Abstract
Pituitary apoplexy in patients with adrenocorticotropic hormone (ACTH) producing tumors is a rare occurrence. We report four patients with Cushing’s disease harboring ACTH-secreting macroadenomas who presented with pituitary apoplexy. We report the endocrinologic and visual outcomes of these patients after emergent transsphenoidal surgery. A retrospective chart review was performed in 4 patients who presented with pituitary apoplexy from hemorrhage into an ACTH-secreting pituitary adenoma. The patient charts were reviewed for clinical presentation, neuroimaging findings, intraoperative surgical findings, pathologic findings, and postoperative endocrinologic and visual outcomes. All patients presented with acute headaches, nausea, vomiting, and visual loss from optic compression. MR imaging demonstrated a hemorrhagic macroadenoma that was confirmed at surgery. All patients underwent emergent transsphenoidal decompression (within 24 h of presentation). One of these underwent an additional craniotomy to resect residual tumor. Postoperatively, all patients showed significant improvement in visual acuity and visual fields with biochemical remission confirmed on laboratory testing. Significant weight loss as well as resolution of diabetes and hypertension was noted in all cases. All four patients remained in biochemical remission at their most recent follow-up visit (mean 40 months, range: 24–72 months). Excellent endocrine and visual outcomes can be achieved after emergent transsphenoidal surgery in patients with Cushing’s disease presenting with pituitary apoplexy. Although the cure rates of non-apoplectic ACTH macroadenomas are generally poor, higher rates of remission can be achieved in cases of pituitary apoplexy. This may be partly due to the effects of tumor infarction.




Similar content being viewed by others
Explore related subjects
Discover the latest articles and news from researchers in related subjects, suggested using machine learning.References
Bills DC, Meyer FB, Laws ER Jr, Davis DH, Ebersold MJ, Scheithauer BW, Ilstrup DM, Abboud CF (1993) A retrospective analysis of pituitary apoplexy. Neurosurgery 33(4):602–608 discussion 608–609
Bonicki W, Kasperlik-Zaluska A, Koszewski W, Zgliczynski W, Wislawski J (1993) Pituitary apoplexy: endocrine, surgical and oncological emergency. Incidence, clinical course and treatment with reference to 799 cases of pituitary adenomas. Acta Neurochir 120(3–4):118–122
Cardoso ER, Peterson EW (1984) Pituitary apoplexy: a review. Neurosurgery 14(3):363–373
da Motta LA, de Mello PA, de Lacerda CM, Neto AP, da Motta LD, Filho MF (1999) Pituitary apoplexy. Clinical course, endocrine evaluations and treatment analysis. J Neurosurg Sci 43(1):25–36
Liu JK, Couldwell WT (2003) Pituitary Apoplexy: Diagnosis and Management. Contemp Neurosurg 25(12)
Muller-Jensen A, Ludecke D (1981) Clinical aspects of spontaneous necrosis of pituitary tumors (pituitary apoplexy). J Neurol 224(4):267–271
Semple PL, Webb MK, de Villiers JC, Laws ER Jr (2005) Pituitary apoplexy. Neurosurgery 56(1):65–72 discussion 72–73
Sibal L, Ball S, Connolly V, James R, Kane P, Kelly W, Kendall-Taylor P, Mathias D, Perros P, Quinton R, Vaidya B (2004) Pituitary apoplexy: a review of clinical presentation, management and outcome in 45 cases. Pituitary 7(3):157–163. doi:10.1007/s11102-005-1050-3
Verrees M, Arafah BM, Selman WR (2004) Pituitary tumor apoplexy: characteristics, treatment, and outcomes. Neurosurg Focus 16(4):E6
Ayuk J, McGregor EJ, Mitchell RD, Gittoes NJ (2004) Acute management of pituitary apoplexy–surgery or conservative management? Clin Endocrinol 61(6):747–752. doi:10.1111/j.1365-2265.2004.02162.x
Liu JK, Fleseriu M, Delashaw JB Jr, Ciric IS, Couldwell WT (2007) Treatment options for Cushing disease after unsuccessful transsphenoidal surgery. Neurosurg Focus 23(3):E8. doi:10.3171/foc.2007.23.3.10
