Resection of a Medulla Oblongata Hemangioblastoma: 2-Dimensional Operative Video

2021 ◽  
Author(s):  
Walid Ibn Essayed ◽  
Ossama Al-Mefty
Keyword(s):  
Venous Drainage ◽  
Radical Resection ◽  
Vascular Tumors ◽  
En Bloc ◽  
Von Hippel Lindau ◽  
Suboccipital Craniotomy ◽  
Von Hippel Lindau Disease ◽  
The Central Nervous System ◽  
Technical Principles ◽  
Dorsal Medulla

Abstract Hemangioblastomas are benign vascular tumors that can be sporadic or multiple, as part of Von Hippel-Lindau disease. They develop at any level of the central nervous system, with a predilection for the dorsal medulla among brainstem locations. Radical resection of the solid portion of the tumor is the best treatment option.1,2 The resection should be en bloc to avoid uncontrollable intraoperative hemorrhage hindering safe dissection. Preservation of the venous drainage during the progressive dissection of the tumor of the surrounding structures and interruption of numerous small arterial feeders is a tenet for safe surgical resection.3 Once the tumor is completely disconnected, the large draining veins can be coagulated, and the tumor removed. We demonstrate these technical principles in the surgery of a 30-yr-old female with an exophytic hemangioblastoma from the dorsal medulla obstructing the fourth ventricle outflow. We demonstrate the resection of this lesion through a suboccipital craniotomy in a sitting position.4 The patient consented to the surgery and publication of images. Image at 1:26 from Kadri and Al-Mefty,4 by permission from the Congress of Neurological Surgeons.

scholarly journals Inactivation of the Arylhydrocarbon Receptor Nuclear Translocator (Arnt) Suppresses von Hippel-Lindau Disease-Associated Vascular Tumors in Mice

2005 ◽  
Vol 25 (8) ◽  
pp. 3163-3172 ◽  
Author(s):  
Erinn B. Rankin ◽  
Debra F. Higgins ◽  
Jacqueline A. Walisser ◽  
Randall S. Johnson ◽  
Christopher A. Bradfield ◽  
...  
Keyword(s):  
Transcription Factors ◽  
Germ Line ◽  
Hypoxia Inducible Factor ◽  
Suppressor Gene ◽  
Vascular Tumors ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease ◽  
Arylhydrocarbon Receptor ◽  
Vhl Disease ◽  
Endothelial Growth Factor

ABSTRACT Patients with germ line mutations in the VHL tumor suppressor gene are predisposed to the development of highly vascularized tumors within multiple tissues. Loss of pVHL results in constitutive activation of the transcription factors HIF-1 and HIF-2, whose relative contributions to the pathogenesis of the VHL phenotype have yet to be defined. In order to examine the role of HIF in von Hippel-Lindau (VHL)-associated vascular tumorigenesis, we utilized Cre-loxP-mediated recombination to inactivate hypoxia-inducible factor-1α (Hif-1α) and arylhydrocarbon receptor nuclear translocator (Arnt) genes in a VHL mouse model of cavernous liver hemangiomas and polycythemia. Deletion of Hif-1α did not affect the development of vascular tumors and polycythemia, nor did it suppress the increased expression of vascular endothelial growth factor (Vegf) and erythropoietin (Epo). In contrast, phosphoglycerokinase (Pgk) expression was substantially decreased, providing evidence for target gene-dependent functional redundancy between different Hif transcription factors. Inactivation of Arnt completely suppressed the development of hemangiomas, polycythemia, and Hif-induced gene expression. Here, we demonstrate genetically that the development of VHL-associated vascular tumors in the liver depends on functional ARNT. Furthermore, we provide evidence that individual HIF transcription factors may play distinct roles in the development of specific VHL disease manifestations.

