TY - JOUR
T1 - Ganglioglioma of brain stem and cervicomedullary junction
T2 - A 50 years review of literature
AU - Janjua, M. Burhan
AU - Ivasyk, Iryna
AU - Pisapia, David J.
AU - Souweidane, Mark M.
N1 - Funding Information:
I.I. was supported by a Medical Scientist Training Program grant from the National Institute of General Medical Sciences of the National Institutes of Health under award number T32GM007739 to the Weill Cornell/Rockefeller/Sloan-Kettering Tri-Institutional MD-PhD Program. This study is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health.
Publisher Copyright:
© 2017 Elsevier Ltd
PY - 2017/10
Y1 - 2017/10
N2 - Gangliogliomas are rare low-grade brain tumors composed of both neoplastic glial and neuronal cell elements. The treatment modalities are relatively different in this location and hence factors affecting outcome are poorly understood. We identified 142 brain stem GG patients across 46 studies. The average age was 11.4 years with significant difference b/w males and females under the age of 20 (p = 0.001). 100% of tumors in the CMJ while, 72% of type I and 86% of type II tumors demonstrated contrast enhancement. 72% of type I and 86% of type II tumors demonstrated contrast enhancement. All BRAF mutation positive tumors demonstrated contrast enhancement. Medulla and pons was the most favorable location followed by medulla alone, and the CMJ. In all tumors “gross total resection” (GTR, 16%), “subtotal resection” (STR, 48%) or “partial resection” (PR, 36%) was achieved. Most subtypes II and III were partially resected (86% and 66%), while, subtype I underwent STR (66%). Only 55% of the patients were positive for the BRAF V600E mutation. The overall survival dropped from 50% at 24 to 10% at 60 months, postoperatively. Through this review, we found that an early diagnosis, location, and with the imaging characteristics are vital part of the preoperative planning. Surgical resection is highly dependent on location in the brain stem with radical resection only limited to the most contrast enhancing portion of these tumors. BRAF V600E mutation status should be considered to allow the possibility of targeted therapy in case of a residual tumor and/or regrowth.
AB - Gangliogliomas are rare low-grade brain tumors composed of both neoplastic glial and neuronal cell elements. The treatment modalities are relatively different in this location and hence factors affecting outcome are poorly understood. We identified 142 brain stem GG patients across 46 studies. The average age was 11.4 years with significant difference b/w males and females under the age of 20 (p = 0.001). 100% of tumors in the CMJ while, 72% of type I and 86% of type II tumors demonstrated contrast enhancement. 72% of type I and 86% of type II tumors demonstrated contrast enhancement. All BRAF mutation positive tumors demonstrated contrast enhancement. Medulla and pons was the most favorable location followed by medulla alone, and the CMJ. In all tumors “gross total resection” (GTR, 16%), “subtotal resection” (STR, 48%) or “partial resection” (PR, 36%) was achieved. Most subtypes II and III were partially resected (86% and 66%), while, subtype I underwent STR (66%). Only 55% of the patients were positive for the BRAF V600E mutation. The overall survival dropped from 50% at 24 to 10% at 60 months, postoperatively. Through this review, we found that an early diagnosis, location, and with the imaging characteristics are vital part of the preoperative planning. Surgical resection is highly dependent on location in the brain stem with radical resection only limited to the most contrast enhancing portion of these tumors. BRAF V600E mutation status should be considered to allow the possibility of targeted therapy in case of a residual tumor and/or regrowth.
KW - BRAF V600E mutation status
KW - Brain stem ganglioglioma
KW - Extent of resection
KW - Imaging characteristics
UR - http://www.scopus.com/inward/record.url?scp=85021712517&partnerID=8YFLogxK
U2 - 10.1016/j.jocn.2017.06.021
DO - 10.1016/j.jocn.2017.06.021
M3 - Review article
C2 - 28687443
AN - SCOPUS:85021712517
SN - 0967-5868
VL - 44
SP - 34
EP - 46
JO - Journal of Clinical Neuroscience
JF - Journal of Clinical Neuroscience
ER -