Premium
Treatment response of CNS high‐grade neuroepithelial tumors with MN1 alteration
Author(s) -
Baroni Lorena V.,
Rugilo Carlos,
Lubieniecki Fabiana,
Sampor Claudia,
Freytes Candela,
Nobre Liana,
Hansford Jordan R.,
Malalasekera Vajiranee S.,
Zapotocky Michal,
Dodgshun Andrew,
Martinez Ofelia Cruz,
La Madrid Andres Morales,
Lavarino Cinzia,
Suñol Mariona,
Rutkowski Stefan,
Schuller Ulrich,
Bouffet Eric,
Ramaswamy Vijay,
Alderete Daniel
Publication year - 2020
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.28627
Subject(s) - medicine , radiation therapy , malignancy , central nervous system , chemotherapy , neuroepithelial cell , adjuvant radiotherapy , radiology , surgery , pathology , stem cell , neural stem cell , biology , genetics
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.