Hemangioblastoma diagnosis and surveillance in von Hippel–Lindau disease: a consensus statement

2021 ◽  
pp. 1-6
Author(s):  
Kristin Huntoon ◽  
Matthew J. Shepard ◽  
Rimas V. Lukas ◽  
Ian E. McCutcheon ◽  
Anthony B. Daniels ◽  
...  
Keyword(s):  
Age At Onset ◽  
Benign Tumors ◽  
Expert Committee ◽  
Level Of Evidence ◽  
Von Hippel Lindau ◽  
Assessment Development ◽  
History Of ◽  
Von Hippel Lindau Disease ◽  
The Central Nervous System ◽  
Cancer Network

OBJECTIVE Hemangioblastomas are a frequent underlying cause of neurological morbidity and death in patients with von Hippel–Lindau disease (VHL). Although these benign tumors can cause significant neurological debility when undetected and untreated, unified evidence-based surveillance recommendations for VHL patients have not been established. To develop consensus recommendations, the VHL Alliance established an expert committee, named the International VHL Surveillance Guidelines Consortium, to define surveillance recommendations. METHODS The Central Nervous System (CNS) Hemangioblastoma Subcommittee of the Guidelines Consortium was formed as a multidisciplinary team of experts in the diagnosis and management of hemangioblastomas. Recommendations were formulated using the Grading of Recommendations, Assessment, Development and Evaluation (GRADE) and National Comprehensive Cancer Network Categories of Evidence and Consensus categorization after a comprehensive literature review. RESULTS Published studies (n = 49) that discussed age at onset, MRI frequency, natural history of VHL, and the risks and benefits of surveillance were analyzed. Based on this analysis, the authors recommend that clinical evaluation (yearly) be used as the primary screening tool for hemangioblastomas in VHL. The subcommittee suggests that screening be performed between the ages of 11 and 65 years, or with the onset of symptoms, for synchronicity with other testing regimens in VHL. The subcommittee also recommends that baseline MRI be first performed at the age of 11 years (suggested 2B, level of evidence D) or after identification of neurological symptoms or signs (if earlier) and continue every 2 years (recommended 2A, level of evidence A). CONCLUSIONS The CNS Hemangioblastoma Subcommittee of the International VHL Surveillance Guidelines Consortium here proposes guidelines that aim to increase the early detection of VHL-associated hemangioblastomas to reduce their morbidity and mortality.

scholarly journals Extra and intradural spinal Hemangioblastoma

2012 ◽  
Vol 11 (3) ◽  
pp. 242-244
Author(s):  
Marcelo Campos Moraes Amato ◽  
Caio César Marconato Simões Matias ◽  
João Alberto Assirati Junior ◽  
Aline Paixão Becker ◽  
Carlos Gilberto Carlotti Junior ◽  
...  
Keyword(s):  
Histopathological Examination ◽  
Correct Diagnosis ◽  
White Male ◽  
Residual Tumor ◽  
Illustrative Case ◽  
Low Grade ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease ◽  
The Central Nervous System ◽  
Spinal Hemangioblastoma

Hemangioblastomas of the central nervous system (CNS) are low-grade highly vascularized tumors that may be sporadic or associated with Von Hippel-Lindau disease. Extradural hemangioblastomas are uncommon and those located extra and intradurally are even rarer. This study uses an illustrative case and literature review to discuss the difficulties to consider the correct diagnosis and to select the best surgical approach. A 57 years-old white male patient presented with myelopathy and right C5 radiculopathy. The images showed a lobulated, hourglass shaped, highly enhanced extra/intradural lesion that occupied the spinal canal and widened the C4-C5 right intervertebral foramen. Total resection of the intradural lesion was achieved through a posterior approach, but the extradural part could only be partially removed. Complete improvement was observed after four months of follow-up and the residual tumor has been followed up clinically and radiologically. Even though the preoperative impression was of a spinal schwannoma, the histopathological examination revealed grade I hemangioblastoma as per WHO. Despite their rarity, current complementary exams allow considering the diagnosis of hemangioblastoma preoperatively. That is essential to a better surgical planning in view of the particular surgical features of this lesion.

