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CS-02 CLINICAL AND MOLECULAR ANALYSIS OF ASTROBLASTOMAS

Astroblastoma is extremely rare brain tumor which mostly arise in cerebral hemisphere of children and young adult. Limited data exists on its clinical feature and molecular analysis. We recently experienced two female patients with astroblastoma in the cerebrum. Case 1 is three-year-old girl. She de...

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Detalles Bibliográficos
Autores principales: Ichikawa, Tomotsugu, Mino, Satoshi, Kenmotsu, Naoya, Kuramoto, Satoshi, Ono, Yasuhiro, Kawauchi, Masamitsu, Kurozumi, Kazuhiko, Date, Isao, Shimada, Akira, Nakamura, Satoko, Yanai, Hiroyuki, Hirato, Junko, Yokoo, Hideaki
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7213145/
http://dx.doi.org/10.1093/noajnl/vdz039.173
Descripción
Sumario:Astroblastoma is extremely rare brain tumor which mostly arise in cerebral hemisphere of children and young adult. Limited data exists on its clinical feature and molecular analysis. We recently experienced two female patients with astroblastoma in the cerebrum. Case 1 is three-year-old girl. She developed left hemiparesis. CT and MRI revealed large supratentorial mass with cystic component and calcification. Gross total removal was achieved. She is well without recurrence on MRI one year after surgery. Case 2 is 42-year-old lady. She developed partial seizure. CT and MRI revealed a mass with ring-enhancement in the left temporal lobe. Gross total removal was achieved under awake craniotomy. She is well without recurrence on MRI six months after surgery. Pathologic examination of both patients showed pseudorosette formation of tumor cells around vasculature. Molecular analysis revealed rearrangement of MN-1 in case 1 but not in case 2. Case 2 showed BRAF V600E mutation and loss of CDKN2A/2B. Both patients received no adjuvant therapy. Prognosis of astroblastoma varies and standard of treatment is not established. Gross total resection is associated with increased survival, but the role of adjuvant chemotherapy and radiation therapy are controversial. Advances in molecular analysis will lead to establish molecular classification and risk-adapted treatment strategy.