Choroid plexus tumors in adults: a retrospective mono-institutional study

Abstract

Purpose Choroid plexus tumors (CPT) are rare entities, and even rarer in adulthood. Methods A retrospective consecutive series of 24 adult CPT patients was reviewed. Results We described 24 adult CPTs. Clinical onset included cerebellar signs (n = 11, 45.8%), intracranial hypertension signs (n = 8, 33.4%), cranial nerves impairment (n = 5, 20.8%), incidental findings (n = 4, 16.6%), seizures (n = 1, 4.2%), spinal signs (n = 1, 4.2%). At first diagnosis, CPT was mostly located in the ventricular system, but other locations can occur, including the spine (one case); meningeal involvement was present in one, pre-surgical hydrocephalus in one case only. CPT histological grade ranged from grade 1 (n = 17), grade 2 (n = 6), and grade 3 (n = 1). TERTp mutation was detected in 17.6% (n = 3/17). TP53 mutation in 5.9% (n = 1/17). Gross Total, Subtotal, Partial resection and Biopsy were achieved in 17 (70.8%), 3 (12.5%), 3 (12.5%) and 1 (4.2%) of patients, respectively. 76% of cases (n = 16/21) experienced clinical worsening suddenly after surgery for different reasons, and mostly gradually recovered. For three cases no data was available. Adjuvant therapy was performed only for grades 2 and 3. At recurrence, surgery, radiosurgery, radiotherapy and chemotherapy were considered. The median Overall Survival from surgery was 219.25 months (95% CI, 188.83–249.67). Conclusions We confirm that CPT can occur in adults and are mostly grade 1 tumors located in the ventricular system. The surgical approach is the gold standard, although 76% of clinical worsening occurred, often transient. Adjuvant treatment was limited to higher grade CPT; however, no consensus has already been achieved about adjuvant therapy.