Abstract
While choroid plexus tumors represent less than 1% of CNS tumors overall, they are responsible for a much greater proportion of CNS tumors in the early years of life. Of 170 newborn brain tumors (reported from twelve institutions), 12% were choroid plexus papillomas (CPPs) (1). CPPs preferentially involve the lateral ventricles in children, while the fourth ventricle is most often involved in adults. CPPs have occasionally arisen within the third ventricle (2). Presentation as a cerebellopontine angle tumor is also unusual. In the latter location, CPPs may either arise from the choroid plexus emanating from the foramen of Lushka, extend out from other locations within the fourth ventricle, or represent leptomeningeal dissemination (3). Bilaterality is distinctly unusual, may result in congenital hydrocephalus, has been reported in infants with duplication of chromosome 9p, and is also referred to as hyperplasia or villous hypertrophy of the choroid plexus (4). CPPs usually present with headaches, most often secondary to increased intracranial pressure as a result of excessive CSF production by the overgrown choroid plexus epithelium. Retention of this functional capacity is accompanied by retention of most of the morphologic characteristics of normal choroid plexus epithelium. While the usual cytologic and architectural features of low-grade papillary tumors (overgrowth, crowding, nuclear hyperchromasia and pleomorphism) may or may not be apparent, a fairly reliable feature of CPPs is loss of the normal hobnail appearance of nonneoplastic choroid plexus epithelium (Figs. 18-1 and 18-2). CPPs may, on occasion, demonstrate epithelial variations including tubule formation (5), oncocytic change, and pigmentation (6). Stromal lamellar calcification may frequently be seen within CPPs and may be so heavy as to interfere with tissue sectioning (7).
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References
Isaacs, H. (1997) Tumors of the fetus and newborn. Major Probl. Pathol. 35: 187–228.
Nakano, I., Kondo, A., Iwasaki, K. (1997) Choroid plexus papilloma in the posterior third ventricle: case report. Neurosurgery 40: 1279–1282.
Tacconi, L., Delfini, R., Cantore, G. (1996) Choroid plexus papillomas: consideration of a surgical series of 33 cases. Acta Neurochir. 13 8: 802–810.
Norman, M.G., Harrison, K.J., Poskitt, K.J., Kalousek, D.K. (1995) Duplication of 9p and hyperplasia of the choroid plexus: a pathologic, radiologic and molecular cytogenetics study. Pediatr. Pathol. Lab. Med. 15: 109–120.
Varga, Z., Vajtal, I., Marino, S., Schauble, B., Yonekawa, Y., Aguzzi, A. (1996) Tubular adenoma of the choroid plexus: evidence for glandular differentiation of the neuroepithelium. Pathol. Res. Pract. 192: 840–844.
Reimund, E.L., Sitton, J.E., Harkin, J.C. (1990) Pigmented choroid plexus papilloma. Arch. Pathol. Lab. Med. 114: 902–905.
McGirr, S.J., et al. (1988) Choroid plexus papillomas: longterm follow-up results in a surgically treated series. J. Neurosurg. 69: 843–849.
Furness, P.N., Lowe, J., Tarrant, G.S. (1990) Subepithelial basement membrane deposition and intermediate filament expression in choroid plexus neoplasms and ependymomas. Histopathology 16: 251–255.
Ang, L.C., Taylor, A.R., Bergin, D., Kaufmann, C.E. (1990) An immunohistochemical study of papillary tumors in the central nervous system. Cancer 65: 2712–2719.
Pencalet, P., et al. (1998) Papillomas and carcinomas of the choroid plexus in children. J. Neurosurg. 88: 521–528.
Berger, C., Thiesse, P., Lellouch-Tubiana, A., Kalifa, C., Pierre-Kahn, A., Bouffet, E. (1998) Choroid plexus carcinomas in childhood: clinical features and prognostic factors. Neurosurgery 42: 470–475.
Kleihues, P., Burger, P.C., Scheithauer, B.W. (1993) Histological Typing of Tumours of the Central Nervous System. (2’ ed.) New York: Springer-Verlag.
Leblanc, R., Bekhor, S., Melanson, D., Carpenter, S. (1998) Diffuse craniospinal seeding from a benign fourth ventricle choroid plexus papilloma. J. Neurosurg. 88: 757–760.
Russell, D.S., Rubinstein, J.L. (1989) Pathology of Tumours of the Nervous System. Revised by L.F. Rubenstein, 51h ed. Baltimore: Williams and Wilkins.
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Prayson, R.A., Cohen, M.L. (2000). Choroid Plexus Tumors. In: Practical Differential Diagnosis in Surgical Neuropathology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-59259-037-7_18
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DOI: https://doi.org/10.1007/978-1-59259-037-7_18
Publisher Name: Humana Press, Totowa, NJ
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