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open access

Vol 69, No 2 (2018)
Review paper
Submitted: 2017-07-02
Accepted: 2017-08-03
Published online: 2018-03-29
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Cystic lesions of the sellar-suprasellar region — diagnosis and treatment

Elżbieta Andrysiak-Mamos1, Karol Sagan1, Leszek Sagan2, Elżbieta Sowińska-Przepiera3, Anhelli Syrenicz3
DOI: 10.5603/EP.2018.0023
·
Pubmed: 29952427
·
Endokrynol Pol 2018;69(2):212-228.
Affiliations
  1. Klinika Endokrynologii, Chorób Metabolicznych i Chorób Wewnętrznych, Pomorski Uniwersytet Medyczny, ul. Unii Lubelskiej 1, 71-252 Szczecin, Poland
  2. Klinika Neurochirurgii i Neurochirurgii Dziecięcej, Pomorski Uniwersytet Medyczny, ul. Unii Lubelskiej 1, 71-252 Szczecin, Poland
  3. Klinika Endokrynologii, Chorób Metabolicznych i Chorób Wewnętrznych, Pomorski Uniwersytet Medyczny, ul. Unii Lubelskiej 1, 71-252 Szczecin, Poland

open access

Vol 69, No 2 (2018)
Reviews — Postgraduate Education
Submitted: 2017-07-02
Accepted: 2017-08-03
Published online: 2018-03-29

Abstract

The differentiation of cystic lesions located in the sellar-suprasellar region is a significant problem in clinical practice because of the similarities in their clinical, radiological, and even histopathological picture. Arriving at the right diagnosis is vital for taking appropriate therapeutic decisions.

The most frequent clinical manifestation of lesions located in the sellar-suprasellar region is headache. It often co-exists with symptoms of anterior pituitary gland insufficiency or hyperprolactinaemia caused by compression of the pituitary stalk. Diabetes insipidus, obe­sity, mental disorders, and circadian rhythm disorders may be associated with lesions penetrating the suprasellar space. It is extremely important to rule out the possible coexistence of pituitary microadenoma and Rathke’s cleft cyst, which became possible with the use of 11C-methionine positron emission tomography/computed tomography (C-MET PET/CT). Reports from literature indicate that pituitary microadenoma may coexist with Rathke’s cleft cyst in 10% of patients. Cystic lesions of the sellar-suprasellar region should also be differentiated from a cystic pituitary adenoma or abscess.

The first-choice therapy in symptomatic cystic lesions of the sellar-suprasellar region is neurosurgery, which usually relieves headache and improves vision impairment, while less frequently restores normal pituitary function. In suprasellar lesions, neurosurgery may trig­ger or aggravate pre-existing symptoms of damage to the hypothalamus. Patients undergoing neurosurgery for cystic lesions located in the sellar-suprasellar region should be monitored for a few years due to their high recurrence rate, potential malignant transformation of these lesions, and possible adenoma development through metaplasia. The advent of targeted therapy of the BRAF/MEK pathway is associated with new therapeutic opportunities for patients with craniopharyngiomas.

Abstract

The differentiation of cystic lesions located in the sellar-suprasellar region is a significant problem in clinical practice because of the similarities in their clinical, radiological, and even histopathological picture. Arriving at the right diagnosis is vital for taking appropriate therapeutic decisions.

The most frequent clinical manifestation of lesions located in the sellar-suprasellar region is headache. It often co-exists with symptoms of anterior pituitary gland insufficiency or hyperprolactinaemia caused by compression of the pituitary stalk. Diabetes insipidus, obe­sity, mental disorders, and circadian rhythm disorders may be associated with lesions penetrating the suprasellar space. It is extremely important to rule out the possible coexistence of pituitary microadenoma and Rathke’s cleft cyst, which became possible with the use of 11C-methionine positron emission tomography/computed tomography (C-MET PET/CT). Reports from literature indicate that pituitary microadenoma may coexist with Rathke’s cleft cyst in 10% of patients. Cystic lesions of the sellar-suprasellar region should also be differentiated from a cystic pituitary adenoma or abscess.

The first-choice therapy in symptomatic cystic lesions of the sellar-suprasellar region is neurosurgery, which usually relieves headache and improves vision impairment, while less frequently restores normal pituitary function. In suprasellar lesions, neurosurgery may trig­ger or aggravate pre-existing symptoms of damage to the hypothalamus. Patients undergoing neurosurgery for cystic lesions located in the sellar-suprasellar region should be monitored for a few years due to their high recurrence rate, potential malignant transformation of these lesions, and possible adenoma development through metaplasia. The advent of targeted therapy of the BRAF/MEK pathway is associated with new therapeutic opportunities for patients with craniopharyngiomas.

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Keywords

cystic lesion, sellar-suprasellar region, diagnosis, treatment

About this article
Title

Cystic lesions of the sellar-suprasellar region — diagnosis and treatment

Journal

Endokrynologia Polska

Issue

Vol 69, No 2 (2018)

Article type

Review paper

Pages

212-228

Published online

2018-03-29

Page views

6126

Article views/downloads

47722

DOI

10.5603/EP.2018.0023

Pubmed

29952427

Bibliographic record

Endokrynol Pol 2018;69(2):212-228.

Keywords

cystic lesion
sellar-suprasellar region
diagnosis
treatment

Authors

Elżbieta Andrysiak-Mamos
Karol Sagan
Leszek Sagan
Elżbieta Sowińska-Przepiera
Anhelli Syrenicz

References (65)
  1. Trifanescu R, Ansorge O, Wass JAH, et al. Rathke's cleft cysts. Clin Endocrinol (Oxf). 2012; 76(2): 151–160.
    CrossRefPubMed
  2. Zada G, Lin N, Ojerholm E, et al. Craniopharyngioma and other cystic epithelial lesions of the sellar region: a review of clinical, imaging, and histopathological relationships. Neurosurg Focus. 2010; 28(4): E4.
    CrossRefPubMed
  3. Teramoto A, Hirakawa K, Sanno N, et al. Incidental pituitary lesions in 1,000 unselected autopsy specimens. Radiology. 1994; 193(1): 161–164.
    CrossRefPubMed
  4. Hama S, Arita K, Nishisaka T, et al. Changes in the epithelium of Rathke cleft cyst associated with inflammation. J Neurosurg. 2002; 96(2): 209–216.
    CrossRefPubMed
  5. Nishioka H, Haraoka Jo, Izawa H, et al. Headaches Associated with Rathke's Cleft Cyst. Headache: The Journal of Head and Face Pain. 2006; 46(10): 1580–1586.
    CrossRef
  6. Babu R, Back AG, Komisarow JM, et al. Symptomatic Rathke's cleft cyst with a co-existing pituitary tumor; Brief review of the literature. Asian J Neurosurg. 2013; 8(4): 183–187.
    CrossRefPubMed
  7. Isono M, Kamida T, Kobayashi H, et al. Clinical features of symptomatic Rathke's cleft cyst. Clin Neurol Neurosurg. 2001; 103(2): 96–100.
    PubMed
  8. Trifanescu R, Stavrinides V, Plaha P, et al. Outcome in surgically treated Rathke's cleft cysts: long-term monitoring needed. Eur J Endocrinol. 2011; 165(1): 33–37.
    CrossRefPubMed
  9. Valassi E, Biller BMK, Klibanski A, et al. Clinical features of nonpituitary sellar lesions in a large surgical series. Clin Endocrinol (Oxf). 2010; 73(6): 798–807.
    CrossRefPubMed
  10. Nishioka H, Haraoka Jo, Izawa H, et al. Magnetic resonance imaging, clinical manifestations, and management of Rathke's cleft cyst. Clin Endocrinol (Oxf). 2006; 64(2): 184–188.
    CrossRefPubMed
  11. Brassier G, Morandi X, Tayiar E, et al. Rathke's cleft cysts: surgical-MRI correlation in 16 symptomatic cases. J Neuroradiol. 1999; 26(3): 162–171.
    PubMed
  12. Tate MC, Jahangiri A, Blevins L, et al. Infected Rathke cleft cysts: distinguishing factors and factors predicting recurrence. Neurosurgery. 2010; 67(3): 762–9; discussion 769.
    CrossRefPubMed
  13. Ikeda H, Yoshimoto T. Clinicopathological study of Rathke's cleft cysts. Clin Neuropathol. 2002; 21(2): 82–91.
    PubMed
  14. Matsushima T, Fukui M, Fujii K, et al. Epithelial cells in symptomatic rathke's cleft cysts. Surgical Neurology. 1988; 30(3): 197–203.
    CrossRef
  15. Cox B, Roose H, Vennekens A, et al. Pituitary stem cell regulation: who is pulling the strings? J Endocrinol. 2017; 234(3): R135–R158.
    CrossRefPubMed
  16. Cimpean AM, Ceausu AR, Corlan A, et al. The "game" of glial fibrillary acidic and S100 proteins in pituitary adenomas: two players or several? Endokrynol Pol. 2017; 68(4): 380–389.
    CrossRefPubMed
  17. Guo Sy, Cai Xq, Ma J, et al. Diagnosis of concomitant pituitary adenoma and Rathke's cleft cyst with magnetic resonance imaging. Int J Surg. 2015; 18: 191–195.
    CrossRefPubMed
  18. Nishio S, Mizuno J, Barrow DL, et al. Pituitary tumors composed of adenohypophysial adenoma and Rathke's cleft cyst elements: a clinicopathological study. Neurosurgery. 1987; 21(3): 371–377.
    PubMed
  19. Ikeda H, Ohhashi G. Demonstration of high coincidence of pituitary adenoma in patients with ruptured Rathke's cleft cyst: Results of a prospective study. Clin Neurol Neurosurg. 2015; 139: 144–151.
    CrossRefPubMed
  20. Feng Z, He D, Mao Z, et al. Utility of 11C-Methionine and 18F-FDG PET/CT in Patients With Functioning Pituitary Adenomas. Clin Nucl Med. 2016; 41(3): e130–e134.
    CrossRefPubMed
  21. Miljić D, Manojlović-Gačić E, Skender-Gazibara M, et al. All that glitters on PET is not cancer! 18F-deoxy-glucose avidity versus tumor biology: pituitary incidentaloma in a survivor of two previous unrelated malignancies. Endokrynol Pol. 2017; 68(3): 352–359.
    CrossRefPubMed
  22. Bunin GR, Surawicz TS, Witman PA, et al. The descriptive epidemiology of craniopharyngioma. J Neurosurg. 1998; 89(4): 547–551.
    CrossRefPubMed
  23. Hölsken A, Sill M, Merkle J, et al. Adamantinomatous and papillary craniopharyngiomas are characterized by distinct epigenomic as well as mutational and transcriptomic profiles. Acta Neuropathol Commun. 2016; 4: 20.
    CrossRefPubMed
  24. Brastianos P, Santagata S. Endocrine Tumors: BRAFV600E mutations in papillary craniopharyngioma. Eur J Endocrinol. 2016; 174(4): R139–R144.
    CrossRefPubMed
  25. Larkin SJ, Preda V, Karavitaki N, et al. BRAF V600E mutations are characteristic for papillary craniopharyngioma and may coexist with CTNNB1-mutated adamantinomatous craniopharyngioma. Acta Neuropathol. 2014; 127(6): 927–929.
    CrossRefPubMed
  26. Liu Yi, Wang CH, Li DL, et al. TREM-1 expression in craniopharyngioma and Rathke's cleft cyst: its possible implication for controversial pathology. Oncotarget. 2016; 7(31): 50564–50574.
    CrossRefPubMed
  27. Schweizer L, Capper D, Hölsken A, et al. BRAF V600E analysis for the differentiation of papillary craniopharyngiomas and Rathke's cleft cysts. Neuropathol Appl Neurobiol. 2015; 41(6): 733–742.
    CrossRefPubMed
  28. Nielsen EH, Jørgensen JO, Bjerre P, et al. Acute presentation of craniopharyngioma in children and adults in a Danish national cohort. Pituitary. 2013; 16(4): 528–535.
    CrossRefPubMed
  29. Iughetti L, Bruzzi P. Obesity and craniopharyngioma. Ital J Pediatr. 2011; 37: 38.
    CrossRefPubMed
  30. Müller HL. Craniopharyngioma and hypothalamic injury: latest insights into consequent eating disorders and obesity. Curr Opin Endocrinol Diabetes Obes. 2016; 23(1): 81–89.
    CrossRefPubMed
  31. Müller HL. Risk-adapted, long-term management in childhood-onset craniopharyngioma. Pituitary. 2017; 20(2): 267–281.
    CrossRefPubMed
  32. Daubenbüchel AMM, Müller HL. Neuroendocrine disorders in pediatric craniopharyngioma patients. J Clin Med. 2015; 4(3): 389–413.
    CrossRefPubMed
  33. Honegger J, Barocka A, Sadri B, et al. Neuropsychological results of craniopharyngioma surgery in adults: a prospective study. Surg Neurol. 1998; 50(1): 19–28; discussion 28.
    PubMed
  34. Karavitaki N, Cudlip S, Adams CBT, et al. Craniopharyngiomas. Endocr Rev. 2006; 27(4): 371–397.
    CrossRefPubMed
  35. Chakrabarti I, Amar AP, Couldwell W, et al. Long-term neurological, visual, and endocrine outcomes following transnasal resection of craniopharyngioma. J Neurosurg. 2005; 102(4): 650–657.
    CrossRefPubMed
  36. Shin JL, Asa SL, Woodhouse LJ, et al. Cystic lesions of the pituitary: clinicopathological features distinguishing craniopharyngioma, Rathke's cleft cyst, and arachnoid cyst. J Clin Endocrinol Metab. 1999; 84(11): 3972–3982.
    CrossRefPubMed
  37. Robinson AG. Diabetes insipidus in man. In: Czernichow P. ed. International Symposium, Paris, 1984. Karger, Basel 1985 (vol. 13): 247–265.
  38. Huang CH, Chou KJ, Lee PT, et al. A case of lymphocytic hypophysitis with masked diabetes insipidus unveiled by glucocorticoid replacement. Am J Kidney Dis. 2005; 45(1): 197–200.
    CrossRefPubMed
  39. Non L, Brito D, Anastasopoulou C. Neurosarcoidosis-associated central diabetes insipidus masked by adrenal insufficiency. BMJ Case Rep. 2015; 2015.
    CrossRefPubMed
  40. MacDonald SM, Rapalino O, Sherry NA, et al. Case 32-2016. A 20-Year-Old Man with Gynecomastia. N Engl J Med. 2016; 375(16): 1567–1579.
    CrossRefPubMed
  41. Eisenberg Y, Frohman LA. Adipsic diabetes insipidus. A review. Endocr Pract. 2016; 22(1): 76–83.
    CrossRefPubMed
  42. Adrogué HJ, Madias NE. Hypernatremia. N Engl J Med. 2000; 342(20): 1493–1499.
    CrossRefPubMed
  43. Kristof RA, Rother M, Neuloh G, et al. Incidence, clinical manifestations, and course of water and electrolyte metabolism disturbances following transsphenoidal pituitary adenoma surgery: a prospective observational study. J Neurosurg. 2009; 111(3): 555–562.
    CrossRefPubMed
  44. Smith D, Finucane F, Phillips J, et al. Abnormal regulation of thirst and vasopressin secretion following surgery for craniopharyngioma. Clin Endocrinol (Oxf). 2004; 61(2): 273–279.
    CrossRefPubMed
  45. Crowley RK, Hamnvik OP, O'Sullivan EP, et al. Morbidity and mortality in patients with craniopharyngioma after surgery. Clin Endocrinol (Oxf). 2010; 73(4): 516–521.
    CrossRefPubMed
  46. Weiner HL, Wisoff JH, Rosenberg ME, et al. Craniopharyngiomas: a clinicopathological analysis of factors predictive of recurrence and functional outcome. Neurosurgery. 1994; 35(6): 1001–10; discussion 1010.
    PubMed
  47. Sartoretti-Schefer S, Wichmann W, Aguzzi A, et al. MR differentiation of adamantinous and squamous-papillary craniopharyngiomas. AJNR Am J Neuroradiol. 1997; 18(1): 77–87.
    PubMed
  48. Hofmann BM, Kreutzer J, Saeger W, et al. Nuclear beta-catenin accumulation as reliable marker for the differentiation between cystic craniopharyngiomas and rathke cleft cysts: a clinico-pathologic approach. Am J Surg Pathol. 2006; 30(12): 1595–1603.
    CrossRefPubMed
  49. Lee InHo, Zan E, Bell WR, et al. Craniopharyngiomas: Radiological Differentiation of Two Types. J Korean Neurosurg Soc. 2016; 59(5): 466–470.
    CrossRefPubMed
  50. Brastianos PK, Shankar GM, Gill CM, et al. Dramatic Response of BRAF V600E Mutant Papillary Craniopharyngioma to Targeted Therapy. J Natl Cancer Inst. 2016; 108(2).
    CrossRefPubMed
  51. Dubuisson AS, Stevenaert A, Martin DH, et al. Intrasellar arachnoid cysts. Neurosurgery. 2007; 61(3): 505–513; discussion 513.
    CrossRefPubMed
  52. Meyer FB, Carpenter SM, Laws ER. Intrasellar arachnoid cysts. Surg Neurol. 1987; 28(2): 105–110.
    PubMed
  53. Busch W. Morphology of sella turica and its relationship to the pituary gland. Virchows Archiv. 1951; 320: 437–458.
  54. Iqbal J, Kanaan I, Al Homsi M. Non-neoplastic cystic lesions of the sellar region presentation, diagnosis and management of eight cases and review of the literature. Acta Neurochir (Wien). 1999; 141(4): 389–97; discussion 397.
    PubMed
  55. Syrenicz A. (ed.) Endokrynologia w codziennej paraktyce lekarskiej,. Wyd. 1. Pomorski Uniwersytet Medyczny, Szczecin 2009.
  56. Yoshida M, Miyata M, Ueda H, et al. Subclinical Cushing syndrome associated with an empty sella turcica. Intern Med. 2014; 53(6): 637–638.
    PubMed
  57. Liu W, Zhou H, Neidert MC, et al. Growth hormone secreting pituitary microadenomas and empty sella - An under-recognized association? Clin Neurol Neurosurg. 2014; 126: 18–23.
    CrossRefPubMed
  58. Zieliński G, Witek P, Maksymowicz M. Outcomes in pituitary surgery in Nelson's syndrome--therapeutic pitfalls. Endokrynol Pol. 2015; 66(6): 504–513.
    CrossRefPubMed
  59. Melmed S, Polonsky KS, Larsen PR. Williams Textbook of Endocrinology, 13th Edition. Elsevier, Philadelphia, USA 2016: 305–313.
  60. Kurowska M, Tarach JS, Malicka J, et al. Long-term complete remission of Crooke's corticotropinoma after temozolomide treatment. Endokrynol Pol. 2016; 67(5): 526–533.
    CrossRefPubMed
  61. Messer CK, Fowkes ME, Gabrilove JL, et al. ACTH-producing remnants following apoplexy of an ACTH-secreting pituitary macroadenoma. Pituitary. 2012; 15 Suppl 1: S6–S9.
    CrossRefPubMed
  62. Sibal L, Ball SG, Connolly V, et al. Pituitary apoplexy: a review of clinical presentation, management and outcome in 45 cases. Pituitary. 2004; 7(3): 157–163.
    CrossRefPubMed
  63. Hekimsoy Z, Yünten N, Sivrioglu S. Coexisting acromegaly and primary empty sella syndrome. Neuro Endocrinol Lett. 2004; 25(4): 307–309.
    PubMed
  64. Petrakakis I, Pirayesh A, Krauss JK, et al. The sellar and suprasellar region: A "hideaway" of rare lesions. Clinical aspects, imaging findings, surgical outcome and comparative analysis. Clin Neurol Neurosurg. 2016; 149: 154–165.
    CrossRefPubMed
  65. Yang B, Yang C, Fang J, et al. Clinicoradiologic features and surgical outcomes of sellar xanthogranulomas: A single-center 10-year experience. World Neurosurg. 2017; 99: 439–447.
    CrossRefPubMed

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