Survival benefit for individuals with constitutional mismatch repair deficiency syndrome and brain tumors who undergo surveillance protocol. a report from the international replication repair consortium

Ercan, AB;Scheers, Isabelle;et.al.
(2020) 19th International Symposium on Pediatric Neuro-Oncology, ISPNO 2020 — Location: Nagano, Japan (13.December.2020)

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METHODS: We surveyed all confirmed CMMRD patients in the International Replication Repair Deficiency Consortium. A surveillance protocol consisting of frequent biochemical, endoscopic and imaging (CNS and total body MRI) studies were employed. Survival analyses and efficacy of each method were assessed. RESULTS: Surveillance data were collected from 105 CMMRD individuals from 41 countries. Of the 193 malignant tumors, CNS malignancies were the most common (44%). The surveillance protocol uncovered 49 asymptomatic tumors including 16 glioblastomas and medulloblastomas. Five-year overall survival was 89% for tumors discovered by surveillance, and 61% for symptomatic tumors (p<0.004). Similarly, 5-year survival was 82+/-11% and 24+/-6% for surveillance and non-surveillance of brain tumors (p=0.005). Yearly total body and q6 month brain MRI detected asymptomatic cancers in all but 3 symptomatic CNS gliomas. These were tumors uncovered when time between scans was >6 months as per protocol. Finally, of the low grade tumors identified asymptomatically, 5 were low grade gliomas. All of the low grade gliomas, which were not resected transformed to high grade tumors at a median of 1.6 ± 0.9 years. CONCLUSION: These data support a survival benefit in CMMRD patients undergoing a surveillance protocol. Adherence to protocol and resection of lower grade lesions may improve survival for patients with CNS tumors.
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Ercan, A., Scheers, I., & et al. (2020). Survival benefit for individuals with constitutional mismatch repair deficiency syndrome and brain tumors who undergo surveillance protocol. a report from the international replication repair consortium. 19th International Symposium on Pediatric Neuro-Oncology, ISPNO 2020, Nagano, Japan.