Choroid plexus papillomas are rare neuroepithelial tumors found mainly in children. Although well studied in the pediatric population, there is a paucity of literature focusing specifically on adults. We sought to assess the relative advantage of gross total resection (GTR) and further characterize the natural history of this disease in adults. A comprehensive PubMed search was performed to identify adults who underwent surgical resection for choroid plexus papillomas with clearly reported age, tumor location, and extent of resection. Kaplan-Meier analysis was used to assess progression-free survival (PFS) and overall survival (OS). Multivariate analysis was performed using Cox proportional hazards models. A total of 193 patients were identified with a mean age of 39.9±1.1years. GTR was achieved in 72% of patients with subtotal resection (STR) in 28%. GTR was associated with a significant increase in both PFS (p=0.015) and OS (p=0.004) compared to STR. In a multivariate Cox proportional hazards model we found that only GTR was associated with recurrence (hazard ratio [HR]=0.47, 95% confidence interval [CI] 0.25-0.90), while both age (HR=1.03, 95% CI 1.00-1.05) and GTR (HR=0.36, 95% CI 0.17-0.78) were associated with OS. Interestingly, our observed recurrence and death rates were higher than those in previously published studies. These findings demonstrate the benefit of GTR for the treatment of choroid plexus papillomas in adults. Our analysis suggests that these lesions are not as indolent as previously thought and while GTR is preferred, it is not always curative.
[Show abstract][Hide abstract] ABSTRACT: Choroid plexus tumors (CPTs) are intraventricular lesions originating from ventricular neuroepithelium and represent up to 4 % of brain neoplasms affecting pediatric population. They are more frequently benign papillomas, but malignant carcinomas can sometimes occur.
The authors present a description of surgical approach for CPTs, particularly focusing on the complications related to the cerebrospinal fluid (CSF) circulation, which may affect outcome.
Microsurgical resection represents the first line treatment for CPTs. The goal is the complete removal of the tumor and the restoration of a physiological CSF circulation.
[Show abstract][Hide abstract] ABSTRACT: Background:
Choroid plexus tumors are rare brain tumors with clinical features that vary according to the histological grade. We reviewed the treatment outcomes of 15 adult patients with choroid plexus tumors, focusing on surgical outcomes and current therapeutic strategies.
Patient demographic and clinical characteristics, operative findings, adjuvant therapies, disease progression and survival rates were reviewed.
The median age at diagnosis was 33.7 ± 10 years (19-59 years) for patients with choroid plexus tumors. Postoperative chemotherapy was given to 26.7 % of patients, and 13.3 % of patients received radiotherapy. The Ki-67 labeling index and mitotic index increased at higher histological grades. All of the choroid plexus papilloma and atypical choroid plexus papilloma patients have survived. The overall survival rate of patients with choroid plexus carcinoma was 50 % in the first year, but none of the patients survived to the second year. Five patients underwent permanent cerebrospinal fluid diversion surgery because of hydrocephalus or subdural effusion.
Choroid plexus papilloma and atypical choroid plexus papilloma patients can be treated with complete surgical resection. Choroid plexus carcinoma has a poor prognosis, and aggressive multi-modal treatments are generally needed for treatment. Chemotherapy and radiotherapy are important adjuvant therapies for choroid plexus carcinoma. If hydrocephalus and/or subdural effusion occur, permanent cerebrospinal fluid (CSF) diversion should be added to the therapeutic strategy.
[Show abstract][Hide abstract] ABSTRACT: Choroid plexus tumors (CPTs) are rare neoplasms arising from the choroid plexus epithelium, and occur predominantly in infants and children. CPTs may be associated with TP53 germline mutations (Li–Fraumeni syndrome), but the majority of CPTs are sporadic. Most commonly, CPTs present with signs of increased intracranial pressure; on imaging they are characterized as intraventricular contrast-enhancing masses. Histopathologically, CPTs comprise benign choroid plexus papilloma (CPP, WHO grade I), atypical choroid plexus papilloma (APP, WHO grade II), and malignant choroid plexus carcinoma (CPC, WHO grade III). Complete surgical resection may be curative for CPPs, while CPCs are additionally treated with chemotherapy and radiation. In this chapter we discuss these features in detail, as well as differential diagnosis and immunohistochemical studies. Particular emphasis is placed on the molecular alterations, how they can be detected in the laboratory using immunohistochemistry and molecular testing, and how they may lead to novel-targeted therapeutic approaches.
01/2015: pages 163-175;
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