Skip to main content
SpringerLink
Log in

The Association of Cushing's Disease and Primary Empty Sella Turcica

  • Published:
Pituitary Aims and scope Submit manuscript

Abstract

The empty sella turcica is defined as the extension of the subarachnoid space toward the intrasellar region with displacement of the pituitary towards the posteroinferior wall. By autopsy studies, the incidence in the general population is around 20%. The association of Cushing's disease (CD) and empty sella has been infrequently reported. In our group, from a total of 68 patients with CD studied by magnetic resonance imaging (MRI), we found the presence of a primary empty sella syndrome (ESS) in 11 (16%). Of these, 9 had partial and 2 total ESS, and in four of them a microadenoma could be identified. Remission, ascertained by subnormal postoperative cortisol levels in blood and/or urine was obtained in 5 of 6 patients operated on by the transphenoidal route. Following surgery, 2 patients presented cerebrospinal fluid (CSF) leakage, 2 diabetes insipidus, and 2 some form of hypopituitarism, figures apparently higher than in non-ESS patients with CD. Ketoconazole was indicated as second line treatment in 2 patients and as primary therapy in 4, resulting in normal urinary free cortisol (UFC) values, with no complications. The relationship of ESS and CD is probably fortuitous given that the frequency of ESS in the general population is similar. Although in empty sella both surgery and radiotherapy seem to have greater risk of complications, surgery remains the first line treatment. Nevertheless, chronic treatment with ketoconazole could be a useful first choice, particularly when no adenoma is seen, or in those who have contraindications for surgery.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

REFERENCES

  1. Abboud CS. Anterior pituitary failure. In: Melmed S, ed. The Pituitary. Blackwell Science Ed., 1995:394–395.

  2. Molitch ME. Evaluation and treatment of the patient with a pituitary incidentaloma. JCEM1995;80:3–6.

    Google Scholar 

  3. Gallardo E, Schachter D, Caceres E, Becker P, Colin E, Martinez C, Henriquez C. The empty sella: Results of treatment in 76 successive cases and high frequency of endocrine and neurosurgical disturbances. Clinical Endocrinology 1992;37:529–533.

    Google Scholar 

  4. Hardjasudarma M, White KE, Nandy I, Burns PL. Sellar emptiness on routine magnetic resonance imaging. South Med J 1994;87:340–343.

    Google Scholar 

  5. Ganguly A, Stanchfield JB, Roberts TS, West CD, Tyler FH. Cushing's syndrome in a patient with an empty sella turcica and a microadenoma of the adenohypophysis. Am J Med 1976;60:306–309.

    Google Scholar 

  6. Gautier D, Halimi D, Fromantin M. Maladie de Cushing avec micro-adénome dans une selle turcique vide. Hormone Res 1980;13:340.

    Google Scholar 

  7. Leutenegger M, Gross A, Hublot C. Maladie de Cushing avec microadenome corticotrope et selle turcique vide. La Nouvelle Presse Medicale 1982;11:454–455.

    Google Scholar 

  8. Smith DJ, Kohler PC, Helminiak R, Carroll J. Intermittent Cushing's syndrome with an empty sella turcica. Arch Intern Med 1982;142(12):2185–2187.

    Google Scholar 

  9. Lorcy Y, Allannic H, Faivre JL, Legerrier AM. Maladie de Cushing par microadenome hypophysaire probable associee a un diverticule arachnoidien intrasellaire. La Presse Médicale 1985;14:284.

    Google Scholar 

  10. Boluda Monzo S, Mesa Manteca J, Obiols Alonso G, Simo Canonge R. Enfermedad de Cushing y silla turca vacia primaria. Medicina Clinica 1989;92:396–397.

    Google Scholar 

  11. Conget JI, Halperin I, Vendrell J, et al. Enfermedad de Cushing en una paciente con silla turca vacia primaria. Med Clin (Barc) 1989;92:705–707.

    Google Scholar 

  12. Webb SM, Urgeles JR. Sindrome de Cushing y silla turca vacia. Medicina Clinica 1989;93:517–518.

    Google Scholar 

  13. Mancini A, Calabro F, Lagonigro G, Saporosi A, Colosimo C Jr, Anile C, Maira G, De Marinis L. Cushing's syndrome: New variants and association with empty sella syndrome. The Journal of Nuclear Medicine and Allied Sciences 1990;34:59–66.

    Google Scholar 

  14. Sastre J, López Guzmán A, Díxez J, Salvador J. Actitud terepeútica de la enfermedad de Cushing asociada a silla turca vacía. Medicina Clínica 1991;97:52.

    Google Scholar 

  15. Spagnolli W, Ramponi C, Daví MV, Francia G. Malattia di Cushing associata a sella vuota: un caso clinico trattato per sette anni con ketoconazolo. Ann Ital Med Int 1996;11:275–278.

    Google Scholar 

  16. Burke CW, Beardwell CG. Cushing's syndrome. An evaluation of the clinical usefulness of urinary free cortisol and other urinary steroid measurements in diagnosis. Q J Med 1973;42:175–205.

    Google Scholar 

  17. Nugent CA, Nichols T, Tyler FH. Diagnosis of Cushing's syndrome. Single dose dexamethasone suppression test. Arch Intern Med 1965;116:172–176.

    Google Scholar 

  18. Contreras LN, Hane S, Tyrrell B. Urinary cortisol in the assessment of pituitary-adrenal function: Utility of 24-hour and spot determination. J Clin Endocrinol Metab 1986;62:965–969.

