Advertisement

Pineal Region Tumors

  • Nicholas Trakul
  • Jason Ye
Chapter

Abstract

This chapter describes the four recognized histologic types of pineal parenchymal tumors (PPTs). Clinical presentation and common signs and symptoms caused by tumors of the pineal region are described. Clinical workup, including relevant imaging studies and laboratory testing, is outlined. The general treatment approach is considered. Roles of surgery, chemotherapy, and radiation in the definitive and adjuvant setting are discussed for each histologic type. Radiation therapy techniques are described in detail. Included are delineation of treatment volumes, commonly used treatment doses, and treatment delivery systems, including stereotactic radiosurgery and intensity-modulated radiotherapy (IMRT). Possible acute and chronic radiation toxicities and suggestions for posttreatment surveillance conclude the chapter.

Keywords

Pineal parenchymal tumor Pineocytoma Pineal parenchymal tumors of intermediate differentiation Pineoblastoma Papillary tumors of the pineal region Stereotactic radiosurgery 

References

  1. 1.
    Brastianos HC, Brastianos PK, Blakeley J. Pineal region tumors. In: Norden AD, Reardon DA, Wen PCY, editors. Primary central nervous system tumors. Totowa, NJ: Humana Press; 2011. p. 435–55.CrossRefGoogle Scholar
  2. 2.
    Chin LS, Regine WF. Principles and practice of stereotactic radiosurgery. New York: Springer; 2015.CrossRefGoogle Scholar
  3. 3.
    Louis DN, Perry A, Reifenberger G, et al. The 2016 World Health Organization classification of tumors of the central nervous system: a summary. Acta Neuropathol. 2016;131:803–20.CrossRefGoogle Scholar
  4. 4.
    Al-Hussaini M, Sultan I, Abuirmileh N, et al. Pineal gland tumors: experience from the SEER database. J Neurooncol. 2009;94:351–8.CrossRefPubMedPubMedCentralGoogle Scholar
  5. 5.
    Ostrom QT, Gittleman H, Fulop J, et al. CBTRUS statistical report: primary brain and central nervous system tumors diagnosed in the United States in 2008–2012. Neuro Oncol. 2015;17(Suppl 4):iv1–iv62.CrossRefPubMedPubMedCentralGoogle Scholar
  6. 6.
    Konovalov AN, Pitskhelauri DI. Principles of treatment of the pineal region tumors. Surg Neurol. 2003;59:250–68.CrossRefPubMedGoogle Scholar
  7. 7.
    Norden AD, Reardon DA, Wen PY. Primary central nervous system tumors: pathogenesis and therapy. New York: Springer; 2011.CrossRefGoogle Scholar
  8. 8.
    Luther N, Stetler WR, Dunkel IJ, et al. Subarachnoid dissemination of intraventricular tumors following simultaneous endoscopic biopsy and third ventriculostomy. J Neurosurg Pediatr. 2010;5:61–7.CrossRefPubMedGoogle Scholar
  9. 9.
    Chang CG, Kageyama N, Kobayashi T, et al. Pineal tumors: clinical diagnosis, with special emphasis on the significance of pineal calcification. Neurosurgery. 1981;8:656–68.CrossRefPubMedGoogle Scholar
  10. 10.
    Leston J, Mottolese C, Champier J, et al. Contribution of the daily melatonin profile to diagnosis of tumors of the pineal region. J Neurooncol. 2009;93:387–94.CrossRefPubMedGoogle Scholar
  11. 11.
    Chiechi MV, Smirniotopoulos JG, Mena H. Pineal parenchymal tumors: CT and MR features. J Comput Assist Tomogr. 1995;19:509–17.CrossRefPubMedGoogle Scholar
  12. 12.
    Awa R, Campos F, Arita K, et al. Neuroimaging diagnosis of pineal region tumors-quest for pathognomonic finding of germinoma. Neuroradiology. 2014;56:525–34.CrossRefPubMedGoogle Scholar
  13. 13.
    Ganti SR, Hilal SK, Stein BM, et al. CT of pineal region tumors. AJR Am J Roentgenol. 1986;146:451–8.CrossRefPubMedGoogle Scholar
  14. 14.
    Vaquero J, Ramiro J, Martínez R, et al. Clinicopathological experience with pineocytomas: report of five surgically treated cases. Neurosurgery. 1990;27:612–8. discussion 618–9CrossRefPubMedGoogle Scholar
  15. 15.
    Korogi Y, Takahashi M, Ushio Y. MRI of pineal region tumors. J Neurooncol. 2001;54:251–61.CrossRefPubMedGoogle Scholar
  16. 16.
    Nakamura M, Saeki N, Iwadate Y, et al. Neuroradiological characteristics of pineocytoma and pineoblastoma. Neuroradiology. 2000;42:509–14.CrossRefPubMedGoogle Scholar
  17. 17.
    Reddy AT, Wellons JC, Allen JC, et al. Refining the staging evaluation of pineal region germinoma using neuroendoscopy and the presence of preoperative diabetes insipidus. Neuro Oncol. 2004;6:127–33.CrossRefPubMedPubMedCentralGoogle Scholar
  18. 18.
    Regis J, Bouillot P, Rouby-Volot F, et al. Pineal region tumors and the role of stereotactic biopsy: review of the mortality, morbidity, and diagnostic rates in 370 cases. Neurosurgery. 1996;39:907–12. discussion 912–4PubMedGoogle Scholar
  19. 19.
    Borit A, Blackwood W, Mair WG. The separation of pineocytoma from pineoblastoma. Cancer. 1980;45:1408–18.CrossRefPubMedGoogle Scholar
  20. 20.
    Jouvet A, Saint-Pierre G, Fauchon F, et al. Pineal parenchymal tumors: a correlation of histological features with prognosis in 66 cases. Brain Pathol. 2000;10:49–60.CrossRefPubMedGoogle Scholar
  21. 21.
    Cohan JN, Moliterno JA, Mok CL, et al. Pineal parenchymal tumor of intermediate differentiation with papillary features: a continuum of primary pineal tumors? J Neurooncol. 2011;101:301–6.CrossRefPubMedGoogle Scholar
  22. 22.
    Louis DN, Ohgaki H, Wiestler OD, et al. The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol. 2007;114:97–109.CrossRefPubMedPubMedCentralGoogle Scholar
  23. 23.
    Deshmukh VR, Smith KA, Rekate HL, et al. Diagnosis and management of pineocytomas. Neurosurgery. 2004;55:349–55. discussion 355–7CrossRefPubMedGoogle Scholar
  24. 24.
    Fauchon F, Jouvet A, Paquis P, et al. Parenchymal pineal tumors: a clinicopathological study of 76 cases. Int J Radiat Oncol Biol Phys. 2000;46:959–68.CrossRefPubMedGoogle Scholar
  25. 25.
    Fèvre-Montange M, Vasiljevic A, Frappaz D, et al. Utility of Ki67 immunostaining in the grading of pineal parenchymal tumours: a multicentre study. Neuropathol Appl Neurobiol. 2012;38:87–94.CrossRefPubMedGoogle Scholar
  26. 26.
    Herrick MK, Rubinstein LJ. The cytological differentiating potential of pineal parenchymal neoplasms (true pinealomas). A clinicopathological study of 28 tumours. Brain. 1979;102:289–320.CrossRefPubMedGoogle Scholar
  27. 27.
    Jacobs JJ, Rosenberg AE. Extracranial skeletal metastasis from a pinealoblastoma. A case report and review of the literature. Clin Orthop Relat Res. 1989;(247):256–60.Google Scholar
  28. 28.
    Schild SE, Scheithauer BW, Schomberg PJ, et al. Pineal parenchymal tumors. Clinical, pathologic, and therapeutic aspects. Cancer. 1993;72:870–80.CrossRefPubMedGoogle Scholar
  29. 29.
    Chang SM, Lillis-Hearne PK, Larson DA, et al. Pineoblastoma in adults. Neurosurgery. 1995;37:383–90. discussion 390–1CrossRefPubMedGoogle Scholar
  30. 30.
    Mena H, Ribas JL, Enzinger FM, et al. Primary angiosarcoma of the central nervous system. Study of eight cases and review of the literature. J Neurosurg. 1991;75:73–6.CrossRefPubMedGoogle Scholar
  31. 31.
    Farnia B, Allen PK, Brown PD, et al. Clinical outcomes and patterns of failure in pineoblastoma: a 30-year, single-institution retrospective review. World Neurosurg. 2014;82:1232–41.CrossRefPubMedGoogle Scholar
  32. 32.
    Jakacki RI, Burger PC, Kocak M, et al. Outcome and prognostic factors for children with supratentorial primitive neuroectodermal tumors treated with carboplatin during radiotherapy: a report from the Children’s Oncology Group. Pediatr Blood Cancer. 2015;62:776–83.CrossRefPubMedPubMedCentralGoogle Scholar
  33. 33.
    Lee JYK, Wakabayashi T, Yoshida J. Management and survival of pineoblastoma: an analysis of 34 adults from the brain tumor registry of Japan. Neurol Med Chir (Tokyo). 2005;45:132–41. discussion 141–2CrossRefGoogle Scholar
  34. 34.
    Fèvre-Montange M, Hasselblatt M, Figarella-Branger D, et al. Prognosis and histopathologic features in papillary tumors of the pineal region: a retrospective multicenter study of 31 cases. J Neuropathol Exp Neurol. 2006;65:1004–11.CrossRefPubMedGoogle Scholar
  35. 35.
    Fauchon F, Hasselblatt M, Jouvet A, et al. Role of surgery, radiotherapy and chemotherapy in papillary tumors of the pineal region: a multicenter study. J Neurooncol. 2013;112:223–31.CrossRefPubMedGoogle Scholar
