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Abstract BACKGROUND Between 2/2012 and 7/2018, 112 patients with non-germinoma were registered as intend-to-treat patients within the SIOP CNS GCT II trial: 88 patients were male and 24 female. Median age was 13 years (range: 0,1-25,1 years). 95 patients had localised and 17 metastastic disease. 21 patients with bifocal tumors were reported, defined as a synchronous suprasellar and pineal tumour at diagnosis. 6/21 had metastases. PATIENTS/METHODS All Patients presented with several main clinical symptoms: diabetes insipidus (DI) (18), vision impairment (16), raised intracranial pressure (10), headache (10), cognitive impairments (7), somnolence (5). Diagnosis was confirmed by tumor markers in all, two with additional resection and 4 with biopsy. All patients received upfront PEI (cisplatin/etoposide/ifosfamide) chemotherapy, with dose-intensification (HD-PEI) for those with diagnostic serum or CSF AFP 1000 ng/ml (high-risk), before RT. Patients without dissemination received local radiotherapy (RT) of 54 Gy to both sites. Patients with dissemination received CSI 30 Gy plus boost to primary sites of 24 Gy and RT to visible metastases. RESULTS Among 12 standard-risk patients with localised disease 3/12 achieved CR, 1/12 PR and 8/12 relapsed. Of the 8 relapses, 3 achieved a 2nd CR. Three patients with localised disease were high risk. Among them only one patient achieved CR after treatment, but all 3 -pts relapsed and 2 died. Among 6 patients with dissemination and standard risk 5/6 achieved CR, 1/6 PR, no relapses occurred. Bifocal localised non-Germinoma are not sufficiently controlled by chemotherapy and focal tumor boost. Other treatment concepts including upfront chemo-intensification in standard risk patients and whole-ventricular RT need to be explored. For the SIOP CNS GCT group, supported in part by German Childhood Cancer Aid
Calaminus et al. (Tue,) studied this question.