Pineal region tumors: A diverse pathology in the seat of the soul

  • Rajiv Jha National Academy Of Medical Sciences
  • Jagannath Tiwari

Abstract

Introduction: Pineal region tumors include a variety of neoplasms of different histological origin arising from the pineal gland itself and from structures of the parapineal area. It is a rare tumor which accounts for 0.4-1% of all intracranial tumors. The objective of this study was to describe the epidemiology, clinical presentation and histopathology of pineal region tumors at National Neurosurgical Referral Center, Nepal.
 
Methods: This was a descriptive study of all cases of pineal region tumors which were operated between January 2013 and December 2019 at National Neurosurgical Referral Center, National Academy of Medical Sciences, Bir Hospital.
 
Results: A total of 14 cases were operated during the study period. The mean age was 12 years (4-50 years) with male preponderance (64%). Most of these patients had clinical features of raised intracranial pressure due to obstructive hydrocephalus followed by oculomotor sign in the form of Perinaud syndrome and endocrine dysfunction. Most common histopathology was Pineoblastoma in our study (4 out of 14).
Conclusion: Pineal region tumors are most commonly seen in adolescent age group. Most common symptoms were features of raised intra cranial pressure due to hydrocephalus followed by Perinaud syndrome. Pineal region tumors consist of diverse histopathological variants.

References

1. Jennings MT, Gelman R, Hochberg F. Intracranial germ-cell tumors: natural history and pathogenesis. J Neurosurg.1985;63(2):155?67.

2. Kleihues P, Louis DN, Scheithauer BW, et al. The WHO classification of tumors of the nervous system. J Neuropathol Exp Neurol.2002 Mar;61(3):215?29.

3. Schild SE, Scheithauer BW, Schomberg PJ, Hook CC, Kelly PJ, Frick L et al. Pineal parenchymal tumors. Clinical, pathologic, and therapeutic aspects. Cancer.1993 Aug 1;72(3):870?80.

4. Zulch KJ,Brain tumors:their histology and their pathology,ed 2.Ney York:Springer-Verlag,1965,326

5. 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, morbidity, and diagnostic rates in 370 cases. Neurosurgery. 1996 Nov;39(5):907?14.

6. Kirkpatrick P. Neurosurgical management of aneurysmal subarachnoid haemorrhage. J Neurol Neurosurg Psychiatry. 2000 Apr;68(4):538E.

7. Villano JL, Propp JM, Porter KR, Stewart AK, Valyi-Nagy T, Li X et al. Malignant pineal germ-cell tumors: an analysis of cases from three tumor registries. Neuro Oncol. 2008 Apr;10(2):121-30.

8. Krause F: Operative Freilegung der Vierhüge, nebst Beobachtungen bein Hirndruck un Dekompression. Zentralbl Chir. 1926;53:2812-9.

9. Stein BM. The infratentorial supracerebellar approach to pineal lesions. J Neurosurg. 1971 Aug;35(2):197?202.

10. Jamieson KG: Excision of pineal tumors. J Neurosurg. 1971 Nov;35(5):550-3.

11.Mufti ST, Jamal A. Primary intracranial germ cell tumors. Asian J Neurosurg. 2012 Oct;7(4):197-202.

12. Smith AB, Rushing EJ, Smirniotopoulos JG. From the archives of the AFIP: lesions of the pineal region: radiologic-pathologic correlation. Radiographics. 2010 Nov;30(7):2001?20.

13. Poppen JL, Marino R Jr. Pinealomas and tumors of the posterior portion of the third ventricle. J Neurosurg. 1968 Apr;28(4):357?64.

14. Carr C, O'Neill BE, Hochhalter CB, Strong MJ, Ware ML. Biomarkers of Pineal Region Tumors: A Review. Ochsner J. Spring 2019;19(1):26-31.

15. Tien RD, Barkovich AJ, Edwards MS. MR Imaging of pineal tumors. AJR Am J Roentgenol. 1990 Jul;155(1):143-51.


16. Allen JC, DaRosso RC, Donahue B, Nirenberg A. A phase II trial of preirradiation carboplatin in newly diagnosed germinoma of the central nervous system. Cancer. 1994 Aug 1;74(3):940?4.

17. Kretschmar C, Kleinberg L, Greenberg M, Burger P, Holmes E, Wharam M. Pre-radiation chemotherapy with response-based radiation therapy in children with central nervous system germ cell tumors: a report from the Children's Oncology Group. Pediatr Blood Cancer. 2007 Mar;48(3):285?91.

18. Choi JU, Kim DS, Chung SS, Kim TS. Treatment of germ cell tumors in the pineal region. Childs Nerv Syst. Jan-Feb 1998;14(1-2):41?8.

19. Gururangan S, McLaughlin C, Quinn J, Rich J, Reardon D, Halperin EC et al. High-dose chemotherapy with autologous stem-cell rescue in children and adults with newly diagnosed pineoblastomas. J Clin Oncol. 2003 Jun 1;21(11):2187?91.

20. Gilheeney SW, Saad A, Chi S, Turner C, Ullrich NJ, Goumnerova L et al. Outcome of pediatric pineoblastoma after surgery, radiation and chemotherapy. J Neurooncol. 2008 Aug;89(1):89?95.

21. Selçuki M, Attar A, Yüceer N, Tuna H, Cakiro?lu E. Mature teratoma of the lateral ventricle: report of two cases. Acta Neurochir (Wien). 1998;140(2):171?4.

22. Stein BM, Bruce JN. Surgical management of pineal region tumors (honored guest lecture). Clin Neurosurg. 1992;39:509?32.
Published
2020-12-06
How to Cite
JHA, Rajiv; TIWARI, Jagannath. Pineal region tumors: A diverse pathology in the seat of the soul. J Soc Surg Nep, [S.l.], v. 23, n. 1, p. 37-43, dec. 2020. ISSN 2392-4772. Available at: <http://jssn.org.np/index.php?journal=jssn&page=article&op=view&path%5B%5D=405>. Date accessed: 25 june 2021.