Journal article

Treatment response of CNS high-grade neuroepithelial tumors with MN1 alteration.

Lorena V Baroni, Carlos Rugilo, Fabiana Lubieniecki, Claudia Sampor, Candela Freytes, Liana Nobre, Jordan R Hansford, Vajiranee S Malalasekera, Michal Zapotocky, Andrew Dodgshun, Ofelia Cruz Martinez, Andres Morales La Madrid, Cinzia Lavarino, Mariona Suñol, Stefan Rutkowski, Ulrich Schuller, Eric Bouffet, Vijay Ramaswamy, Daniel Alderete

Pediatric Blood & Cancer | Published : 2020

Abstract

Central nervous system high-grade neuroepithelial tumor with MN1 alteration (CNS HGNET-MN1) is a rare recently described entity. Fourteen CNS HGNET-MN1 patients were identified using genome-wide methylation arrays/RT-PCR across seven institutions. All patients had surgery (gross total resection: 10; subtotal resection: four) as initial management followed by observation alone in three patients, followed by radiotherapy in eight patients (focal: five; craniospinal: two; CyberKnife: one) and systemic chemotherapy in three patients. Seven patients relapsed; five local and two metastatic, despite adjuvant radiotherapy, of which three died. Treatment of CNS HGNET-MN1 remains a major treatment cha..

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