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Current management of choroid plexus carcinomas

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Abstract

Choroid plexus carcinoma (CPC) is a World Health Organization (WHO) grade III brain tumor with a poor prognosis that occurs mainly in children. Gross total resection of CPC is highly recommended and is associated with improved overall survival, although it is often associated with increased morbidity. The use of adjuvant therapies has yet to be standardized, although evidence suggests that for patients with incompletely resected CPCs, a combination of chemotherapy and radiation therapy may be beneficial. The use of radiation therapy for younger children (<3 years old) with CPC, however, is not recommended, due to the potential negative neurological sequelae associated with radiation to the developing brain. Given that the majority of CPC patients are young children, questions regarding optimal radiation dose, chemotherapy agents, and how to combine these two adjuvant treatment modalities to achieve the best outcomes remain unanswered. In this paper we summarize the current management of CPC in the literature. Further studies are needed to standardize the treatment paradigm for this malignant brain tumor.

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Acknowledgment

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Conflict of interest

The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

Funding

M.Z. Sun is a Howard Hughes Medical Institute medical research fellow. M. Safaee was supported by a grant from the Doris Duke Charitable Foundation. Dr. Oh received a National Research Service Award from the National Institutes of Health (F32NS073326-01). Dr. Ivan is funded by the National Research Education Foundation through the American Association of Neurological Surgeons. Dr. Parsa is partially funded by the Reza and Georgianna Khatib Endowed Chair in Skull Base Tumor Surgery.

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  1. Department of Neurological Surgery, University of California San Francisco, 505 Parnassus Ave., San Francisco, CA, 94117, USA

    Matthew Z. Sun, Michael C. Oh, Michael E. Ivan, Gurvinder Kaur, Michael Safaee, Joseph M. Kim, Joanna J. Phillips & Kurtis I. Auguste

  2. Department of Pathology, University of California San Francisco, San Francisco, CA, USA

    Joanna J. Phillips

  3. Department of Neurological Surgery, Northwestern University, Feinberg School of Medicine, 676 N. St. Clair Street, Suite 2210, Chicago, IL, 60611-2922, USA

    Andrew T. Parsa

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  1. Matthew Z. Sun
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Comments

Concezio Di Rocco, Hannover, Germany

Choroid plexus carcinomas (CPC) remain one of the few tumors of the CNS which did not benefit of the progresses that have been recorded in surgical and medical management in the last decades. Similarly to what is observed in case of high-grade cerebral ependymomas, another tumor that continues being a therapeutic challenge, the surgical excision still constitutes the most effective therapeutic option and the Ki-67 index the most reliable prognostic predictor in spite of refinements in multimodal therapeutic strategies and the advances made in molecular biology. The extreme rarity of this tumor prevents the oncologist to collect sufficiently large series in a relatively short period of time in order to evaluate the specific role of various factors that can influence the late outcome, such as the age of the patient, the location of the tumor, its invasiveness of surrounding structures, the presence of an associated hydrocephalus, the extent of surgical removal, and the effect of adjuvant treatments. The persistently high rate of failure in the management of CPC, even when multiple therapeutic modalities are adopted, is emphasized in this accurate review by Sun and co-workers. The authors point on the still existing difficulties in differentiating these tumors preoperatively from the more benign and common choroid plexus papillomas (CPP) on the grounds of clinical manifestation as well as neuroimaging. In clinical practice such a limitation may impact on the therapeutic planning as the surgeon is faced with an obvious impairment in predicting the prognosis and in offering the family of the young patient a variety of therapeutic options without the possibility of reliably predicting their effective role. Actually, according to the authors of this study, none of the following therapeutic approaches can be suggested as the best or at least necessary option in the majority of the cases, namely radical versus partial surgical excision, the adoption of a surgical two-stage tumor removal, the attempt of preoperatively embolizing the tumor-feeding arteries, the use of preoperative chemotherapy, and, in case of incomplete surgical removal, the decision for a second look procedure or for an adjuvant radio- and/or chemotherapy. In many cases, in fact, the young patient age and the specific tumor characteristics constitute considerable obstacles for satisfactory management. This difficulty is quite obvious when reading the present review that clearly demonstrates the insufficiency of currently available data on which the surgeon should base his/her therapeutic planning. Indeed, most of the reported experiences analyzed by the authors are often discordant and concerning small-sized clinical series or even anecdotal cases. Even the surgical decision to pursue a total excision of the tumor versus a partial but safer removal can be questioned, in spite of the fact that radical tumor excision is associated with longer survival in the majority of cases. In fact, this advantage, often consisting of merely prolonging the survival for a few more months, should be weighed against the risk of inflicting additional functional damage to the still developing brain by tumor infiltration of the subependymal parenchyma. A similar caution can be applied to the use of radiotherapy and, perhaps to a lesser extent, of chemotherapy.

In summary, the authors should be congratulated for their significant effort to provide an objective picture of the current status in the management of CCP, although their conclusions are far from offering optimistic future perspectives. They rather confirm the existing pessimistic attitude that characterizes the management of these formidable tumors and the persisting limits in opposing their homonymous evolution in the majority of our children.

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Sun, M.Z., Oh, M.C., Ivan, M.E. et al. Current management of choroid plexus carcinomas. Neurosurg Rev 37, 179–192 (2014). https://doi.org/10.1007/s10143-013-0499-1

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