Choroid Plexus Tumors
Choroid plexus tumors are rare, predominantly intraventricular tumors that occur in children. Pathologically they are classified as choroid plexus papillomas that are benign and almost always treated by surgery alone, choroid plexus carcinomas that are malignant aggressive tumors, and the intermediary atypical choroid plexus papillomas. There is a strong association between choroid plexus carcinoma and TP53 mutations. These tumors present with hydrocephalus or macrocephaly. They are challenging surgically as their high vascularity often leads to significant blood loss in small children with a low circulating blood volume. This can be mitigated by preoperative embolization or neoadjuvant chemotherapy. Maximal safe surgical resection is central to effective treatment, and most multivariate analyses have underlined the importance of gross total resection. Second-look surgery has also been associated with improved prognosis. Choroid plexus carcinomas also require adjuvant chemotherapy and consideration of radiotherapy. However, the role of radiotherapy is not clear; in view of the large size of these tumors, the young age of most patients, and the relatively high frequency of underlying tumor-predisposing conditions such as Li-Fraumeni syndrome, radiotherapy should be avoided in a high proportion of cases. Overall survival in most series of choroid plexus carcinomas has been poor.
The rarity of these tumors has made the development of clear guidelines challenging. Most case series are small. Retrospective analyses from epidemiological databases or meta-analyses from published series are compromised by incomplete individual data or multiple representations of the same patients. The value of central discussion of these patients with a specialist choroid plexus tumor interest group cannot be overemphasized. Recent studies have improved understanding of the genetic landscape of these tumors and should hopefully lead on to clinical trials of targeted agents in the near future.
KeywordsChoroid plexus tumor Choroid plexus papilloma Choroid plexus carcinoma Atypical choroid plexus papilloma
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