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 Differences in genetic and epigenetic alterations between von Hippel–Lindau disease–related and sporadic hemangioblastomas of the central nervous system

2017 ◽  
Vol 19 (9) ◽  
pp. 1228-1236 ◽  
Author(s):  
Shunsaku Takayanagi ◽  
Akitake Mukasa ◽  
Shota Tanaka ◽  
Masashi Nomura ◽  
Mayu Omata ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Epigenetic Alterations ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease ◽  
The Central Nervous System

scholarly journals Resection of spinal hemangioblastoma

2014 ◽  
Vol 37 (v2supplement) ◽  
pp. Video15
Author(s):  
Giuseppe Lanzino ◽  
Saul F. Morales-Valero ◽  
William E. Krauss
Keyword(s):  
Spinal Cord ◽  
Venous Drainage ◽  
Complete Resection ◽  
Cervical Cord ◽  
Von Hippel Lindau ◽  
Link Type ◽  
Single Piece ◽  
Von Hippel Lindau Disease ◽  
Spinal Hemangioblastoma

Spinal cord hemangioblastomas occur as sporadic lesions or in the setting of Von Hippel-Lindau disease. In this intraoperative video we present a case of sporadic cervical cord hemangioblastoma and illustrate the main surgical steps to achieve safe and complete resection which include: identification and division of the feeding arteries; careful circumferential dissection of the tumor from the surrounding gliotic cord; identification, isolation and division of the main venous drainage and single piece removal of the tumor.The video can be found here: http://youtu.be/I7DxqRrfTxc.

scholarly journals Propranolol and Somatostatin Agonist Therapy Resulting in Symptom Improvement and Stabilization of von Hippel-Lindau-Associated Hemangioblastoma: A Case Report

2021 ◽  
Author(s):  
Maran Ilanchezhian ◽  
Reinier Alvarez ◽  
Amanda Carbonell ◽  
Brigitte C. Widemann ◽  
Prashant Chittiboina ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Autosomal Dominant ◽  
Symptom Improvement ◽  
Agonist Therapy ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease ◽  
The Central Nervous System ◽  
Tumor Types ◽  
Systemic Therapies

Abstract Von Hippel-Lindau disease is an inherited, autosomal dominant tumor predisposition syndrome which leads to susceptibility for developing hemangioblastomas of the central nervous system and retina, as well as other tumor types. No approved systemic therapies exist for this disease. Here, we present a patient diagnosed with Von Hippel-Lindau disease, with central nervous system and retinal hemangioblastomas, treated with propranolol and subsequently with Lanreotide. On both treatments, the patient showed notable symptom improvement. In addition, since starting Lanreotide, the patients hemangioblastoma has remained stable on imaging. Based on the symptom improvement in this patient and other reported findings in the literature, propranolol and somatostatin agonist therapy may have an anti-tumor effect in Von Hippel-Lindau associated hemangioblastomas; however, further investigation is warranted.

scholarly journals Pathology of the Nervous System in Von Hippel-Lindau Disease

2015 ◽  
Vol 2 (3) ◽  
pp. 114-129 ◽  
Author(s):  
Alexander O. Vortmeyer ◽  
Ahmed K. Alomari
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Hypoxia Inducible Factors ◽  
Metastatic Renal Cancer ◽  
Vhl Gene ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease ◽  
The Central Nervous System ◽  
Vhl Disease ◽  
Common Manifestation

Von Hippel-Lindau (VHL) disease is a tumor syndrome that frequently involves the central nervous system (CNS). It is caused by germline mutation of the VHL gene. Subsequent VHL inactivation in selected cells is followed by numerous well-characterized molecular consequences, in particular, activation and stabilization of hypoxia-inducible factors HIF1 and HIF2. The link between VHL gene inactivation and tumorigenesis remains poorly understood. Hemangioblastomas are the most common manifestation in the CNS; however, CNS invasion by VHL disease-associated endolymphatic sac tumors or metastatic renal cancer also occur, and their differentiation from primary hemangioblastoma may be challenging. Finally, in this review, we present recent morphologic insights on the developmental concept of VHL tumorigenesis which is best explained by pathologic persistence of temporary embryonic progenitor cells. 

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