ID #1143 Choroid Plexus Tumors in Latin America: Diagnostic and Therapeutic Challenges
Andrea M Cappellano, Natalia Dassi, Jessica Rodrigues, Tatiana El jaick Costa, Regina M Navarro, Flávia D Martins, Gabriela Oigman, Thais Junqueira, Alayde Vieira, Nasjla S SilvaAbstract
Introduction
There are limited data on the incidence and clinical characteristics of patients with choroid plexus tumors (CPT) in Latin America.
Methods
Cross-sectional study based on questionnaire-derived database analysis.
Results
Six centers from Brazil and one from Mexico participated, contributing data from 111 patients. Most centers were part of the public health system and had intensive care units and pediatric neurosurgery available. CPT cases were classified into the following pathological subtypes: 61 choroid plexus carcinoma (CPC), 40 choroid plexus papilloma (CPP), and ten atypical choroid plexus papilloma (aCPP). Among CPC cases, 31 (50.8%) patients were female. The median age at diagnosis was 10.2 months (range: 1–204 months), and 21 patients (34.4%) had metastatic disease at diagnosis. All patients underwent neurosurgical treatment, with four receiving neoadjuvant chemotherapy. Gross total resection was achieved in 18 cases, and significant intraoperative bleeding occurred in 14 cases. Immunohistochemistry was available for 45 cases (73.8%), including Ki-67 and p53 data. Thirty-one cases underwent somatic and germline molecular analyses, of which 20 (64.5%) harbored the TP53 R337H mutation. The most commonly used chemotherapy protocols were CPT-SIOP, Head Start, and ICE. Ten patients (16.4%) also received radiotherapy as part of initial treatment. Relapses after first-line treatment occurred in 26 patients (42.6%), and 26 patients (42.6%) were alive at last follow-up.
Conclusion
This study underscores the urgent need for robust data registries in Latin America, in addition to addressing treatment heterogeneity and the limited access to molecular diagnostic techniques, particularly in the context of rare brain tumors such as choroid plexus tumors and those associated with genetic cancer predisposition syndromes.