    Google Scholar 

  19. Bruno OD, Rossi MA, Contreras LN. Nocturnal high-dose dexamethasone suppression test in the aetiological diagnosis of Cushing's syndrome. Acta Endocrinol 1985;109:158–162.

    Google Scholar 

  20. Rossi MA, Chervin R, Bruno OD. Supresión Nocturna con Dexametasona y Test de Metopirona en el Diagnostico Etiológico del Sindrome de Cushing. Medicina (Bs.As.) 1996;56:455–462.

    Google Scholar 

  21. Moncet D, Morando JD, Katz SB, Rossi MA, Bruno OD. Ketoconazole therapy in Cushing's syndrome. In: Abstracts of The Endocrine Society's 82nd Annual Meeting. Toronto: The Endocrine Society Press, 2000:466.

    Google Scholar 

  22. Busch W. Die morphologie der sella turcica und ihre Beziehungen zur Hypophyse. Virchows Arch Pathol Anat 1951;320:437–458.

    Google Scholar 

  23. Neelon FA, Goree JA, Lebovitz HE. The primary empty sella: Clinical and radiographic characteristics and endocrine function. Medicine 1973;52:73–91.

    Google Scholar 

  24. Kaufman B. The empty sella turcica–A manifestation of the intrasellar subarachnoid space. Radiology 1968;90:931–941.

    Google Scholar 

  25. Jordan RM, Kendall JW, Kerber CW. The primary empty sella syndrome. Analysis of the clinical characteristics, radiographic features, pituitary function, and cerebrospinal fluid adenohypophysial hormone concentrations. Am J Med 1977;62:569–579.

    Google Scholar 

  26. Komatsu M, Kondo T, Yamauchi K, Yokokawa N, Ichikawa K, Ishihara M, Aizawa T, Yamada T, Imai Y, Tanaka K, Taniguchi K, Watanabe T, Takahashi K. Antipituitary antibodies in patients with the primary empty sella syndrome. J Clin Endocrinol Metab 1988;67:633–638.

    Google Scholar 

  27. Pallardo Sanchez LF, Albero Gamboa R, Perez Alvarez M, Sanchez Peinado C, Cerdan Vallejo A. Silla turca vacía idiop00E1;tica e hiperprolactinemia. Revista Clínica Española 1978;148:233–238.

    Google Scholar 

  28. Montalbán J, Sumalla J, Fernandez JL, Molins A, Simó R, Codina A. Empty sella syndrome and pituitary apoplexy. The Lancet 1988;1:774.

    Google Scholar 

  29. Browne JD, Kohut RI. Headache and the primary empty sella syndrome. Arch Otolaryngol Head Nech Surg 1986;112:883–885.

    Google Scholar 

  30. Berke JP, Buxton LF, Kokmen E. The empty sella. Neurology 1975;25:1137–1143.

    Google Scholar 

  31. Valenta LJ, Sostrin RD, Eisenberg H, Tamkin JA, Elias AN. Diagnosis of pituitary tumors by hormone assays and computerized tomography. Am J Med 1982;72:861–873.

    Google Scholar 

  32. Mau M, Phillips TM, Ratner R. Presence of anti-pituitary hormone antibodies in patients with empty sella syndrome and pituitary tumours. Clinical Endocrinology 1993;38:495–500.

    Google Scholar 

  33. Domingue JN, DouglasWing S, Wilson CB. Coexisting pituitary adenomas and partially empty sellas. J Neurosurg 1978;48:23–28.

    Google Scholar 

  34. Bjerre P, Lindholm J, Videbaek H. The spontaneous course of pituitary adenomas and occurrence of an empty sella in untreated acromegaly. J Clin Endocrinol Metab 1986;63:287–291.

    Google Scholar 

  35. Buchfelder M, Nistor R, Fahlbusch R, Huk W. The accuracy of CT and MR evaluation of the sella turcica for detection of adrenocorticotropic hormone secreting adenomas in Cushing's disease. AJNR 1993;14:1183–1190.

    Google Scholar 

  36. Ishikawa S, Furuse M, Saito T, Okada K, Kuzuya T. Empty sella in control subjects and patient with hypopituitarism. Endocrinol Jpn 1988;35:665–674.

    Google Scholar 

  37. Terano T, Seya A, Tamura Y, Yoshida S, Hirayama T. Characteristics of the pituitary gland in elderly subjects from magnetic resonance images: Relationship to pituitary hormone secretion. Clin Endocrinol Oxf 1996;45:273–279.

    Google Scholar 

  38. Dedov II, Zenkova TS, Mel'nichenco GA, Fedina ID. The role of magnetic resonance tomography in the diagnosis of an empty sella turcica. Probl Endokrinol Mosk 1993; 39:4–7.

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. División Endocrinología, Hospital de Clínicas, Buenos Aires University, Avenida Córdoba 2351 5° piso, (, 1020, Ciudad de Buenos Aires, Argentina

    Marcos P. Manavela, Cecilia M. Goodall, Silvia B. Katz, Daniel Moncet & Oscar D. Bruno

Authors
  1. Marcos P. Manavela
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Cecilia M. Goodall
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Silvia B. Katz
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Daniel Moncet
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Oscar D. Bruno
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Manavela, M.P., Goodall, C.M., Katz, S.B. et al. The Association of Cushing's Disease and Primary Empty Sella Turcica. Pituitary 4, 145–151 (2001). https://doi.org/10.1023/A:1015310806063

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1023/A:1015310806063

Search

Navigation