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Cureus 2022 May 18;14(5):e25125. Epub 2022 May 18.

Department of Neurosurgery, Inova Neuroscience and Spine Institute, Fairfax, USA.

Hemangioblastomas are uncommon in the spine, accounting for less than 3% of all spinal cord tumors and occurring even more rarely in the intradural extramedullary vicinity. We present a unique case report of an intradural extramedullary hemangioblastoma. A 62-year-old man presented with a five-month history of neck pain radiating to the left arm. Read More

J Neuroimaging 2022 Jun 22. Epub 2022 Jun 22.

Department of Radiology, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan.

Background And Purpose: Differentiation between hemangioblastoma and brain metastasis remains a challenge in neuroradiology using conventional MRI. Amide proton transfer (APT) imaging can provide unique molecular information. This study aimed to evaluate the usefulness of APT imaging in differentiating hemangioblastomas from brain metastases and compare APT imaging with diffusion-weighted imaging and dynamic susceptibility contrast perfusion-weighted imaging. Read More

World Neurosurg 2022 Jun 13. Epub 2022 Jun 13.

Department of Neurosurgery, China International Neuroscience Institute, Xuanwu Hospital, Capital Medical University, Beijing, China. Electronic address:

Eur J Med Genet 2022 Jun 13;65(8):104538. Epub 2022 Jun 13.

Department of Clinical Genetics, Odense University Hospital, Odense, Denmark; Department of Clinical Research, University of Southern Denmark, Odense, Denmark.

von Hippel Lindau disease (vHL) is caused by a hereditary predisposition to multiple neoplasms, especially hemangioblastomas in the retina and CNS, renal cell carcinomas (RCC), pheochromocytomas, neuroendocrine pancreatic tumours (PNET) and endolymphatic sac tumours. Evidence based approaches are needed to ensure an optimal clinical care, while minimizing the burden for the patients and their families. This guideline is based on evidence from the international vHL literature and extensive research of geno- and phenotypic characteristics, disease progression and surveillance effect in the national Danish vHL cohort. Read More

Clin Nucl Med 2022 Jun 10. Epub 2022 Jun 10.

From the Department of Nuclear Medicine, Marmara University Pendik Training and Research Hospital, Istanbul, Turkey.

Abstract: Von Hippel-Lindau (VHL) disease is an autosomal dominant hereditary cancer syndrome that is characterized by the presence of various malignant and benign tumors, including retinal and central nervous system hemangioblastomas. Hemangioblastomas are highly vascular tumors that can occur sporadically or within VHL disease. Herein, we present 68Ga-DOTATATE PET/CT findings of a unique case of suprasellar hemangioblastoma in a 52-year-old man with VHL disease. Read More

Front Oncol 2022 16;12:903655. Epub 2022 May 16.

Department of Radiology, The Second Affiliated Hospital of Zun Yi Medical University, Zunyi, China.

Myopericytoma (MPC) is a benign soft tissue tumor that develops from perivascular myoid cells and is part of the perivascular tumor group. MPC most commonly occurs in the subcutaneous soft tissues of the extremities, while intracranial MPC is remarkably rare. Herein, we report the case of a 45-year-old woman with myopericytoma who had a 2-week history of recurrent dizziness. Read More

Cureus 2022 Apr 27;14(4):e24527. Epub 2022 Apr 27.

Neuropathology, Instituto Nacional de Neurología y Neurocirugia, Mexico City, MEX.

Hemangioblastoma is considered a benign neoplasm characterized by abnormal vasculature and stromal cells; several pathophysiological mechanisms have been proposed, such as genetic predisposition, hormonal factors, and arterial wall ischemia. Fibromuscular dysplasia is characterized by hyperplasia or thinning of the smooth muscle, elastic fibre destruction, fibrous tissue proliferation, and arterial wall disorganization. We present a cerebellar hemangioblastoma case not associated with Von Hippel Lindau syndrome. Read More

Int J Surg Pathol 2022 Jun 1:10668969221102560. Epub 2022 Jun 1.

