scholarly journals Central Nervous System Hemangioblastoma

2020 ◽  
Author(s):  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
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 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.

Management and outcomes of pregnant patients with central nervous system hemangioblastoma

2018 ◽  
Vol 57 ◽  
pp. 126-130 ◽  
Author(s):  
Xiu-jian Ma ◽  
Guo-bin Zhang ◽  
Teng-xian Guo ◽  
Cheng-bei Li ◽  
Yu-jia Chen ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Central Nervous System Hemangioblastoma
Sign in / Sign up

Export Citation Format

Share Document