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

purposeChoroid plexus tumors (CPT) are rare entities, and even rarer in adulthood.

methodsA retrospective consecutive series of 24 adult CPT patients was reviewed.

resultsWe 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).

conclusionsWe 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.