Pineal Region

  • César R. Lacruz
  • Javier Saénz de Santamaría
  • Ricardo H. Bardales
Part of the Essentials in Cytopathology book series (EICP, volume 13)


The pineal region is comprised by the pineal gland, posterior third ventricle, tela choroidea, and velum interpositum. Most pineal region-related neoplasms are germ cell tumors (mainly germinoma), followed by pineal parenchymal tumors and gliomas. Other tumor types that may occasionally occur in this region are meningioma, papillary tumor of the pineal region (PTPR), craniopharyngioma, atypical teratoid/rhabdoid tumor, pineal anlage tumor, and rhabdomyosarcoma. Such a variety of tumor types makes biopsy diagnosis of the tumor essential. Given its axial deep-seated localization, stereotactic- or endoscopic-guided biopsy is the method of choice for obtaining diagnostic tissue, and since biopsies are often of small size, the cytologic method is the most convenient for intraoperative consultation. This chapter reviews the cytomorphologic features and differential diagnosis of pineocytoma, pineal parenchymal tumor of intermediate differentiation, pineoblastoma, and PTPR.


Intraoperative brain assessment Cytology Squash preparation Smear preparation Tumors of the pineal region Pineal parenchymal tumors Pineocytoma PPTID Pineoblastoma PTPR Pineal cyst Retinoblastoma 1 gene DICER1 gene 

Suggested Reading

  1. Chang AH, Fuller GN, Debnam JM, Karis JP, et al. MR imaging of papillary tumor of the pineal region. Am J Neurodiol. 2008;29:187–9.CrossRefGoogle Scholar
  2. Clark AJ, Ivan ME, Sughrue ME, Yang I, et al. Tumor control after surgery and radiotherapy for pineocytoma. J Neurosurg. 2010;113:319–24.CrossRefGoogle Scholar
  3. De Kock L, Sabbaghian N, Druker H, Weber E, et al. Germ-line and somatic DICER1 mutations in pineoblastoma. Acta Neuropathol. 2014;128:583–95.CrossRefGoogle Scholar
  4. Fauchon F, Jouvet A, Paquis P, Saint-Pierre G, et al. Parenchymal pineal tumors: a clinicopathological study of 76 cases. Int J Radiat Oncol Biol Phys. 2000;46:959–68.CrossRefGoogle Scholar
  5. Fèvre-Montange M, Szathmari A, Champier J, Mokhtari K, et al. Pineocytoma and pineal parenchymal tumors of intermediate differentiation presenting cytologic pleomorphism: a multicenter study. Brain Pathol. 2008;18:354–9.CrossRefGoogle Scholar
  6. Goschzik T, Gessi M, Denkhaus D, Pietsch T. PTEN mutations and activation of the PI3K/Akt/mTOR signaling pathway in papillary tumors of the pineal region. J Neuropathol Exp Neurol. 2014;73:747–51.CrossRefGoogle Scholar
  7. Heim S, Sill M, Jones DT, Vasiljevic A, et al. Papillary tumor of the pineal region: a distinct molecular entity. Brain Pathol. 2016;26:199–205.CrossRefGoogle Scholar
  8. Jiménez-Heffernan JA, Bárcena C, Gordillo C, Cañizal JM. Cytologic features of papillary tumor of the pineal region: a case report showing tigroid background. Diagn Cytopathol. 2016;44:1098–101.CrossRefGoogle Scholar
  9. Jouvet A, Fauchon F, Liberski P, Saint-Pierre G, et al. Papillary tumor of the pineal region. Am J Surg Pathol. 2003;27:505–12.CrossRefGoogle Scholar
  10. Kreth FW, Schätz CR, Pagenstecher A, Faist M, Volk B, Ostertag CB. Stereotactic management of lesions of the pineal region. Neurosurgery. 1996;39:280–91.CrossRefGoogle Scholar
  11. Kumar P, Tatke M, Sharma A, Singh D. Histological analysis of lesions of the pineal region: a retrospective study of 12 years. Pathol Res Pract. 2006;202:85–92.CrossRefGoogle Scholar
  12. Lau SK, Cykowski MD, Desai S, Cao Y, Fuller GN, Bruner J, Okazaki I. Primary rhabdomyosarcoma of the pineal gland. Am J Clin Pathol. 2015;143:728–33.CrossRefGoogle Scholar
  13. Maiti TK, Arimappamagan A, Mahadevan A, Yasha TC, Pandey P, Santosh V. Rare pathologies in the posterior third ventricular region in children: case series and review. Pediatr Neurosurg. 2015;50:42–7.CrossRefGoogle Scholar
  14. Mena H, Armonda RA, Ribas JL, Ondra SL, Rushing EJ. Nonneoplastic pineal cysts: a clinicopathologic study of twenty-one cases. Ann Diagn Pathol. 1997;1:11–8.CrossRefGoogle Scholar
  15. Murali R, Scheithauer BW, Chaseling RW, Owler BK, Ng T. Papillary tumour of the pineal region: cytological features and implications for intraoperative diagnosis. Pathology. 2010;42:474–9.CrossRefGoogle Scholar
  16. Parwani AV, Baisden BL, Erozan YS, Burger PC, Ali SZ. Pineal gland lesions: a cytopathologic study of 20 specimens. Cancer. 2005;105:80–6.CrossRefGoogle Scholar
  17. Raleigh DR, Solomon DA, Lloyd SA, Lazar A, et al. Histopathologic review of pineal parenchymal tumors identifies novel morphologic subtypes and prognostic factors for outcome. Neuro-Oncology. 2017;19:78–88.CrossRefGoogle Scholar
  18. Regis J, Bouillot P, Rouby-Volot F, Figarella-Branger D, Dufour H, Peragut JC. Pineal region tumors and the role of stereotactic biopsy: review of the mortality, morbility, and diagnostic rates in 370 cases. Neurosurgery. 1996;39:907–12.PubMedGoogle Scholar
  19. Shimada K, Nakamura M, Kuga Y, Taomoto K, Ohnishi H, Konishi N. Cytologic feature by squash preparation of pineal parenchyma tumor of intermediate differentiation. Diagn Cytopathol. 2008;36:749–53.CrossRefGoogle Scholar
  20. Varikatt W, Dexter M, Mahajan H, Murali R, Ng T. Usefulness of smears in intra-operative diagnosis of newly described entities of CNS. Neuropathology. 2009;29:641–8.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2018

Authors and Affiliations

  • César R. Lacruz
    • 1
  • Javier Saénz de Santamaría
    • 2
  • Ricardo H. Bardales
    • 3
  1. 1.Professor of PathologyComplutense University School of MedicineMadridSpain
  2. 2.Professor of PathologyUniversity Hospital Extremadura, Medical SchoolBadajozSpain
  3. 3.Pathologist, Director Ultrasound-guided Fine Needle Aspiration ServiceOutpatient Pathology Associates / Precision PathologySacramentoUSA

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