Final Diagnosis -- Oncocytic variant of choroid plexus papilloma



Choroid plexus papilloma (CPP) is an uncommon, slowly-growing, usually benign intracranial neoplasm that is commonly located in the ventricular system. It may demonstrate various types of metaplastic changes. The stroma may exhibit focal metaplastic changes such as xanthomatous change, calcification and rarely ossification. The epithelial component may show mucinous metaplasia, pigmentation with presence of neuromelanin, ependymal differentiation or oncocytic transformation. The oncocytic variant of choroid plexus papilloma is rare and has been reported in seven cases to date in the international literature, to the best of our knowledge (1, 2,3,4,5).

The term 'oncocyte' was introduced by Hamperl in 1931 (6) to describe epithelial cells that appear large with abundant eosinophilic granular cytoplasm. Oncocytic transformation has been observed in various pathologic and non-pathologic conditions possibly as a consequence of compensatory mitochondrial hyperplasia due to defects of oxidative metabolism (7). Although oncocytic transformation may occur in almost all tumors, it is more commonly seen in renal, thyroid, parathyroid, and salivary gland tumors. On electron microscopic examination all oncocytes have in common a large number of mitochondria.

Oncocytic transformation is rare in intracranial tumors with the exception of pituitary adenomas. However, a few cases of oncocytic meningioma have been described in the literature (8). Kepes (9) reported oncocytic transformation of choroid plexus epithelium in all the cerebral ventricles in a 27-year old woman who suffered from adult form of Leigh's disease and expired.

Choroid plexus papilloma is an uncommon tumor in the brain. It is most common in children and usually is in the lateral ventricles, while in adults it typically arises in the fourth ventricle. The oncocytic variant of choroid plexus papilloma is extremely rare and a report published by Stefanko in 1985 (5) appears to be the first one describing the occurrence of oncocytic transformation in choroid plexus papilloma. Since then only seven cases have been reported in the literature. The oncocytic variant has been found to occur more often in older individuals and the fourth ventricle has been the most common site among these cases.

Focal positivity for GFAP has been observed in choroid plexus papillomas and choroid plexus carcinomas in 35-40% of cases (4). It has been suggested that focal GFAP positivity indicates glial or possibly ependymal differentiation in the choroid plexus epithelium. GFAP-positive cells are normally not seen in choroid plexus epithelium.

Choroid plexus papilloma is usually a benign W.H.O grade I tumor with low or absent mitoses and without invasion of the adjacent brain (10). Tumors with increased mitotic activity are likely to behave in an aggressive manner. Since only a few cases have been reported in literature the biological behavior of oncocytic variant is unclear. Malignant transformation of an oncocytic choroid plexus papilloma has been reported by Diengdoh and Shaw (2). Additional cases may help to ascertain the biological behavior of this tumor.


  1. Buccoliero AM, Bacci S, Mennonna P, Taddei GL. Pathologic quiz case: infratentorial tumor in a middle-aged woman. Oncocytic variant of choroid plexus papilloma. Arch Pathol Lab Med. 2004 Dec;128(12):1448-50.
  2. Diengdoh JV, Shaw MDD. Oncocytic variant of choroid plexus papilloma: evolution from benign to malignant "oncocytoma.". Cancer 1993;71:855-858.
  3. Muller W, Reifferscheidt I, Firsching R. Oncocytic transformation in papilloma of the choroid plexus. Light-microscopic study of 2 cases. Neuropatol Pol 1987;25:205-9.
  4. Bonnin JM, Colon LE, Morawetz RB. Focal glial differentiation and oncocytic transformation in choroid plexus papilloma. Acta Neuropathol Berl 1987;72:277-280.
  5. Stefanko SZ, Vuzevski VD. Oncocytic variant of choroid plexus papilloma. Acta Neuropathol Berl 1985;66:160-162.
  6. Hamperl H (1931 b) Onkocyten and Geschwulste der Speicheldrusen.. Virchows Arch [Path Anat] 282;724-36.
  7. Nappi O, Ferrara G, Wick MR. Neoplasms composed of eosinophilic polygonal cells: an overview with consideration of different cytomorphologic patterns. Sem Diagn Pathol 1999;16:82-90.
  8. Caldarella A, Buccoliero AM, Marini M, Taddei A, Mennonna P, Taddei GL. Oncocytic meningioma: A case report. Pathol Res Pract 2002; 198:109-113.
  9. Kepes JJ. Oncocytic transformation of choroid plexus epithelium. Acta Neuropathol Berl 1983;62:145-8.
  10. Kleihues P, Cavenee WK. eds. Pathology and Genetics of Tumours of the Nervous System. Lyon, France: IARC Press; 2000. World Health Organization Classification of Tumours; vol 2.

Contributed by Hanni Gulwani, DNB, MRCP and Subimal Roy, MD, PhD

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