Papillary Tumor of the Pineal Region: Diagnosis

  • Hirohito Yano
  • Toru Iwama
Part of the Tumors of the Central Nervous System book series (TCNS, volume 10)


Papillary tumor of the pineal region (PTPR) has been recently included in the 2007 World Health Organization (WHO) “Classification of tumors of the central nervous system.” PTPRs are considered to originate from the subcommissular organ (SCO), which consists of secretory ependymocytes. The origin of the tumor is reflected in the neuroradiological, microscopic, immunohistochemical, and ultrastructural findings. In non-contrast T1-weighted magnetic resonance imaging (MRI) scans, the lesion is represented as a heterogeneous hyperintense area due to the presence of secretory glycoprotein inclusions. PTPRs are most frequently misdiagnosed as ependymomas or choroid plexus (CP) tumors owing to the morphological similarity among these tumors. PTPRs are histologically characterized by loose papillary and densely cellular diffuse, patternless areas showing pseudorosettes with fibrovascular cores covered by several layers of columnar or cuboidal cells. PTPRs are composed of a greater number of epithelial cells than that in ependymomas and lesser number of papillary cells than choroid plexus (CP) tumors. Characteristic immunohistochemical findings of PTPRs include characteristic small ring- and dot-like staining patterns indicative of cytokeratin 18 immunoreactivity, which are similar to those obtained for CP tumors. Furthermore, both PTPRs and CP tumors showed focal transthyretin (prealbumin; TTR) immunoreactivity. On the other hand, PTPRs and ependymomas occasionally showed immunoreactivities to epithelial membranous antigen (EMA), glial fibrillary acidic protein (GFAP), and neural cell adhesion molecule (NCAM). In addition to the abovementioned makers, strong diagnostic markers for PTPRs and factors that distinguish PTPRs from ependymomas or CP tumors are expression of neuronal markers, including microtubule-associated protein 2 (MAP2), neuron-specific enolase (NSE), and neuronal nuclei (NeuN). These findings can be attributed to the extensive involvement of SCO in neuronal differentiation. Ultrastructural examination of PTPRs showed the presence of microvilli and desmosomes—characteristics of ependymoma cells—and abundant rough endoplasmic reticulum, lipid droplets, etc.—characteristics of CP tumor cells.


