Choroid plexus tumors: a review of 28-year experience

The aims of the study were to review the patients with choroid plexus tumor (CPT) treated in Slovenia between 1972-1999, to calculate the incidence of CPTs, and to evaluate treatment results in respect to tumor histology and mode of therapy. Cancer Registry of Slovenia database was used for patientś identification. Histological specimens and medical records were reviewed. Twelve patients (7 females, 5 males), 0.8-43 years old (median 6.1 years; <15 years: 10/12,83%) with CPT, representing 0.36% of all intracranial tumors registered during the period under study, were identified. Considering children less than 15 years old only, the corresponding percentage was 3.69. There were eight papillomas (CPPs) and four carcinomas (CPCs) with no difference in age distribution between the groups. Of seven patients with gross tumor resection in CPP group, one patient died of postoperative meningitis and one had local recurrence 1.6 years after surgery; the latter is disease-free 17.9 years after re-operation. One patient with macroscopic residue after surgery is alive and asymptomatic after 16.5 years. In the CPC-group, only the patient who received adjuvant BEP chemotherapy and craniospinal irradiation following incomplete surgery is alive with no signs of disease after 6.5 years. Ten-year disease-specific survival for all CPTs and for CPP subgroup was 73% and 100%, respectively. In Slovenia, CPTs represent 0.36% of intracranial tumors. In children, the incidence is 3.69%. CPPs are twice as frequent as CPCs. In CPPs, the treatment of choice is surgery alone. After incomplete surgery, wait-and-see policy seems to be justified. For CPCs, adjuvant multiagent chemotherapy and craniospinal radiotherapy following surgery should be considered.