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Front Oncol 2021 24;11:683021. Epub 2021 May 24.

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

Background: Hemangioblastoma is a benign tumor of the central nervous system and may appear as a component of von Hippel-Lindau (VHL) disease. At present, approximately 40 cases of optic nerve HGBs have been reported in the literature. VHL disease is a rare autosomal-dominant inherited cancer syndrome with different phenotypes caused by variants in the VHL gene. Read More

World Neurosurg 2021 Jun;150:213

Department of Neurosurgery, Tokyo Metropolitan Tama Medical Center, Tokyo, Japan. Electronic address:

Arch Soc Esp Oftalmol (Engl Ed) 2021 Jun 2;96(6):337-341. Epub 2020 Aug 2.

Departamento de Retina & Vítreo, Fundación Hospital "Nuestra Señora de la Luz", I.A.P., Mexico City, Mexico.

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

World Neurosurg 2021 Jun 1. Epub 2021 Jun 1.

Department of Neurological Surgery, Loyola University Medical Center, Maywood, IL; Edward Hines Jr V.A. Medical Center, Hines, IL. Electronic address:

Background: Hemangioblastomas are benign vascular neoplasms of the central nervous system that may occur sporadically or in association with Von Hippel Lindau (VHL) disease. A minority of these lesions can occur in the spine, mostly as intramedullary masses. The authors present a rare CASE of primary sporadic spinal hemangioblastoma occurring as an intradural extramedullary (IDEM) lesion. Read More

J Musculoskelet Neuronal Interact 2021 Jun;21(2):326-331

1st Physical Medicine and Rehabilitation Department, National Rehabilitation Center EKA, Athens, Greece.

Von Hippel Lindau (VHL) is a hereditary multiple neoplasia syndrome. We report a case series of two siblings with Von Hippel Lindau (VHL) disease admitted to the rehabilitation department after surgical excision of Central Nervous System (CNS) haemangioblastomas. These clinical cases present rehabilitation challenges in VHL disease. Read More

Ophthalmic Surg Lasers Imaging Retina 2021 Mar 1;52(3):165-167. Epub 2021 Mar 1.

This is a case report detailing a pars plana vitrectomy and resection of a retinal capillary hemangioblastoma in a 19-year-old female with a history of von Hippel-Lindau syndrome with secondary retinal detachment and sub-retinal exudates limiting her vision. Intraoperatively, long duration endolaser and generous endodiathermy applied to the feeder vessels are essential to minimize bleeding. Complete and careful resection of the lesion and installation of a silicone oil tamponade are recommended. Read More

Oper Neurosurg (Hagerstown) 2021 Apr 30. Epub 2021 Apr 30.

Department of Neurosurgery, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.

Pineal region tumors remain challenging lesions to safely resect because of their central location.1 Patients frequently present with symptoms associated with hydrocephalus and brainstem compression.2 Local anatomy, primarily the tentorium angle and venous anatomy, plays a central role in the selection of the approach. Read More

Clin Neuroradiol 2021 May 17. Epub 2021 May 17.

Department of Radiology, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea (Republic of).

Oper Neurosurg (Hagerstown) 2021 May 6. Epub 2021 May 6.

Department of Neurosurgery, Mayo Clinic, Phoenix, Arizona, USA.

Surg Neurol Int 2021 14;12:155. Epub 2021 Apr 14.

Department of Neurosurgery, Mayo Clinic, Scottsdale, Arizona, United States.

Background: Primary spinal tumors are rare benign lesions that represent around 2-4% of all central nervous system neoplasms.[1,2] Intradural intramedullary tumors are predominately glial in origin and are most commonly astrocytomas or ependymomas. Intradural extramedullary tumors, on the other hand, are usually neurofibromas, schwannomas, or meningiomas. Read More

Neurol India 2021 Mar-Apr;69(2):508-510

Consultant, Neurosurgery, Aditya Birla Memorial Hospital, Pune, Maharashtra, India.

Am J Surg Pathol 2021 Apr 23. Epub 2021 Apr 23.

Institute of Pathology, Friedrich Alexander University Erlangen-Nürnberg, University Hospital, Erlangen Department of Thoracic Oncology, Thoraxklinik, National Center for Tumor Diseases at Heidelberg University Hospital Translational Lung Research Center Heidelberg (TLRC), Member of the German Center for Lung Research (DZL) Department of Thoracic Surgery, Thoraxklinik, Heidelberg University Hospital Institute of Pathology, University of Heidelberg, Heidelberg, Germany Department of Pathology, Charles University, Faculty of Medicine in Plzen Bioptical Laboratory Ltd, Plzen, Czech Republic Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY.

