scholarly journals Progressive peritumoral edema defining the optic fibers and resulting in reversible visual loss

2008 ◽  
Vol 109 (2) ◽  
pp. 313-317 ◽  
Author(s):  
Martin Baggenstos ◽  
Emily Chew ◽  
John A. Butman ◽  
Edward H. Oldfield ◽  
Russell R. Lonser
Keyword(s):  
Color Vision ◽  
Vision Loss ◽  
Tumor Resection ◽  
Vascular Tumor ◽  
Peritumoral Edema ◽  
Von Hippel Lindau ◽  
Extended Transsphenoidal Approach ◽  
Von Hippel Lindau Disease ◽  
Optic Fibers ◽  
Reversible Visual Loss

Hemangioblastomas are frequently associated with peritumoral edema caused by extravasation of plasma ultrafiltrate through permeable neoplastic vessels. The authors report the clinical and imaging findings in a 62-year-old man with von Hippel–Lindau disease who presented with rapid (within 24 hours) loss of color vision and nearcomplete loss of left eye vision (acuity too poor to test). Serial MR imaging demonstrated a stable vascular tumor in the medioinferior aspect of the left optic nerve, associated with progressive edema extending from the nerve through to the bilateral optic radiations. Complete resection of the lesion was performed via an extended transsphenoidal approach, and histological examination confirmed the lesion was a hemangioblastoma. Postoperatively, the patient recovered color vision and had improvement in visual acuity (20/320). Serial imaging in this unique case captured the progressive extravasation of peritumoral edema that tracked and defined the parallel white matter tracts of first- and second-order neurons of the optic system, causing vision loss. Tumor resection led to resolution of the edema and improvement in visual function.

scholarly journals A RARE CASE OF SPORADIC SUPRATENTORIAL HEMANGIOBLASTOMA

2020 ◽  
pp. 57-63
Author(s):  
V. E. Kocharyan ◽  
T. G. Sarkisyan ◽  
G. I. Kovalev ◽  
G. G. Muzlaev ◽  
A. I. Bogrov
Keyword(s):  
World Literature ◽  
Rare Case ◽  
Benign Tumor ◽  
Tumor Resection ◽  
Von Hippel Lindau ◽  
Dense Networks ◽  
Rare Benign Tumor ◽  
Von Hippel Lindau Disease ◽  
The Central Nervous System ◽  
The Brain

We report the rare case of 65-year-old male patient with supratentorial hemangioblastoma and gigantic cyst of the left hemisphere of the brain without von Hippel-Lindau disease. Hemangioblastoma is a rare benign tumor classified as grade I by the WHO of the central nervous system of uncertain histogenesis. This neoplasm’s stroma consists of dense networks of thin-walled blood vessels of various calibers. About 140 cases of supratentorial localization of this type of tumor are presented in the world literature. We have given a fairly complete clinical, neuroradiological and histological picture that allows one to differentiate hemangioblastoma from other histological structures. Promising modalities in the treatment of patients with this pathology when it is impossible to perform total tumor resection are considered.

scholarly journals Medullary hemangioblastoma in a child with von Hippel-Lindau disease: vascular tumor perfusion depicted by arterial spin labeling and dynamic contrast-enhanced imaging

2015 ◽  
Vol 16 (1) ◽  
pp. 50-53 ◽  
Author(s):  
Hyun Woo Goo ◽  
Young-Shin Ra
Keyword(s):  
Arterial Spin Labeling ◽  
Vascular Tumor ◽  
Spin Labeling ◽  
Diagnostic Value ◽  
Tumor Perfusion ◽  
Von Hippel Lindau ◽  
Dynamic Contrast Enhanced Imaging ◽  
Dynamic Contrast Enhanced ◽  
Contrast Enhanced ◽  
Von Hippel Lindau Disease

Medullary hemangioblastoma is very rare in children. Based on small nodular enhancement with peritumoral edema and without dilated feeding arteries on conventional MRI, hemangioblastoma, pilocytic astrocytoma, oligodendroglioma, and ganglioglioma were included in the differential diagnosis of the medullary tumor. In this case report, the authors emphasize the diagnostic value of arterial spin labeling and dynamic contrast-enhanced MRI in demonstrating vascular tumor perfusion of hemangioblastoma in a 12-year-old boy who was later found to have von Hippel-Lindau disease.

scholarly journals More than it meets the eye! Vision loss as a presenting symptom of von Hippel–Lindau disease

2020 ◽  
Vol 15 (3) ◽  
pp. 314
Author(s):  
AdityaV Nair ◽  
Asish Vijayaraghavan ◽  
PullumpallilT Alexander ◽  
ArunM Mani ◽  
Divya Giridhar ◽  
...  
Keyword(s):  
Vision Loss ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease

Adenomas of the Temporal Bone

1988 ◽  
Vol 97 (6) ◽  
pp. 605-612 ◽  
Author(s):  
Thomas L. Eby ◽  
Miro S. Makek ◽  
Ugo Fisch
Keyword(s):  
Tumor Invasion ◽  
Bone Destruction ◽  
Complete Removal ◽  
Vascular Tumor ◽  
Petrous Bone ◽  
Von Hippel Lindau ◽  
Subtotal Petrosectomy ◽  
Benign Process ◽  
Von Hippel Lindau Disease ◽  
Ear Symptoms

Three cases of primary adenomas of the petrous bone are presented and compared to an adenoma arising from the external ear canal. In contrast to other adenomatous tumors, these caused slowly progressive inner ear symptoms without other signs, hence making differentiation from other benign lesions difficult. In one case, association with von Hippel-Lindau disease is suspected because of a strong family history and recent discovery of a similar lesion in the opposite ear. Bone destruction was found radiographically and clinically with vascular tumor tissue and cysts permeating the bone, causing adjacent bone destruction and remodeling. The histopathologic examination demonstrated this to be a benign process, however, with no direct tumor invasion or other signs of malignancy. Subtotal petrosectomy is a proven approach for complete removal of these tumors. Review of the literature revealed no previous reports of benign adenomas originating in the petrous bone.

scholarly journals Pathological and Clinical Features and Management of Central Nervous System Hemangioblastomas in von Hippel-Lindau Disease

2014 ◽  
Vol 1 (4) ◽  
pp. 46-55 ◽  
Author(s):  
Hiroshi Kanno ◽  
Natsuki Kobayashi ◽  
Satoshi Nakanowatari
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Clinical Features ◽  
Vascular Tumor ◽  
Preoperative Embolization ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease ◽  
Vhl Disease ◽  
Perioperative Hemorrhage

Central nervous system (CNS) hemangioblastoma is the most common manifestation of von Hippel-Lindau (VHL) disease. It is found in 70-80% of VHL patients. Hemangioblastoma is a rare form of benign vascular tumor of the CNS, accounting for 2.0% of CNS tumors. It can occur sporadically or as a familial syndrome. CNS hemangioblastomas are typically located in the posterior fossa and the spinal cord. VHL patients usually develop a CNS hemangioblastoma at an early age. Therefore, they require a special routine for diagnosis, treatment and follow-up. The surgical management of symptomatic tumors depends on many factors such as symptom, location, multiplicity, and progression of the tumor. The management of asymptomatic tumors in VHL patients is controversial since CNS hemangioblastomas grow with intermittent quiescent and rapid-growth phases. Preoperative embolization of large solid hemangioblastomas prevents perioperative hemorrhage but is not necessary in every case. Radiotherapy should be reserved for inoperable tumors. Because of complexities of VHL, a better understanding of the pathological and clinical features of hemangioblastoma in VHL is essential for its proper management.

scholarly journals Sporadic Intradural Extramedullary Hemangioblastoma of Cauda Equina with Large Peritumoral Cyst—A Rare Presentation

2022 ◽  
Author(s):  
Pushpa B. Thippeswamy ◽  
Dilip C. R. Soundararajan ◽  
Ríshi M. Kanna ◽  
Venkata S. Kuna ◽  
Shanmuganathan Rajasekaran
Keyword(s):  
Cauda Equina ◽  
Surgical Excision ◽  
Vascular Tumor ◽  
Spinal Tumors ◽  
Rare Presentation ◽  
Von Hippel Lindau ◽  
Drop Metastasis ◽  
Neural Axis ◽  
Von Hippel Lindau Disease ◽  
Intradural Extramedullary

AbstractCauda equina intradural tumors commonly reported include ependymoma, schwannoma, neurofibroma, meningioma, and drop metastasis. Hemangioblastoma of the neural axis is a rare benign vascular tumor comprising only 1.6 to 6.4% of spinal tumors, and are usually associated with Von-Hippel Lindau disease. Sporadic intradural extramedullary hemangioblastoma involving cauda equina is very rare with only countable reports, and the presence of peritumoral cyst has been reported only once. We report one such case of hemangioblastoma with a large peritumoral cyst, which was diagnosed radiologically and confirmed by histopathology following surgical excision. Pertinent radiological characteristics, diagnostic clues, treatment, and surgical outcomes are discussed.

The yield and cost of radiological screening in von Hippel-Lindau disease

2019 ◽  
Author(s):  
Timothy McMillan ◽  
Anju Sahdev ◽  
Jane Evanson ◽  
Lorraine McAndrew ◽  
Lee Martin ◽  
...  
Keyword(s):  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease

A novel heterozygous mutation in exon 3 of VHL gene leading to Von Hippel-Lindau disease in a Turkish family

2019 ◽  
Author(s):  
Ozge Tasgin Yildirim ◽  
Ismail Yildiz ◽  
Fatih Horozoglu ◽  
Aysun Gonen ◽  
Cenk Murat Yazici ◽  
...  
Keyword(s):  
Heterozygous Mutation ◽  
Vhl Gene ◽  
Von Hippel Lindau ◽  
Turkish Family ◽  
Von Hippel Lindau Disease
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