12285Geisel School of Medicine at Dartmouth, Hanover, NH, USA.

Hemangioblastoma, one of the characteristic tumors associated with Von Hippel-Lindau (VHL) disease, most often presents in the central nervous system (CNS) but can uncommonly arise in extraneuraxial, or previously referred to as peripheral, locations. Without the clinical context of known VHL disease, hemangioblastoma may not enter the differential for a soft tissue mass outside the CNS. Here, we present two patients with diagnostically challenging extraneuraxial hemangioblastoma to highlight the importance of considering this entity within the differential diagnosis of soft tissue neoplasms containing clear cells and delicate vasculature. Read More

Lancet 2022 05;399(10340):2065

Division of Neurosurgery, University of Connecticut Health Center, UConn Health, Farmington, CT, USA. Electronic address:

Brain Pathol 2022 May 30:e13083. Epub 2022 May 30.

Institute of Neuropathology, University Hospital Münster, Münster, Germany.

Hemangioblastomas (HBs) of the central nervous system are highly vascular neoplasms that occur sporadically or as a manifestation of von Hippel-Lindau (VHL) disease. Despite their benign nature, HBs are clinically heterogeneous and can be associated with significant morbidity due to mass effects of peritumoral cysts or tumor progression. Underlying molecular factors involved in HB tumor biology remain elusive. Read More

Surg Neurol Int 2021 21;12:296. Epub 2021 Jun 21.

Departments of Neurosurgery and Radiology, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, New York, United States.

Background: Hemangioblastomas are benign (World Health Organization Grade I), highly vascular neoplasms commonly associated with Von Hippel-Lindau (VHL) disease.[2] The VHL tumor-suppressor gene, located on chromosome 3, is implicated in sporadic cases and cases associated with VHL disease. Hemangioblastomas most commonly arise in the posterior fossa; however, they may also be found supratentorially or within the spinal cord. Read More

Gastroenterology 2022 May 14. Epub 2022 May 14.

Université de Paris-Cité, Centre of Research on Inflammation, INSERM U1149, Paris, France; Université de Paris-Cité, Department of Digestive Endoscopy, Beaujon University Hospital (APHP), Clichy, France.

Cancer 2022 May 17. Epub 2022 May 17.

Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, the Netherlands.

Background: Clinical pathways are care plans established to describe essential steps in the care of patients with a specific clinical problem. They translate (inter)national guidelines into local applicable protocols and clinical practice. The purpose of this article is to establish a multidisciplinary integrated care pathway for specialists and allied health care professionals in caring for individuals with von Hippel-Lindau (VHL) disease. Read More

Neurospine 2022 May 16. Epub 2022 May 16.

Department of Neurosurgery, Nagoya University School of Medicine, Nagoya, Japan.

The study of genetic alterations and molecular biology in central nervous system (CNS) tumors has improved the accuracy of estimations of patient prognosis and tumor categorization. Therefore, the updated 2021 World Health Organization (WHO) classification includes various diagnostic genes, molecules, and pathways for diagnosis, as well as histological findings. These findings are expected both to have diagnostic applications and to facilitate new targeted therapies that target tumor-specific genetic changes and molecular biology. Read More

Front Oncol 2022 27;12:859157. Epub 2022 Apr 27.

Department of Oncology, The First Affiliated Hospital of Nanjing Medical University, Nanjing, China.

Background: Hemangioblastoma (HB) is a rare and highly vascularized tumor that originates from the central nervous system as well as other part of the body. They can appear sporadically or as part of von Hippel-Lindau (VHL) disease, a rare hereditary cancer syndrome. Although surgery can cure the majority of HBs, the disease shows a treatment-refractory challenge upon recurrence. Read More

Cureus 2022 Apr 13;14(4):e24099. Epub 2022 Apr 13.

Clinic of Neurosurgery, Sv. Georgi Hospital, Plovdiv, BGR.