Clear cell (hemangioblastoma-like) stromal tumor of the lung (CCST-L) is a recently described distinctive rare pulmonary neoplasm of unknown histogenesis and molecular pathogenesis. Only 7 cases have been reported in 2 recent studies, although additional cases might have been reported under the heading of extraneural pulmonary hemangioblastoma. We herein describe 4 CCST-L cases, 3 of them harboring a YAP1-TFE3 fusion. Read More

Ophthalmol Retina 2021 Apr 20. Epub 2021 Apr 20.

Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA. Electronic address:

Purpose: To report the efficacy of photodynamic therapy (PDT) for management of retinal hemangioblastoma.

Design: Retrospective case series.

Participants: Seventeen patients with retinal hemangioblastoma treated with PDT. Read More

A A Pract 2021 Apr 21;15(4):e01451. Epub 2021 Apr 21.

From the Department of Anesthesia and Intensive Care.

A 38-year-old pregnant woman in her 24th week of gestation was admitted to our neurosurgical intensive care unit with a 5-cm cerebellar hemangioblastoma and acute hydrocephalus. Initial management included the placement of an external ventricular drain to prevent neurological deterioration. Five days after the initial diagnosis, the patient successfully underwent a neurosurgical intervention to remove the lesion. Read More

Surg Neurol Int 2021 30;12:126. Epub 2021 Mar 30.

Department of Neurology, Uji Takeda Hospital, Kyoto, Japan.

Background: Spinal hemangioblastomas account for 1-3% of all spinal cord tumors and are mostly intramedullary in location. Here, we report an intradural extramedullary hemangioblastoma of the thoracic spine, occurring in in a patient without von Hippel-Lindau disease.

Case Description: A 58-year-old female had a 5-year history of progressive left lower extremity weakness. Read More

Neurosurg Rev 2021 Apr 20. Epub 2021 Apr 20.

Department of Neurosurgery, Faculty of Medicine, University of Miyazaki, 5200 Kihara, Kiyotake-cho, Miyazaki, 889-1692, Japan.

Cerebellar hemangioblastomas remain surgically challenging because of the narrow, deep surgical corridors and tumor hypervascularity. Various surgical approaches are used according to the location, but optimal approaches have not been established. We propose a system of surgical approaches based on the venous drainage systems to facilitate surgical planning and achieve acceptable neurological outcomes. Read More

Br J Neurosurg 2021 Apr 20:1-5. Epub 2021 Apr 20.

Department of Neurosurgery, Borgo Trento Hospital, AOUI Verona, Verona, Italy.

Hemangioblastoma is a rare benign vascular tumour of the central nervous system that occurs either sporadically or in association with Von Hippel-Lindau syndrome. Hemangioblastoma can be found throughout the central nervous system and usually present with late manifestations due to their slow growth rate. Cauda equina hemangioblastomas are extremely rare, and in the literature, no cases are reported on the surgical management of pregnant patients with this condition. Read More

J Fr Ophtalmol 2021 Apr 16. Epub 2021 Apr 16.

Université Tunis El Manar, faculté de médecine de Tunis, hôpital Habib-Thameur, 3, rue A. Ben Ayed, 1089 Tunis, Tunisie.

J Craniofac Surg 2021 Apr 15. Epub 2021 Apr 15.

Department of Neurosurgery of Wuhan Third Hospital Department of Surgery and Anesthesia, Taikang Tongji (Wuhan) Hospital, Wuhan Department of Neurosurgery of Shenzhen People's Hospital, Shenzhen, China.

Abstract: Cerebellar hemangioblastomas with pregnancy is rare, but coexistence of pregnancy and recurrent cerebellar hemangioblastomas is rather rare. And coexistence of other diseases during pregnancy usually leads to misdiagnosis because of pregnancy reaction. A 29-year-old woman, at the 8th week of pregnancy, complained of nausea, vomiting, and episodic posterior occipital pain and was misdiagnosed pregnancy reaction without any special treatment during her several visits to local hospital. Read More

J Fr Ophtalmol 2021 Apr 14. Epub 2021 Apr 14.

Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, 840, Walnut Street, 14th Floor, 19107 Philadelphia, PA, United States. Electronic address:

Zh Vopr Neirokhir Im N N Burdenko 2021 ;85(2):60-66

Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russia.

Hemangioblastoma of the filum terminale and cauda equina is a rare tumor of nervous system. There are only few case reports in the literature. In this manuscript, the authors analyze the diagnosis and treatment of patients with this disease. Read More

Acta Ophthalmol 2021 Apr 8. Epub 2021 Apr 8.

