In a recent article entitled : HGNET-BCOR Tumors of the Cerebellum Clinicopathologic and Molecular Characterization of 3 Cases by Romain Appay and al. in press in the American Journal of Surgical Pathology, the authors from the departments of Pathology and Neuropathology-Radiotherapy-Hematology & Pediatric Oncology-Pediatric Neurosurgery, Timone Hospital, France; the INSERM, CRO2 UMR_S 911, Aix-Marseille Université; the Clinical Cooperation Unit Neuropathology;Division of Pediatric Neurooncology, German Cancer Research Center; Cancer Consortium, Core Center Heidelberg, Heidelberg; and Department of Neuropathology, Medical Center Sigmund Freud, University of Bonn, Bonn, Germany; report on 3 cases of the newly identified type of tumours with a very dismal prognosis. While 2 patients died quickly because of local and or metastatic relapse, the thirs patient is alive. Although the follow up is short, following a craniospinal irradiation the patient received a metronomic combination with temozolomide-irinotecan and itraconazole as an SHH inhibitor since this type of tumour has been reported to have activation of the SHH pathway. Additional cases are warranted to confirlm both the potential role of radiotherapy and metronomics in this disease.