Abstract
Purpose
To show a rare case of Cushing’s disease and possible cause of failed transsphenoidal surgery.
Method
We report on a 50-year-old woman suffering from ACTH-dependent Cushing’s syndrome. Endocrinological work-up including low-dose/high-dose dexamethasone test (Liddle-test) and CRH test were clearly compatible with pituitary origin. Although an MRI showed no pituitary tumor, CRH-stimulated petrosal sinus sampling revealed a significant central-peripheral gradient in ACTH concentrations, rendering Cushing’s disease very likely. The patient underwent transsphenoidal surgery with negative exploration of the pituitary gland. After intraoperative re-evaluation of the preoperative MRI, a “polyp” at the bottom of the sphenoid sinus was identified. The intraoperative microscopic aspect as well as instantaneous sections and cytology of a biopsy confirmed an adenoma, which was then removed. Histological analysis demonstrated an ACTH-producing pituitary adenoma adjacent to respiratory mucous membrane consisting of ciliated epithelium with submucous connective tissue. Postoperatively, ACTH concentrations were decreased and intermittent hydrocortisone substitution treatment was initiated. At the 3-month follow up, Cushing’s stigmata were found to be alleviated and the hydrocortisone dosage could be reduced.
Conclusion
Ectopic pituitary adenoma tissue causing Cushing’s disease is extremely rare but a potential cause for surgical failure or re-evaluation.
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References
Lüdecke DK, Flitsch J, Knappe UJ, Saeger W (2001) Cushing’s disease: a surgical view. J Neurooncol 54:151–161
Flitsch J, Lüdecke DK, Knappe UJ, Grzyska U (2002) Cavernous sinus sampling in selected cases of Cushing’s. Exp Clin Endocrinol Diabetes 110:329–335
Kim LJ, Klopfenstein JD, Cheng M, Nagul M, Coons S, Fredenberg C, Brachman DG, White WL (2003) Ectopic intracavernous sinus adrenocorticotropic hormone-secreting microadenoma: could this be a common cause of failed transsphenoidal surgery in Cushing disease? Case report. J Neurosurg 98:1312–1317
Koizumi M, Usui T, Yamada S, Fujisawa I, Tsuru T, Nanba K, Hagiwara H, Kimura T, Tamanaha T, Tagami T, Naruse M, Shimatsu A (2011) Successful treatment of Cushing’s disease caused by ectopic intracavernous microadenoma. Pituitary 14:295–298
Hodish I, Giordano TJ, Starkman MN, Schteingart DE (2009) Location of ectopic adrenocortical hormone-secreting tumors causing Cushing’s syndrome in the paranasal sinuses. Head Neck 31:699–706
Willhauck MJ, Pöpperl G, Rachinger W, Giese A, Auernhammer CJ, Spitzweg C (2012) An unusual case of ectopic ACTH syndrome. Exp Clin Endocrinol Diabetes 120:63–67
Gurazada K, Ihuoma A, Galloway M, Dorward N, Wilhelm T, Khoo B, Bouloux PM (2013) Nasally located ectopic ACTH-secreting pituitary adenoma (EAPA) causing Nelson’s syndrome: diagnostic challenges. Pituitary. doi:10.1007/s11102-013-0520-2
Horiuchi T, Tanaka Y, Kobayashi S, Unoki T, Yokoh A (1997) Rapidly growing ectopic pituitary adenoma within the sphenoid sinus. Neurol Med Chir (Tokyo) 37:399–402
Tsuboi M, Adachi H, Miyoshi Y, Aoi M (1999) A case of ectopic pituitary adenoma localized in the sphenoid sinus. No Shinkei Geka 27:1007–1011
Suzuki J, Otsuka F, Ogura T, Kishida M, Takeda M, Tamiya T, Nishioka T, Tanaka Y, Hashimoto K, Makino H (2004) An aberrant ACTH-producing ectopic pituitary adenoma in the sphenoid sinus. Endocr J 51:97–103
Wilson CB, Mindermann T, Tyrell JB (1995) Extrasellar, intracavernous sinus adrenocorticotropin-releasing adenoma causing Cushing’s disease. J Clin Endocrinol Metab 80:1774–1777
Slonim SM, Haykal HA, Cushing GW, Freidberg SR, Lee AK (1993) MRI appearances of an ectopic pituitary adenoma: case report and review of the literature. Neuroradiology 35:546–548
Pluta MR, Nieman L, Doppman JL, Watson JC, Tresser N, Katz DA, Oldfield EH (1999) Extrapituitary parasellar microadenoma in Cushing’s disease. J Clin Endocrinol Metab 84:2912–2923
Hou L, Harnshbarger T, Herrick M, Tse V (2002) Suprasellar adrenocortocotropic hormone-secreting ectopic pituitary adenoma: case report and literature review. Neurosurgery 50:618–625
Sanno N, Tahara S, Yoshida Y, Onose H, Wakabayashi I, Teramoto A (1999) Ectopic corticotroph adenoma in the cavernous sinus: case report. Neurosurgery 45:914–918
Burch W, Kramer R, Kenan P, Hammond CB (1985) Cushing’s disease caused by an ectopic adenoma within the sphenoid. N Engl J Med 312:587–588
Lloyd RV, Chandler WF, Kovacs K, Ryan N (1986) Ectopic pituitary adenomas with normal anterior pituitary glands. Am J Surg Pathol 10:546–552
Schteingart DE, Chandler WF, Lloyd RV, Ibarra-Perez G (1987) Cushing’s syndrome caused by an ectopic pituitary adenoma. Neurosurgery 21:223–227
Graham KE, Samuels MH, Nesbit GM (1999) Cavernous sinus sampling is highly accurate in distinguishing Cushing disease from the ectopic adrenocorticotropin syndrome and in predicting intrapituitary tumor location. J Clin Endocrinol Metab 84:1602–1610
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Flitsch, J., Schmid, S.M., Bernreuther, C. et al. A pitfall in diagnosing Cushing’s disease: ectopic ACTH-producing pituitary adenoma in the sphenoid sinus. Pituitary 18, 279–282 (2015). https://doi.org/10.1007/s11102-014-0591-8
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DOI: https://doi.org/10.1007/s11102-014-0591-8