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. 2020 Dec;67(12):e28627.
doi: 10.1002/pbc.28627. Epub 2020 Sep 22.

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

Lorena V Baroni  1 , Carlos Rugilo  2 , Fabiana Lubieniecki  3 , Claudia Sampor  1 , Candela Freytes  1 , Liana Nobre  4 , Jordan R Hansford  5   6   7 , Vajiranee S Malalasekera  5 , Michal Zapotocky  8 , Andrew Dodgshun  9 , Ofelia Cruz Martinez  10 , Andres Morales La Madrid  10 , Cinzia Lavarino  11 , Mariona Suñol  12 , Stefan Rutkowski  13 , Ulrich Schuller  13   14   15 , Eric Bouffet  4 , Vijay Ramaswamy  4   16 , Daniel Alderete  1
Affiliations

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

Lorena V Baroni et al. Pediatr Blood Cancer. 2020 Dec.

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 challenge despite aggressive surgical resections and upfront radiotherapy, warranting new approaches to this rare malignancy.

Keywords: CNS HGNET-MN1; MN1; astroblastoma; ependymoma; neuroepithelial tumor.

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REFERENCES

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