Glial Fibrillary Acidic Protein Choroid Plexus Neural Cell Adhesion Molecule Epithelial Membranous Antigen Pineal Region 
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  1. Blümcke I, Müller S, Buslei R, Riederer BM, Wiestler OD (2004) Microtubule-associated protein-2 immunoreactivity: a useful tool in the differential diagnosis of low-grade neuroepithelial tumors. Acta Neuropathol 108:89–96PubMedCrossRefGoogle Scholar
  2. Boco T, Aalaei S, Musacchio M, Byrne R, Cochran E (2008) Papillary tumor of the pineal region. Neuropathology 28:87–92PubMedCrossRefGoogle Scholar
  3. Cenacchi G, Glangaspero F (2004) Emerging tumor entities and variants of CNS neoplasms. J Neuropathol Exp Neurol 63:185–192PubMedGoogle Scholar
  4. Chang AH, Fuller GN, Debnam JM, Karis JP, Coons SW, Ross JS, Dean BL (2008) MR imaging of papillary tumor of the pineal region. AJNR Am J Neuroradiol 29:187–189PubMedCrossRefGoogle Scholar
  5. Cruz-Sanchez FF, Rossi ML, Hughes JT, Coakham HB, Figols J, Eynaud PM (1989) Choroid plexus papillomas: an immunohistological study of 16 cases. Histopathology 15:61–69PubMedCrossRefGoogle Scholar
  6. Dagnew E, Langford LA, Lang FF, DeMonte F (2007) Papillary tumor of the pineal region: case report. Neurosurgery 60:953–955CrossRefGoogle Scholar
  7. Fevre-Montange M, Champier J, Szathmari A, Brisson C, Reboul A, Mottolese C, Fauchon F, Claustrat B, Jouvet A (2006) Prognosis and histopathologic features in papillary tumors of the pineal region: a retrospective multicenter study of 31 cases. J Neuropathol Exp Neurol 65:1004–1011PubMedCrossRefGoogle Scholar
  8. Figarella-Branger D, Lepidi H, Poncet C, Gambarelli D, Bianco N, Rougon G, Pellissier JF (1995) Differential expression of cell adhesion molecules (CAM), neural CAM, and epithelial cadherin in ependymomas and choroid plexus tumors. Acta Neuropathol 89:148–157CrossRefGoogle Scholar
  9. Hasselblatt M, Blümcke I, Jeibmann A, Rickert CH, Jouvet A, van de Nes JA, Kuchelmeister K, Brunn A, Fevre-Montange M, Paulus W (2006) Immunohistochemical profile and chromosomal imbalances in papillary tumors of the pineal region. Neuropathol Appl Neurobiol 32:278–283PubMedCrossRefGoogle Scholar
  10. Hirato J, Nakazato Y, Iijima M, Yokoo H, Sasaki A, Yokota M, Ono N, Hirato M, Inoue H (1997) An unusual variation of ependymoma with extensive tumor cell vacuolization. Acta Neuropathol 93:310–316PubMedCrossRefGoogle Scholar
  11. Jouvet A, Fauchon F, Liberski P, Saint-Pierre G, Didier-Bazes M, Heitzmann A, Delisle MB, Biassette HA, Vincent S, Mikol J, Streichenberger N, Ahboucha S, Brisson C, Belin MF, Fèvre-Montange M (2003) Papillary tumor of the pineal region. Am J Surg Pathol 27:505–512PubMedCrossRefGoogle Scholar
  12. Kawahara I, Tokunaga Y, Yagi N, Iseki M, Abe K, Hayashi T (2007) Papillary tumor of the pineal region-case report. Neuro Med Chir (Tokyo) 47:568–571CrossRefGoogle Scholar
  13. Kern M, Robbins P, Lee G, Watson P (2006) Papillary tumor of the pineal region—a new pathological entity. Clin Neuropathol 25:185–192PubMedGoogle Scholar
  14. Kuchelmeister K, Hügens-Penzel M, Jödicke A, Schachenmayr W (2006) Papillary tumor of the pineal region: histodiagnostic considerations. Neuropathol Appl Neurobiol 32:203–208PubMedCrossRefGoogle Scholar
  15. Lee MK, Rebhun LI, Frankfurter A (1990) Posttranslational modification of class III β-tubulin. Proc Natl Acad Sci USA 87:7195–7199PubMedCrossRefGoogle Scholar
  16. Louis DN, Ohgaki H, Wiestler OD, Cavence WK, Burger PC, Jouvet A, Scheithauer BW, Kleihues P (2007) The 2007 WHO classification of tumors of the central nervous system. Acta Neuropathol 114:97–109PubMedCrossRefGoogle Scholar
  17. Meiniel A (2001) SCO-spondin, a glycoprotein of the subcommissural organ/Reissner’s fiber complex: evidence of a potent activity on neuronal development in primary cell cultures. Microsc Res Tech 52:484–495PubMedCrossRefGoogle Scholar
  18. Nakamura H, Makino K, Kochi M, Nakazato Y, Kuratsu J (2009) Successful treatment of neoadjuvant therapy for papillary tumor of the pineal region. Brain Tumor Pathol 26:73–77PubMedCrossRefGoogle Scholar
  19. Preusser M, Laggner U, Haberler C, Heinzl H, Budka H, Hainfellner JA (2006) Comparative analysis of NeuN immunoreactivity in primary brain tumours: conclusions for rational use in diagnostic histopathology. Histopathology 48:438–444PubMedCrossRefGoogle Scholar
  20. Rodrigeus EM, Oksche A, Montecinos H (2001) Human subcommissural organ, with particular emphasis on its secretory activity during the fetal life. Microsc Res Tech 52:573–590CrossRefGoogle Scholar
  21. Roncaroli F, Scheithauer BW (2007) Papillary tumor of the pineal region and spindle cell oncocytoma of the pituitary: new tumor entities in the 2007 WHO classification. Brain Pathol 17:314–318PubMedCrossRefGoogle Scholar
  22. Shibahara J, Todo T, Morita A, Mori H, Aoki S, Fukayama M (2004) Papillary neuroepithelial tumor of the pineal region. A case report. Acta Neuropathol 108:337–340PubMedCrossRefGoogle Scholar
  23. Yano H, Oka N, Okumura A, Shinoda J, Sakai N (2003) Clinicopathological study of recurrent ependymoma in childhood (in Japanese). Nerv Syst Child 28:274–281Google Scholar
  24. Yano H, Ohe N, Nakayama N, Shinoda J, Iwama T (2009) Clinicopathological features from long-term observation of a papillary tumor of the pineal region (PTPR): a case report. Brain Tumor Pathol 26:83–88PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media Dordrecht 2013

Authors and Affiliations

  1. 1.Department of NeurosurgeryGifu University Graduate School of MedicineGifu CityJapan

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