioblastomas (RH) in von Hippel-Lindau (VHL) disease and correlation of these features with the genotype of VHL germline mutation carriers.

Methods: Retrospective analysis of a longitudinal cohort of 21 VHL germline mutation carriers and RH. Clinical and genetic data were obtained to analyse the correlation of genotype with phenotype and treatment outcomes. Read More

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http://dx.doi.org/10.1111/aos.14360DOI Listing
January 2020

[Retinal laser treatment-avoiding mistakes].

Ophthalmologe 2020 Feb;117(2):169-188

Universitäts-Augenklinik, Göttingen, Deutschland.

Even in the era of intravitreal injection therapy (intravitreal operative injection of medication, IVOM) for the treatment of macular and retinal diseases, such as age-related macular degeneration (AMD), proliferative diabetic retinopathy (DR) and diabetic macular edema (DME) as well as proliferative stages and/or macular edema due to retinal vein occlusion (RVO), conventional retinal laser treatment is still of importance. It can be focally performed on an on-label basis for DME and macular edema due to branch RVO (BRVO) and its use as panretinal treatment for proliferative stages in retinal diseases as well as for the treatment of retinal holes is undisputed. The spectrum is extended by the treatment of less common diseases, such as retinal hemangioblastoma, macroaneurysms and subhyaloid macular hemorrhage. Read More

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http://dx.doi.org/10.1007/s00347-019-01035-yDOI Listing
February 2020

MASSIVE RETINAL NEOVASCULARIZATION IN VON HIPPEL-LINDAU DISEASE: ANTI-VASCULAR ENDOTHELIAL GROWTH FACTOR, VITRECTOMY, IMMUNOHISTOCHEMISTRY, AND OPTICAL COHERENCE TOMOGRAPHY FEATURES.

Retin Cases Brief Rep 2020 Jan 17. Epub 2020 Jan 17.

Clinical Hospital of the University of São Paulo (HCFMUSP), São Paulo, Brazil.

Purpose: To report a rare presentation of ocular von Hippel-Lindau disease associated with a massive epiretinal vascular proliferation over the macula removed by vitrectomy and submitted to histological analysis.

Methods: Interventional case report.

Results: A 13-year-old woman with von Hippel-Lindau disease reported progressive visual loss in the right eye over the preceding 6 months. Read More

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http://dx.doi.org/10.1097/ICB.0000000000000960DOI Listing
January 2020

Microsurgical resection of a radicular hemangioblastoma with subarachnoid hemorrhage: how I do it.

Acta Neurochir (Wien) 2020 04 20;162(4):957-959. Epub 2020 Jan 20.

Assistance Publique, Hôpitaux de Paris, Centre Hospitalier Universitaire de Kremlin-Bicêtre, Service de Neurochirurgie, Le Kremlin-Bicêtre, France.

Background: Hemangioblastomas (HBL) are benign tumors occurring sporadically or associated with Von Hippel-Lindau syndrome (VHL).

Method: We present the pre-, per-, and postoperative course of a rare case with radicular HBL presenting with subarachnoid hemorrhage (SAH) in the frame of VHL. We describe the microsurgical approach. Read More

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http://dx.doi.org/10.1007/s00701-020-04229-5DOI Listing

Microsurgical treatment of cervical spinal hemangioblastoma.

Neurochirurgie 2020 Feb 14;66(1):56-60. Epub 2020 Jan 14.

Department of Neurosurgery, Wuxi Second Hospital Affiliated to Nanjing Medical University, 68 Zhongshan Road, Wuxi 214002, Jiangsu, China. Electronic address:

Background: Hemangioblastoma is a rare intracranial vascular tumor, mostly found in the cerebellum. Cervical spinal hemangioblastoma (CSHB) has rarely been reported, and microsurgical treatment even less.

Objective: To present our experience with CSHB and conduct a literature review, in order to provide a systematic approach to diagnosis and microsurgical treatment. Read More

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http://dx.doi.org/10.1016/j.neuchi.2019.11.005DOI Listing
February 2020

Differentiating dural metastases from meningioma: role of 68Ga DOTA-NOC PET/CT.

Nucl Med Commun 2020 Apr;41(4):356-362

Departments of Nuclear Medicine and Molecular Imaging.

Objective: To assess the ability of Ga DOTA-NOC PET/computed tomography (CT) to differentiate dural metastases from meningioma.

Patients And Methods: Patients who underwent a Ga DOTA-NOC PET/CT for differentiating meningiomas from dural metastases were included in the study. A visual score was assigned to the dural lesions (visual score - 1 to 3) in relation to the uptake in liver and spleen and variation in the visual score was evaluated. Read More

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http://dx.doi.org/10.1097/MNM.0000000000001155DOI Listing

Surgical management of spinal intramedullary tumors: Ten-year experience in a single institution.

J Clin Neurosci 2020 Mar 10;73:201-208. Epub 2020 Jan 10.

Department of Neurosurgery, Cerrahpasa Medical Faculty, Istanbul University-Cerrahpasa, Istanbul, Turkey.

Despite their rare occurrence, intramedullary spinal cord tumors can cause considerable morbidity and mortality without treatment. Timing of surgery, extent of resection and selection of favorable treatment option are important considerations for a good surgical outcome. In this clinical study, we report our patient series and convey our treatment strategy. Read More

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http://dx.doi.org/10.1016/j.jocn.2019.12.054DOI Listing

Retinal capillary hemangioblastoma and hemiretinal vein occlusion in a patient with primary congenital glaucoma: A case report.

Saudi J Ophthalmol 2019 Oct-Dec;33(4):401-404. Epub 2018 Dec 8.

Vitreoretinal Division, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia.

The presence of retinal capillary hemangioblastoma and cerebellar hemangioblastoma in the context of Von Hippel-Lindau syndrome (VHL) is not characteristically associated with other ophthalmologic conditions. Here, we report the case of a 22-yearold female with a history of bilateral primary congenital glaucoma who presented with a right juxtapapillary retinal capillary hemangioblastoma and an old hemiretinal vein occlusion in which the retinal capillary hemangioblastoma was likely the contributing factor. Her systemic work up was positive for VHL syndrome and revealed the presence of a fatal large brainstem hemangioblastoma. Read More

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http://dx.doi.org/10.1016/j.sjopt.2018.11.008DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6950941PMC
December 2018

Hemangioblastoma-like Clear Cell Stromal Tumor of the Lung: A Clinicopathologic and Immunohistochemical Study of 5 Cases.

Am J Surg Pathol 2020 Jun;44(6):771-775

Department of Pathology, The University of Texas, MD Anderson Cancer Center, Houston, TX.

Five cases of an unusual primary benign stromal tumor designated as hemangioblastoma-like clear cell stromal tumor of the lung are presented. The patients are 4 women and 1 man between the ages of 39 and 52 years of age (average: 45.5 y). Read More

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http://dx.doi.org/10.1097/PAS.0000000000001429DOI Listing

Combined Microsurgery and Radiotherapy for Multiple Spinal Cord Hemangioblastomas with Holocord Syrinx in von Hippel-Lindau Disease: A Case Report.

J Neurol Surg Rep 2019 Oct 31;80(4):e46-e50. Epub 2019 Dec 31.

Department of Neurosurgery, University of Leipzig, Leipzig, Germany.

Spinal and cerebellar hemangioblastomas are common in von Hippel-Lindau disease (vHLD) and usually treated surgically. Multifocal presence and surgically not amenable locations are issues that require a combined microsurgical and radiosurgical approach to control complex cases. We would like to present the case of a 37-year-old male patient who was diagnosed vHLD with multiple spinal and one infratentorial hemangioblastomas and holocord syrinx formation of the whole spinal cord. Read More

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http://dx.doi.org/10.1055/s-0039-3401808DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6938460PMC
October 2019