Sporadic spinal extramedullary hemangioblastomas of the conus medullaris are extremely rare. We present the case of a 40-year-old male with symptoms of severe back pain and monoradiculopathy. The magnetic resonance imaging (MRI) revealed an intradural extramedullary tumor attached to the conus medullaris. Read More

Ophthalmol Retina 2022 May;6(5):410

Sankara Eye Hospital, Bangalore, India.

BMC Ophthalmol 2022 May 6;22(1):205. Epub 2022 May 6.

Department of Ophthalmology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, 450000, China.

Background: To report the multimodal imaging and treatment of fifteen retinal capillary hemangioblastomas (RCHs) associated with Von Hippel-Lindau syndrome in a monocular patient during a long-term following-up, which supply high-resolution exquisite SS-OCTA images (VG200; SVision Imaging, Ltd., Luoyang, China) and management experience about multiple RCHs.

Case Presentation: A 34-year-old monocular male patient complained decreased visual acuity (20/100) without pain and redness in the left eye five years ago. Read More

Front Med (Lausanne) 2022 18;9:836012. Epub 2022 Apr 18.

Department of Medical Oncology, Affiliated Dongyang Hospital of Wenzhou Medical University, Dongyang, China.

Background: Hemangioblastoma-like clear cell stromal tumor (HLCCST) is a recently reported neoplasm of the lung. Only 13 cases have been reported in four recent studies. Because HLCCST is very rare, it has not been included in the 2021 WHO classification of lung tumors. Read More

Surg Neurol Int 2022 29;13:174. Epub 2022 Apr 29.

Department of Neurosurgery, Fukuoka University, Fukuoka, Japan.

Background: Cerebellar hemangioblastoma is a highly vascular benign tumor and the growth rate of hemangioblastomas is believed to often accelerate during pregnancy; however, the reason for this rapid increase in size remains poorly understood. There are several case reports of symptomatic hemangioblastoma during pregnancy; however, the favorable management strategy has not been well established.

Case Description: A 35-year-old woman, gravida 2 para 1, with no significant medical history presented with vertigo and difficulty walking at around 11 weeks of pregnancy and was referred to our institute at 30 weeks of gestation because of worsening symptoms. Read More

Pediatr Neurosurg 2022 May 4. Epub 2022 May 4.

Introduction: The benefits of performing open and endovascular procedures in a hybrid neuroangiography surgical suite include confirmation of treatment results and reduction in number of procedures, leading to improved efficiency of care. Combined procedural suites are infrequently used in pediatric facilities due to technical and logistical limitations. We report the safety, utility and lessons learned from a single-institution experience using a hybrid suite equipped with biplane rotational digital subtraction angiography and pan-surgical capabilities. Read More

J Pathol Transl Med 2022 May 3;56(3):115-125. Epub 2022 May 3.

Department of Laboratory Medicine and Pathology, Division of Neuropathology, University of Washington, Seattle, WA, USA.

Hemangioblastoma is a benign, highly vascularized neoplasm of the central nervous system (CNS). This tumor is associated with loss of function of the VHL gene and demonstrates frequent occurrence in von Hippel-Lindau (VHL) disease. While this entity is designated CNS World Health Organization grade 1, due to its predilection for the cerebellum, brainstem, and spinal cord, it is still an important cause of morbidity and mortality in affected patients. Read More

Rep Pract Oncol Radiother 2022 22;27(1):134-141. Epub 2022 Mar 22.

Department of Oncology, General University Hospital in Prague, Prague, Czech Republic.

Background: This retrospective analysis evaluated the long-term outcome of spinal stereotactic body radiotherapy (SBRT) treatment for hemangioblastomas.

Materials And Methods: Between 2010 and 2018, 5 patients with 18 Von-Hippel Lindau-related pial-based spinal hemangioblastomas were treated with fractionated SBRT. After precisely registering images of all relevant datasets, we delineated the gross tumor volume, spinal cord (including intramedullary cysts and/or syrinxes), and past radiotherapy regions. Read More

Oman J Ophthalmol 2022 Jan-Apr;15(1):89-91. Epub 2022 Mar 2.