Randeva HS, Schoebel J, Byrne J, Esiri M, Adams CB, Wass JA (1999) Classical pituitary apoplexy: clinical features, management and outcome. Clin Endocrinol 51(2):181–188
Liu JK, Couldwell WT (2006) Pituitary apoplexy in the magnetic resonance imaging era: clinical significance of sphenoid sinus mucosal thickening. J Neurosurg 104(6):892–898. doi:10.3171/jns.2006.104.6.892
Brisman MH, Katz G, Post KD (1996) Symptoms of pituitary apoplexy rapidly reversed with bromocriptine. Case report. J Neurosurg 85(6):1153–1155. doi:10.3171/jns.1996.85.6.1153
Ebersold MJ, Laws ER Jr, Scheithauer BW, Randall RV (1983) Pituitary apoplexy treated by transsphenoidal surgery. A clinicopathological and immunocytochemical study. J Neurosurg 58(3):315–320. doi:10.3171/jns.1983.58.3.0315
Findling JW, Tyrrell JB, Aron DC, Fitzgerald PA, Wilson CB, Forsham PH (1981) Silent pituitary apoplexy: subclinical infarction of an adrenocorticotropin-producing pituitary adenoma. J Clin Endocrinol Metab 52(1):95–97
Maccagnan P, Macedo CL, Kayath MJ, Nogueira RG, Abucham J (1995) Conservative management of pituitary apoplexy: a prospective study. J Clin Endocrinol Metab 80(7):2190–2197
Mohr G, Hardy J (1982) Hemorrhage, necrosis, and apoplexy in pituitary adenomas. Surg Neurol 18(3):181–189
Ostrov SG, Quencer RM, Hoffman JC, Davis PC, Hasso AN, David NJ (1989) Hemorrhage within pituitary adenomas: how often associated with pituitary apoplexy syndrome? AJR Am J Roentgenol 153(1):153–160
Semple PL, Jane JA, Lopes MB, Laws ER (2008) Pituitary apoplexy: correlation between magnetic resonance imaging and histopathological results. J Neurosurg 108(5):909–915. doi:10.3171/JNS/2008/108/5/0909
Jeon BC, Park YS, Oh HS, Kim YS, Chun BK (2007) Pituitary apoplexy complicated by chemical meningitis and cerebral infarction. J Korean Med Sci 22(6):1085–1089
Arita K, Tominaga A, Sugiyama K, Eguchi K, Iida K, Sumida M, Migita K, Kurisu K (2006) Natural course of incidentally found nonfunctioning pituitary adenoma, with special reference to pituitary apoplexy during follow-up examination. J Neurosurg 104(6):884–891. doi:10.3171/jns.2006.104.6.884
Kamoi K, Toyama M, Sudo N (1999) A case of Cushing’s disease revealed six years after postpartum hypopituitarism. J Clin Endocrinol Metab 84(8):2718–2723
Wakai S, Fukushima T, Teramoto A, Sano K (1981) Pituitary apoplexy: its incidence and clinical significance. J Neurosurg 55(2):187–193. doi:10.3171/jns.1981.55.2.0187
Orth DN (1995) Cushing’s syndrome. N Engl J Med 332(12):791–803. doi:10.1056/NEJM199503233321207
Boscaro M, Barzon L, Fallo F, Sonino N (2001) Cushing’s syndrome. Lancet 357(9258):783–791. doi:10.1016/S0140-6736(00)04172-6
Cannavo S, Almoto B, Dall’Asta C, Corsello S, Lovicu RM, De Menis E, Trimarchi F, Ambrosi B (2003) Long-term results of treatment in patients with ACTH-secreting pituitary macroadenomas. Eur J Endocrinol 149(3):195–200
Fomekong E, Maiter D, Grandin C, Raftopoulos C (2009) Outcome of transsphenoidal surgery for Cushing’s disease: a high remission rate in ACTH-secreting macroadenomas. Clin Neurol Neurosurg 111(5):442–449. doi:10.1016/j.clineuro.2008.12.011
Robert F, Hardy J (1991) Cushing’s disease: a correlation of radiological, surgical and pathological findings with therapeutic results. Pathol Res Pract 187(5):617–621
Salassa RM, Kearns TP, Kernohan JW, Sprague RG, MacCarty CS (1959) Pituitary tumors in patients with Cushing’s syndrome. J Clin Endocrinol Metab 19:1523–1539
Woo YS, Isidori AM, Wat WZ, Kaltsas GA, Afshar F, Sabin I, Jenkins PJ, Monson JP, Besser GM, Grossman AB (2005) Clinical and biochemical characteristics of adrenocorticotropin-secreting macroadenomas. J Clin Endocrinol Metab 90(8):4963–4969. doi:10.1210/jc.2005-0070
Katznelson L, Bogan JS, Trob JR, Schoenfeld DA, Hedley-Whyte ET, Hsu DW, Zervas NT, Swearingen B, