Advertisement

Tumors of the Sellar Region

  • Serge Weis
  • Michael Sonnberger
  • Andreas Dunzinger
  • Eva Voglmayr
  • Martin Aichholzer
  • Raimund Kleiser
  • Peter Strasser
Chapter
  • 429 Downloads

Abstract

Tumors affecting the sellar region include pituitary adenoma, pituitary carcinoma as well as craniopharyngioma, other tumors (e.g., gangliocytoma, spindle cell oncocytoma of the adenohypophysis, pituicytomas), and cystic lesions.

Craniopharyngioma (WHO grade I) is a histologically benign, partly cystic epithelial tumor of the sellar region presumably derived from embryonic remnants of the Rathke’s pouch epithelium, with two clinicopathological variants (adamantinomatous and papillary) that have distinct phenotypes and characteristic mutations.

Radiologically, adamantinomatous type presents as a mass with solid and cystic components. Calcifications are frequent.

Histologically, the tumor is made up of squamous epithelium disposed in cords and lobules. Nodules of wet keratin, i.e., remnants of pale nuclei are embedded within an eosinophilic keratinous mass wet keratin.

Craniopharyngioma is thought to be caused by neoplastic transformation of ectodermal-derived epithelial cell remnants of Rathke’s pouch and the craniopharyngeal duct. Mutation of the β-catenin gene is found in adamantinomatous craniopharyngioma. BRAFV600E mutations are solely found in the papillary CP subgroup. Differential gene expression and methylation reveal a distinct upregulation of Wnt- and Shh signaling pathway genes in adamantinomatous CP.

The tumor is surgically excised. Outcome is good. Sixty to 95% of patients have a 10-year recurrence-free survival. Incomplete resection leads to higher recurrence rates because of infiltrative tumor behavior.

Spindle cell oncocytomas, granular cell tumors of the sellar region, and pituicytomas show nuclear expression of TTF1, suggesting that these three tumors may constitute a spectrum of a single nosological entity.

Pituicytoma (WHO grade I) is made up of bipolar, short to elongate spindle-shaped cells with oval to elongate nuclei.

The tumor originates from pituicytes of the neurohypophysis or alternatively from folliculostellate stromal cells of the adenohypophysis.

Surgical removal is the treatment of choice. No malignant transformation.

Granular cell tumor of the sellar region (WHO grade I) consists of densely packed polygonal cells with abundant granular eosinophilic cytoplasm. The cells are arranged in nodules, sheets, and fascicles.

The tumor cells arise from pituicytes (i.e., glial element in the posterior lobe and stalk of the pituitary gland).

Total resection if possible is attempted. Outcome is good as the tumor shows slow progression and lack of invasive growth.

Spindle cell oncocytoma (WHO grade I) is made up of spindle cells with eosinophilic, granular cytoplasm, mild nuclear pleomorphism, and dense chromatin. The tumor cells are arranged in fascicles.

The origin of the tumor is uncertain; it might derive from folliculostellate cells of the anterior hypophysis.

Surgical resection can be followed by radiosurgery. Outcome is good. Local aggressive behavior is possible.

Selected References

  1. Alexandrescu S, Brown RE, Tandon N, Bhattacharjee MB (2012) Neuron precursor features of spindle cell oncocytoma of adenohypophysis. Ann Clin Lab Sci 42(2):123–129Google Scholar
  2. Brat DJ, Wesseling P, Fuller GN, Roncaroli F (2016) Pituicytoma. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (eds) WHO classification of tumours of the central nervous system, Revised 4th edn. IARC, Lyon, pp 332–333Google Scholar
  3. Buslei R, Rushing EJ, Giangaspero F, Paulus W, Burger PC, Santagata S (2016) Craniopharyngioma. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (eds) WHO classification of tumours of the central nervous system, Revised 4th edn. IARC, Lyon, pp 324–328Google Scholar
  4. Covington MF, Chin SS, Osborn AG (2011) Pituicytoma, spindle cell oncocytoma, and granular cell tumor: clarification and meta-analysis of the world literature since 1893. AJNR Am J Neuroradiol 32(11):2067–2072.  https://doi.org/10.3174/ajnr.A2717Google Scholar
  5. El Hussein S, Vincentelli C (2017) Pituicytoma: review of commonalities and distinguishing features among TTF-1 positive tumors of the central nervous system. Ann Diagn Pathol 29:57–61.  https://doi.org/10.1016/j.anndiagpath.2017.05.004Google Scholar
  6. Farooq MU, Bhatt A, Chang HT (2008) Teaching neuroimage: spindle cell oncocytoma of the pituitary gland. Neurology 71(2):e3.  https://doi.org/10.1212/01.wnl.0000316805.30694.4fGoogle Scholar
  7. Fuller GN, Brat DJ, Wesseling P, Roncaroli F (2016) Granular cell tumour of the sellar region. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (eds) WHO classification of tumours of the central nervous system, Revised 4th edn. IARC, Lyon, pp 329–331Google Scholar
  8. Fuller GN, Scheithauer BW, Roncaroli F, Wesseling P (2007) Spindle cell oncocytoma of the adenohypophysis. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (eds) WHO classification of tumours of the central nervous system, 4th edn. International Agency for Research on Cancer, Lyon, pp 245–246Google Scholar
  9. Fuller GN, Wesseling P (2007) Granular cell tumour of the sellar region. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (eds) WHO classification of tumours of the central nervous system, 4th edn. International Agency for Research on Cancer, Lyon, pp 241–242Google Scholar
  10. Furtado SV, Ghosal N, Venkatesh PK, Gupta K, Hegde AS (2010) Diagnostic and clinical implications of pituicytoma. J Clin Neurosci 17(7):938–943.  https://doi.org/10.1016/j.jocn.2009.09.047Google Scholar
  11. Gibbs WN, Monuki ES, Linskey ME, Hasso AN (2006) Pituicytoma: diagnostic features on selective carotid angiography and MR imaging. AJNR Am J Neuroradiol 27(8):1639–1642Google Scholar
  12. Hasiloglu ZI, Ure E, Comunoglu N, Tanriover N, Oz B, Gazioglu N, Mihmanli I (2016) New radiological clues in the diagnosis of spindle cell oncocytoma of the adenohypophysis. Clin Radiol 71(9):937.e935–937.e911.  https://doi.org/10.1016/j.crad.2016.04.022Google Scholar
  13. Holsken A, Sill M, Merkle J, Schweizer L, Buchfelder M, Flitsch J, Fahlbusch R, Metzler M, Kool M, Pfister SM, von Deimling A, Capper D, Jones DT, Buslei R (2016) Adamantinomatous and papillary craniopharyngiomas are characterized by distinct epigenomic as well as mutational and transcriptomic profiles. Acta Neuropathol Commun 4:20.  https://doi.org/10.1186/s40478-016-0287-6Google Scholar
  14. Janzer RC, Burger PC, Giangaspero F, Paulus W (2000) Craniopharyngioma. In: Kleihues P, Cavenee WK (eds) Pathology and genetics of tumours of the nervous system, 3rd edn. IARC Press, Lyon, pp 244–246Google Scholar
  15. Karamchandani J, Syro LV, Uribe H, Horvath E, Kovacs K (2012) Pituicytoma of the neurohypophysis: analysis of cell proliferation biomarkers. Can J Neurol Sci 39(6):835–837Google Scholar
  16. Kleihues P, Burger PC, Scheithauer BW (1993) Histological typing of tumours of the central nervous system, 2nd edn. Springer, BerlinGoogle Scholar
  17. Kosuge Y, Hiramoto J, Morishima H, Tanaka Y, Hashimoto T (2012) Neuroimaging characteristics and growth pattern on magnetic resonance imaging in a 52-year-old man presenting with pituicytoma: a case report. J Med Case Rep 6:306.  https://doi.org/10.1186/1752-1947-6-306Google Scholar
  18. Kowalski RJ, Prayson RA, Mayberg MR (2004) Pituicytoma. Ann Diagn Pathol 8(5):290–294Google Scholar
  19. Kwon MJ, Suh YL (2011) Pituicytoma with unusual histological features. Pathol Int 61(10):598–602.  https://doi.org/10.1111/j.1440-1827.2011.02708.xGoogle Scholar
  20. Lopes MBS, Fuller GN, Roncaroli F, Wesseling P (2016) Spindle cell oncocytoma. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (eds) WHO classification of tumours of the central nervous system, Revised 4th edn. IARC, Lyon, pp 334–336Google Scholar
  21. Machado I, Cruz J, Lavernia J, Llombart-Bosch A (2016) Solitary, multiple, benign, atypical, or malignant: the “granular cell tumor” puzzle. Virchows Arch 468(5):527–538.  https://doi.org/10.1007/s00428-015-1877-6Google Scholar
  22. Manoranjan B, Koziarz A, Kameda-Smith MM, Provias JP (2017) Multiple recurrences require long-term follow-up in patients diagnosed with spindle cell oncocytoma of the Sella turcica. J Clin Neurosci 43:134–146.  https://doi.org/10.1016/j.jocn.2017.05.017Google Scholar
  23. Mende KC, Matschke J, Burkhardt T, Saeger W, Buslei R, Buchfelder M, Fahlbusch R, Westphal M, Flitsch J (2017) Pituicytoma-an outlook on possible targeted therapies. CNS Neurosci Ther 23(7):620–626.  https://doi.org/10.1111/cns.12709Google Scholar
  24. Neidert MC, Leske H, Burkhardt JK, Kollias SS, Capper D, Schrimpf D, Regli L, Rushing EJ (2016) Synchronous pituitary adenoma and pituicytoma. Hum Pathol 47(1):138–143.  https://doi.org/10.1016/j.humpath.2015.08.017Google Scholar
  25. Ogiwara H, Dubner S, Shafizadeh S, Raizer J, Chandler JP (2011) Spindle cell oncocytoma of the pituitary and pituicytoma: two tumors mimicking pituitary adenoma. Surg Neurol Int 2:116.  https://doi.org/10.4103/2152-7806.83932Google Scholar
  26. Ordonez NG (1999) Granular cell tumor: a review and update. Adv Anat Pathol 6(4):186–203Google Scholar
  27. Ordonez NG, Mackay B (1999) Granular cell tumor: a review of the pathology and histogenesis. Ultrastruct Pathol 23(4):207–222Google Scholar
  28. Piccirilli M, Maiola V, Salvati M, D’Elia A, Di Paolo A, Campagna D, Santoro A, Delfini R (2014) Granular cell tumor of the neurohypophysis: a single-institution experience. Tumori 100(4):160e–164e.  https://doi.org/10.1700/1636.17940Google Scholar
  29. Romero-Rojas AE, Melo-Uribe MA, Barajas-Solano PA, Chinchilla-Olaya SI, Escobar LI, Hernandez-Walteros DM (2011) Spindle cell oncocytoma of the adenohypophysis. Brain Tumor Pathol 28(4):359–364.  https://doi.org/10.1007/s10014-011-0051-3Google Scholar
  30. 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(3):314–318.  https://doi.org/10.1111/j.1750-3639.2007.00081.xGoogle Scholar
  31. Rushing EJ, Giangaspero F, Paulus W, Burger PC (2007) Craniopharyngioma. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (eds) WHO classification of tumours of the central nervous system, 4th edn. International Agency for Research on Cancer, Lyon, pp 238–240Google Scholar
  32. Sali A, Epari S (2017) Spindle cell oncocytoma of adenohypophysis: review of literature and report of another recurrent case. Neuropathology 37(6):535–543.  https://doi.org/10.1111/neup.12393Google Scholar
  33. Tampi C, Goel A, Mathkour M, Garces J, Scullen T, Valle-Giler E, Halat S, Arrington T, Ware M (2017) Spindle cell oncocytoma of the pituitary gland. Neuropathology 61(5):554–557.  https://doi.org/10.1111/neup.12393
  34. Teti C, Castelletti L, Allegretti L, Talco M, Zona G, Minuto F, Boschetti M, Ferone D (2015) Pituitary image: pituicytoma. Pituitary 18(5):592–597.  https://doi.org/10.1007/s11102-014-0612-7Google Scholar
  35. Vuong HG, Kondo T, Tran TM, Oishi N, Nakazawa T, Mochizuki K, Inoue T, Kasai K, Tahara I, Jieying W, Katoh R (2016) Spindle cell oncocytoma of adenohypophysis: report of a case and immunohistochemical review of literature. Pathol Res Pract 212(3):222–225.  https://doi.org/10.1016/j.prp.2015.07.014Google Scholar
  36. Wang J, Liu Z, Du J, Cui Y, Fang J, Xu L, Li G (2016) The clinicopathological features of pituicytoma and the differential diagnosis of sellar glioma. Neuropathology 36(5):432–440.  https://doi.org/10.1111/neup.12291Google Scholar
  37. Warzok RW, Vogelsang S, Feiden W, Shuangshoti S (2000) Granular cell tumour of the neurohyophysis. In: Kleihues P, Cavenee WK (eds) Pathology and genetics of tumours of the nervous system, 3rd edn. IARC Press, Lyon, pp 247–248Google Scholar
  38. Wesseling P, Brat DJ, Fuller GN (2007) Pituicytoma. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (eds) WHO classification of tumours of the central nervous system, 4th edn. International Agency for Research on Cancer, Lyon, pp 243–244Google Scholar
  39. Yoshimoto T, Takahashi-Fujigasaki J, Inoshita N, Fukuhara N, Nishioka H, Yamada S (2015) TTF-1-positive oncocytic sellar tumor with follicle formation/ependymal differentiation: non-adenomatous tumor capable of two different interpretations as a pituicytoma or a spindle cell oncocytoma. Brain Tumor Pathol 32(3):221–227.  https://doi.org/10.1007/s10014-015-0219-3Google Scholar
  40. Zhi L, Yang L, Quan H, Bai-ning L (2009) Pituicytoma presenting with atypical histological features. Pathology 41(5):505–509Google Scholar
  41. Zülch KJ (1979) Histological typing of tumours of the central nervous system. World Health Organization, GenevaGoogle Scholar
  42. Zunarelli E, Casaretta GL, Rusev B, Lupi M (2011) Pituicytoma with atypical histological features: are they predictive of unfavourable clinical course? Pathology 43(4):389–394.  https://doi.org/10.1097/PAT.0b013e32834687b3Google Scholar

Copyright information

© Springer-Verlag GmbH Austria, part of Springer Nature 2019

Authors and Affiliations

  • Serge Weis
    • 1
  • Michael Sonnberger
    • 2
  • Andreas Dunzinger
    • 3
  • Eva Voglmayr
    • 2
  • Martin Aichholzer
    • 4
  • Raimund Kleiser
    • 2
  • Peter Strasser
    • 5
  1. 1.Division of Neuropathology, Neuromed CampusKepler University Hospital, Johannes Kepler UniversityLinzAustria
  2. 2.Department of Neuroradiology, Neuromed CampusKepler University Hospital, Johannes Kepler UniversityLinzAustria
  3. 3.Department of Neuro-Nuclear Medicine, Neuromed CampusKepler University Hospital, Johannes Kepler UniversityLinzAustria
  4. 4.Department of Neurosurgery, Neuromed CampusKepler University Hospital, Johannes Kepler UniversityLinzAustria
  5. 5.PMU University Institute for Medical & Chemical Laboratory DiagnosticsSalzburgAustria

Personalised recommendations