  36. 36.
    Heim S, Beschorner R, Mittelbronn M, et al. Increased mitotic and proliferative activity are associated with worse prognosis in papillary tumors of the pineal region. Am J Surg Pathol. 2014;38:106–10.CrossRefPubMedGoogle Scholar
  37. 37.
    Hernesniemi J, Romani R, Albayrak BS, et al. Microsurgical management of pineal region lesions: personal experience with 119 patients. Surg Neurol. 2008;70:576–83.CrossRefPubMedGoogle Scholar
  38. 38.
    Tsumanuma I, Tanaka R, Fujii Y. Occipital transtentorial approach and combined treatments for pineal parenchymal tumors. Prog Neurol Surg. 2009;23:26–43.PubMedGoogle Scholar
  39. 39.
    Lutterbach J, Fauchon F, Schild SE, et al. Malignant pineal parenchymal tumors in adult patients: patterns of care and prognostic factors. Neurosurgery. 2002;51:44–55. discussion 55–6CrossRefPubMedGoogle Scholar
  40. 40.
    Gener MA, Conger AR, Van Gompel J, et al. Clinical, pathological, and surgical outcomes for adult pineoblastomas. World Neurosurg. 2015;84:1816–24.CrossRefPubMedGoogle Scholar
  41. 41.
    Clark AJ, Ivan ME, Sughrue ME, et al. Tumor control after surgery and radiotherapy for pineocytoma. J Neurosurg. 2010;113:319–24.CrossRefPubMedGoogle Scholar
  42. 42.
    Bruce JN, Ogden AT. Surgical strategies for treating patients with pineal region tumors. J Neurooncol. 2004;69:221–36.CrossRefPubMedGoogle Scholar
  43. 43.
    Mallick S, Benson R, Rath GK. Patterns of care and survival outcomes in patients with pineal parenchymal tumor of intermediate differentiation: an individual patient data analysis. Radiother Oncol J Eur Soc Ther Radiol Oncol. 2016;121:204–8.CrossRefGoogle Scholar
  44. 44.
    Das P, Mckinstry S, Devadass A, et al. Are we over treating Pineal Parenchymal tumour with intermediate differentiation? Assessing the role of localised radiation therapy and literature review. Springerplus. 2016;5:26.CrossRefPubMedPubMedCentralGoogle Scholar
  45. 45.
    Kano H, Niranjan A, Kondziolka D, et al. Role of stereotactic radiosurgery in the management of pineal parenchymal tumors. Prog Neurol Surg. 2009;23:44–58.PubMedGoogle Scholar
  46. 46.
    Li W, Zhang B, Kang W, et al. Gamma knife radiosurgery (GKRS) for pineal region tumors: a study of 147 cases. World J Surg Oncol. 2015;13:304.CrossRefPubMedPubMedCentralGoogle Scholar
  47. 47.
    Park JH, Kim JH, Kwon DH, et al. Upfront stereotactic radiosurgery for pineal parenchymal tumors in adults. J Korean Neurosurg Soc. 2015;58:334–40.CrossRefPubMedPubMedCentralGoogle Scholar
  48. 48.
    Wilson DA, Awad A-W, Brachman D, et al. Long-term radiosurgical control of subtotally resected adult pineocytomas. J Neurosurg. 2012;117:212–7.CrossRefPubMedGoogle Scholar
  49. 49.
    Yianni J, Rowe J, Khandanpour N, et al. Stereotactic radiosurgery for pineal tumours. Br J Neurosurg. 2012;26:361–6.CrossRefPubMedGoogle Scholar
  50. 50.
    Stoiber EM, Schaible B, Herfarth K, et al. Long term outcome of adolescent and adult patients with pineal parenchymal tumors treated with fractionated radiotherapy between 1982 and 2003—a single institution’s experience. Radiat Oncol. 2010;5:122.CrossRefPubMedPubMedCentralGoogle Scholar
  51. 51.
    Sonabend AM, Bruce JN. Management paradigms along a histologic spectrum of pineal cell tumors. World Neurosurg. 2014;81:685–7.CrossRefPubMedGoogle Scholar
  52. 52.
    Mynarek M, Pizer B, Dufour C, et al. Evaluation of age-dependent treatment strategies for children and young adults with pineoblastoma: analysis of pooled European Society for Paediatric Oncology (SIOP-E) and US Head Start data. Neuro Oncol. 2017;19(4):576–85.PubMedGoogle Scholar
  53. 53.
    Selvanathan SK, Hammouche S, Smethurst W, et al. Outcome and prognostic features in adult pineoblastomas: analysis of cases from the SEER database. Acta Neurochir (Wien). 2012;154:863–9.CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Radiation OncologyStanford UniversityStanfordUSA
  2. 2.Department of Radiation OncologyKeck School of Medicine of USCLos AngelesUSA

Personalised recommendations