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Choroid Plexus Tumors

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Molecular Pathology of Nervous System Tumors

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.

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Venneti, S., Hasselblatt, M., Wolff, J.E., Judkins, A.R. (2015). Choroid Plexus Tumors. In: Karajannis, M., Zagzag, D. (eds) Molecular Pathology of Nervous System Tumors. Molecular Pathology Library, vol 8. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-1830-0_12

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