LAUSR

While rare cancers have provided insight into mechanisms of tumorigenesis and familial syndromes, patients with rare CNS tumors and providers face unique challenges, including diagnostic delays and errors, lack of standardized approaches to care/treatment, and limited social support. Scant data exists on the natural history, health status, and impact of these tumors on life quality. This report describes an innovative clinic designed to fulfill the mission of the NCI-CONNECT program: To advance our understanding and to improve approaches to care and treatment of rare CNS tumors. A specialized weekly clinic was established incorporating routine comprehensive analysis of tumor tissue, genetic counseling with individual pedigree assessment, group meetings with a health and wellness counselor and comprehensive clinical evaluation. Patients and caregivers both participated. Descriptive statistics report of NCI-CONNECT clinic patients from January 1-May 8, 2019 are presented. 33 patients and 32 caregivers have participated to date, representing 6 of the 12 rare tumors studied in NCI-CONNECT: 19 ependymomas, 9 oligodendrogliomas, 2 PXAs, 1 papillary pineal tumor, 1 medulloblastoma, 1 gliosarcoma. The median time from receiving pathologic material to appointment was 22 days, with analysis including methylation and next generation sequencing testing. All 33 patients underwent genetic counseling, most (n=26) having at least ≥ 2 first- or second-degree relatives diagnosed with cancer (Germline mutations/genetic predisposition testing underway). 44 patients attended (33 with 8 repeat visits) the health and wellness group sessions, with education on coping/wellness techniques included. During this same period, 7 patients participated in a rare CNS basket trial using nivolumab. We have developed an innovative clinic model to interrogate rare CNS tumors for therapeutic targets, understand individual risk, how the patient feels and functions and conduct clinical trials. This effort has the potential to accelerate our understanding of these rare tumors and provide guidance on care.