Department of Ophthalmology, Erasmus Medical Center, Rotterdam, The Netherlands.

Importance: Approximately twenty per cent of Von Hippel-Lindau patients with retinal haemangioblastomas (RH) suffer from visual impairment. Various treatment options are available for peripheral RH. However, management of peripheral RH is complex due to multifocality and bilaterality. Read More

J Craniovertebr Junction Spine 2020 Oct-Dec;11(4):254-261. Epub 2020 Nov 26.

Department of Neurosurgery, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India.

Extradural spinal nerve root hemangioblastoma is a rare entity with very few cases reported in the literature. A comprehensive picture of the treatments and outcomes of the same is thus not available. A systematic search was done according to PRISMA guidelines. Read More

JAMA Neurol 2021 Jun;78(6):755-756

Department of Neurosurgery, Helsinki University Hospital, Helsinki, Finland.

Acta Neurochir (Wien) 2021 Apr 3. Epub 2021 Apr 3.

Department of Neurosurgery, Hadassah-Hebrew University Medical Center, P.O. Box 12000, 91120, Jerusalem, Israel.

Background: Hemangioblastomas (HGBs) are highly vascular benign tumors, commonly located in the posterior fossa, and 80% of them are sporadic. Patients usually present with features of raised intracranial pressure and cerebellar symptoms. HGB can be classified as either mostly cystic or solids. Read More

Semin Ophthalmol 2021 Mar 23:1-12. Epub 2021 Mar 23.

NESMOS Department, Ophthalmology Unit, St. Andrea Hospital, University of Rome La Sapienza, Rome, Italy.

Background: Neurofibromatosis, Von Hippel Lindau disease, and tuberous sclerosis complex are classified under the term phakomatoses. They are characterized by ocular vascular abnormalities such as vascular tortuosity, corkscrew retinal vessel configuration, moyamoya-like aspect, microaneurysms, hemangioblastomas, and focal sheathing of retinal arteries, possibly due to abnormal formation, migration, and differentiation of neural crest cells. These alterations can be the first sign or the hallmark of disease and can be related to vasoproliferative tumors. Read More

World Neurosurg 2021 Jun 19;150:e550-e560. Epub 2021 Mar 19.

Department of Neurosurgery and Gamma Knife Radiosurgery, I.R.C.C.S. San Raffaele Hospital, Milan, Italy. Electronic address:

Objective: The present study aimed to perform a comprehensive data analysis of 47 consecutive patients treated in 8 years and to observe how clinical, radiologic, and surgical factors affect early and long-term outcomes, recurrence rate, and survival.

Methods: Clinical, radiologic, and surgical data were collected retrospectively from the review of a prospectively collected database. The neurologic disability was evaluated according to the modified Rankin Scale (mRS). Read More

Endocr Pract 2021 Mar 15. Epub 2021 Mar 15.

Neuroendocrine Tumors Service, Division of Endocrinology, Diabetes and Metabolism, The Chaim Sheba Medical Center, a VHL Alliance Clinical Care Center;; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. Electronic address:

Objective: The main causes for morbidity and mortality in von Hippel-Lindau (VHL) disease are central nervous system hemangioblastoma and clear cell renal cell carcinoma, whereas the effect of VHL-related pancreatic neuroendocrine tumors (PNET) on patient outcome is unclear. We assessed the impact of PNET diagnosis in patients with VHL on all-cause mortality (ACM) risk METHODS: We used the Surveillance, Epidemiology and End Results database. Of 16,344 patients, 170 had VHL based on clinical diagnostic criteria, and 510 patients had PNET (91 VHL-related and 419 sporadic). Read More

J Cancer Res Ther 2021 Jan-Mar;17(1):279-281

Department of Pathology, Kasturba Medical College, Manipal, Karnataka, India.

Retinal hemangioblastomas are one of the most common and early manifestations of Von Hippel-Lindau disease. Early detection is the key in their management. When left untreated, these benign neoplasms may continue to grow and result in scleral infiltration and extraocular extension warranting enucleation of the globe. Read More

Acta Ophthalmol 2021 Mar 15. Epub 2021 Mar 15.

Eye Centre, Medical Centre - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.

Background/aims: Retinal haemangioblastomas (RH) remain a major cause of visual impairment in patients with von Hippel-Lindau (VHL) disease. Identification of genotype-phenotype correlation is an important prerequisite for better management, treatment and prognosis.

Methods: Retrospective, single-centre cohort study of 200 VHL patients. Read More