gms | German Medical Science

Objective: Choroid plexus tumors (CPT) are rare and highly vascularized tumors of the ventricular system. Most tumors occur in the pediatric population while the incidence in adults is extremely rare. Optimal management is still not well defined.

Method: Between 1987 and 2014 11 adult patients with CPT were treated at our institution and included in this retrospective analysis. Patient demographics, clinical presentation, imaging data, histology and surgical treatment with related morbidity was analyzed from hospital records. Clinical outcome was assessed using the modified Rankin Scale (mRS) and Spitzer Index for quality of life (SQLI).

Results: 6 males and 5 females with a mean age of 46 years (range 24 to 73) were included. Most frequent clinical presentation was headache (45%) followed by hydrocephalus, unsteady gait and vision disorders (36% each). A total of 16 tumor resections were performed using suboccipital (14), frontal transcortical (1) and frontotemporal (1) approaches. Tumors were located in the forth ventricle (14) lateral ventricle (1) and one recurrent tumor in the tuberculum sellae (1). Gross total resection (GTR) was achieved in 14/16 operations (87.5%) and subtotal resection in 2/16 operations (12.5%) due to parenchymal infiltration. With no surgical mortality the morbidity consisted of 12.5% transient and 12.5% permanent deficits (one case epilepsy and another with a CN III paresis). Follow-up period ranged from 3 to 150 months (mean 76 months). At last follow-up the mean mRS (0-6) was 0.8 and SQLI (0-10) was 8.3. 4/16 resected tumors were classified histologically as atypical (aCPT WHO II). Mean Proliferation Index was 3.8 (range 1 to 8). Overall recurrence rate was 4/11 (36%) with one patient having multiple recurrences who underwent adjuvant radiotherapy after the last resection. Only 2 out of 8 patients with inital CPT WHO I developed recurrent tumors whereas recurrence was observed in 2 out of 3 patients with initial aCPT WHO II. Both patients with initial subtotal resection developed a tumor progress over time while the mean progression free survival in this cohort was 4.2 years.

Conclusions: GTR achieves long-term control in CPT with minimal morbidity. Long-term follow-up is warranted especially in patients with a subtotally resected tumor or in those with atypical CPT WHO II.