Clinical features of patients bearing central nervous system hemangioblastoma in von Hippel-Lindau disease

2012 ◽  
Vol 155 (1) ◽  
pp. 1-7 ◽  
Author(s):  
Hiroshi Kanno ◽  
Jun-ichi Kuratsu ◽  
Ryo Nishikawa ◽  
Kazuhiko Mishima ◽  
Atushi Natsume ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Clinical Features ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease ◽  
Central Nervous System Hemangioblastoma

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

2020 ◽  
Vol 148 (1) ◽  
pp. 29-38 ◽  
Author(s):  
Hiroshi Kanno ◽  
Tetsuya Yoshizumi ◽  
Masamichi Shinonaga ◽  
Atsuhiko Kubo ◽  
Hidetoshi Murata ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Signaling Pathway ◽  
Von Hippel Lindau ◽  
Stat Signaling ◽  
Von Hippel Lindau Disease ◽  
Central Nervous System Hemangioblastoma

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 BT-06 CENTRAL NERVOUS SYSTEM HEMANGIOBLASTOMA; DIFFERENCES IN CLINICAL PICTURE OF SPORADIC CASES AND VON-HIPPEL LINDAU DISEASE IN 184 CASES

2019 ◽  
Vol 1 (Supplement_2) ◽  
pp. ii37-ii37
Author(s):  
Hirokazu Takami ◽  
Terry Burns ◽  
Ian Parney
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Clinical Picture ◽  
Mayo Clinic ◽  
Tumor Biology ◽  
Clinical Manifestations ◽  
Von Hippel Lindau ◽  
Significant Difference ◽  
Sporadic Cases ◽  
Central Nervous System Hemangioblastoma

Abstract INTRODUCTION Central nervous system hemangioblastoma (CNS HGB) is a rare neoplasm, which predominantly arise in the posterior fossa and spinal cord. The etiology is divided into sporadic and von-Hippel Lindau (VHL) disease. The difference in clinical picture of these 2 types of HGB and differentiation of treatment have not been extensively unraveled yet. METHODS Retrospective analysis of consecutive, neurosurgically managed CNS HGB at Mayo Clinic, 1988–2018. RESULTS 117 sporadic and 67 VHL HGBs were treated by Mayo Clinic. No significant difference in sex was observed. Compared with sporadic cases, VHL cases were younger (51.8 vs 36.0 years old, p<0.0001), had more frequent family history (0.0 vs 41.5 %, p<0.0001), and higher frequency of germline alteration (0.0 vs 84.2 %, p<0.0001). Regarding imaging findings, VHL cases had multiple lesions at presentation more frequently (3.4 vs 82.1 %, p<0.0001), it was more common for sporadic lesions to contain cysts (72.2 vs 51.0 %, p=0.0004), the solid portion rate in the entire lesion was larger in VHL lesions (60.2 vs 69.5 %, p=0.02), and the volume was larger in sporadic cases (15.1 vs 6.6 cc, p<0.0001). Regarding treatment, 131 and 123 surgeries were performed for sporadic and VHL cases, respectively, among which the indication of surgery was preventative in 8.4 and 47.3 %, respectively (p<0.0001). VHL cases had higher number of treatments per case in the follow-up (1.3 vs 2.1, p<0.0001). Recurrence-free survival of sporadic cases was significantly longer than that of VHL cases (p=0.007) and overall survival was longer in sporadic cases than VHL, but not significant (p=0.07). CONCLUSION Clinical presentation and tumor appearance on imaging are highly dependent on the etiology. Differences in clinical manifestations require further study, but may reflect contrasting tumor biology that are tied to genetic differences.

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.

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