Choroid Plexus Neoplasm

Abstract: Choroid plexus tumours are rare epithelial brain tumours and limited information is available regarding their biology and the best treatment. A meta-analysis was done to determine prognostic factors and the influence of various treatment modalities. A thorough review of the medical literature (1966–1998) revealed 566 well-documented choroid plexus tumours. These were entered into a database, which was analysed to determine prognostic factors and treatment modalities. Most patients with a supratentorial tumour were children, while the most common sites in adults were the fourth ventricle and the cerebellar pontine angle. Cerebellar pontine angle tumours were more frequently benign. Histology was the most important prognostic factor, as one, five, and 10-year projected survival rates were 90, 81, and 77% in choroid plexus-papilloma (n=353) compared to only 71, 41, and 35% in choroid plexus-carcinoma respectively (P<0.0005). Surgery was prognostically relevant for both choroid plexus-papilloma (P=0.0005) and choroid plexus-carcinoma (P=0.0001). Radiotherapy was associated with significantly better survival in choroid plexus-carcinomas. Eight of 22 documented choroid plexus-carcinomas responded to chemotherapy. Relapse after primary treatment was a poor prognostic factor in choroid plexus-carcinoma patients but not in choroid plexus-papilloma patients. Treatment of choroid plexus tumours should start with radical surgical resection. This should be followed by adjuvant treatment in case of choroid plexus-carcinoma, and a ‘wait and see’ approach in choroid plexus-papilloma. British Journal of Cancer (2002) 87, 1086–1091. doi:10.1038/sj.bjc.6600609 www.bjcancer.com © 2002 Cancer Research UK

Abstract: Purpose Choroid plexus carcinomas are pediatric tumors with poor survival rates and a strong, but poorly understood, association with Li-Fraumeni syndrome (LFS). Currently, with lack of biologic predictors, most children are treated with aggressive chemoradiation protocols. Patients and Methods We established a multi-institutional tissue and clinical database, which enabled the analysis of specific alterations of the TP53 tumor suppressor and its modifiers in choroid plexus tumors (CPTs). We conducted high-resolution copy-number analysis to correlate these genetic parameters with family history and outcome. Results We studied 64 patients with CPTs. All individuals with germline TP53 mutations fulfilled LFS criteria, whereas all patients not meeting these criteria harbored wild-type TP53 (P .001). TP53 mutations were found in 50% of choroid plexus carcinomas (CPCs). Additionally, two sequence variants known to confer TP53 dysfunction, TP53 codon72 and MDM2 SNP309, coexisted in the majority of TP53 wild-type CPCs (92%) and not in TP53 mutated CPC (P .04), which suggests a complementary mechanism of TP53 dysfunction in the absence of a TP53 mutation. Highresolution single nucleotide polymorphism (SNP) array analysis revealed extremely high total structural variation (TSV) in TP53-mutated CPC tumor genomes compared with TP53 wild-type tumors and choroid plexus papillomas (CPPs; P .006 and .004, respectively). Moreover, high TSV was associated with significant risk of progression (P .001). Five-year survival rates for patients with TP53-immunopositive and -immunonegative CPCs were 0% and 82 ( 9%), respectively (P .001). Furthermore, 14 of 16 patients with TP53 wild-type CPCs are alive without having received radiation therapy.

Abstract: OBJECTIVE: Choroid plexus carcinomas are rare tumors with dismal prognosis. The role of surgery has been well established, but the benefit of either chemotherapy or radiotherapy remains controversial. To determine prognostic factors and effects of different therapeutic modalities on the outcome, we have reviewed the French experience of choroid plexus carcinoma. METHODS: Twenty-two children were registered in the Societe Francaise d'Oncologie Pediatrique between 1984 and 1995. All these children underwent surgical resection of the primary tumor. The intent of postoperative treatment was to delay or to avoid radiation therapy. Nineteen children received postoperative treatment, with chemotherapy in 17 and radiation therapy in 2. Two responding patients underwent high-dose chemotherapy with stem cell rescue. RESULTS: The 5-year survival rate was 26%. The sole relevant prognostic factor was the extent of surgery. Patients with total or gross total resection had a 86% survival rate. Survival did not correlate with age, sex, delay between first appearance of symptoms and diagnosis, location of the primary tumor, tumor volume, or response to postoperative treatment. All but one patient with incomplete surgery had tumor recurrence within 2 to 23 months. CONCLUSION: Choroid plexus carcinoma has a very poor prognosis when surgery is incomplete. Aggressive surgical resection of the tumor is necessary for survival. Although chemotherapy gives promising responses, local control remains the main challenge, and second look surgery has to be considered for patients with incomplete resection.