Department of Pediatric Ophthalmology, Ophthalmology Service - SAMIC Pediatric Hospital "Prof. Dr. JP Garrahan", Buenos Aires, Argentina.

A 6-year-old girl with visual impairment in the right eye (OD) was referred for an eye evaluation. The fundus of the OD showed a fibrotic orange endophytic lesion located adjacent to the optic disc. In retinal optical coherence tomography, a local tractional retinal detachment and choroidal neovascular membrane were observed together also with the presence of subretinal fluid. Read More

Neurospine 2022 Mar 31;19(1):108-117. Epub 2022 Mar 31.

Department of Neurosurgery, University Hospital of Bordeaux, Bordeaux, France.

Objective: Spinal cord tumors constitute a small part of spinal surgery owing to their rarity. This retrospective study describes their current management.

Methods: Forty-eight patients were treated for an intramedullary tumor between 2014 and 2020 at a single institution. Read More

Clin Case Rep 2022 Mar 22;10(3):e05629. Epub 2022 Mar 22.

Department of Anesthesia Indiana University School of Medicine Indianapolis Indiana USA.

von Hippel-Lindau disease (VHLD) is an autosomal dominant disorder characterized by central nervous system hemangioblastomas and renal tumors. Here, we report a case of thoracic epidural placement in a 35-year-old woman with VHLD presenting for left open heminephrectomy for renal masses. We also reviewed the literature on this topic. Read More

J Coll Physicians Surg Pak 2022 Apr;32(4):531-534

Department of Tumor Surgery, Lanzhou University Second Hospital, Lanzhou, China.

Charcot arthropathy of the shoulder joint is usually referred to as shoulder joint involvement of Charcot arthropathy, which is a chronic, degenerative, destructive condition resulting from decreasing or loss of sensorial innervation. To date, several reports have described the shoulder Charcot arthropathy, caused by malformations of the occipital region with syringomyelia; but nobody has reported the shoulder Charcot arthropathy secondary to hemangioblastoma in conus medullari with syringomyelia. Therefore, we report a 32-year male patient who was diagnosed with Charcot arthropathy of the shoulder joint, which was misdiagnosed as a soft tissue tumor and treated surgically. Read More

BMJ Case Rep 2022 Mar 23;15(3). Epub 2022 Mar 23.

Smt Kanuri Santhamma Centre for Vitreo-Retinal Diseases, LV Prasad Eye Institute, Hyderabad, Telangana, India

A man in his 20s presented to the neurosurgery department 2 years ago with headache and blurred vision. He was diagnosed to have a suprasellar mass on neuroimaging. Best-corrected visual acuity in the right eye was 6/36 and that in the left eye was 6/60. Read More

Endocrinol Diabetes Metab Case Rep 2022 Mar 1;2022. Epub 2022 Mar 1.

Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, The Netherlands.

Summary: Von Hippel-Lindau's disease (VHL) is a hereditary tumor syndrome characterized by its prototype lesions, hemangioblastomas, and renal cell carcinomas. Treatment for renal cell carcinomas can ultimately result in long-term dialysis. Pancreatic neuroendocrine tumors (pNET) can also occur in the course of the disease. Read More

Zhongguo Yi Xue Ke Xue Yuan Xue Bao 2022 02;44(1):24-29

Department of Pathology,PUMC Hospital,CAMS and PUMC,Beijing 100730,China.

Objective To analyze the disease spectrum and clinicopathological characteristics of central nervous system(CNS)diseases diagnosed based on pathological findings in Tibet. Methods We collected the data of all the cases with CNS lesions in Tibet Autonomous Region People's Hospital from January 2013 to December 2020.The clinicopathological features were analyzed via light microscopy,immunohistochemical staining,and special staining. Read More