Intramedullary gangliogliomas: histopathologic and molecular features of 25 cases

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• 作者：Marco Gessi ; MD^a ; ^{mgessimd@yahoo.com" class="auth_mail" title="E-mail the corresponding author} ; ^{marco.gessi@ukb.uni-bonn.de" class="auth_mail" title="E-mail the corresponding author} ; Evelyn Dö ; rner^a ; Verena Dreschmann^a ; Manila Antonelli ; MD^b ; Andreas Waha ; PhD^a ; Felice Giangaspero ; MD^b ; ^c ; Astrid Gnekow ; MD^d ; Torsten Pietsch ; MD^a
• 关键词：Ganglioglioma ; Spinal cord ; BRAF ; Anaplastic ganglioglioma ; Immunohistochemistry ; BRAFV600E
• 刊名：Human Pathology
• 出版年：2016
• 出版时间：March 2016
• 年：2016
• 卷：49
• 期：Complete
• 页码：107-113
• 全文大小：2193 K

文摘

Gangliogliomas are uncommon glioneuronal tumors, which usually arise in the cerebral hemispheres and occasionally in the brain stem. Gangliogliomas occurring in the spinal cord are extremely rare. In this study, we analyzed the clinical, histopathologic, and molecular features of 25 spinal gangliogliomas. The cases included in our series affected mostly children and young adults (15 males and 10 females; mean age, 20 years; median age, 14 years; age range, 1-72 years) and were predominantly localized in the cervical and thoracic spine. From the clinical point of view (detailed follow-up available for 9 pediatric cases; mean follow-up: 2 years 10 months; range, 3 months to 5 years 10 months), most patients showed stable disease after subtotal resection. Radiotherapy was rarely used as adjuvant treatment. Histologically, gangliogliomas (WHO grade I) (21 cases) showed features largely similar to their supratentorial counterparts. Anaplastic gangliogliomas (World Health Organization grade III) (4 cases) showed features of anaplasia (including high cellularity and increased mitotic and proliferation activity). From a molecular point of view, only 2 tumors (2/19, 11%) harbored a BRAF^V600E mutation. In conclusion, although spinal gangliogliomas display histologic and clinical features similar to their supratentorial counterparts, they show a relatively low frequency of BRAF^V600E mutations, alteration otherwise common in hemispheric and brain stem gangliogliomas.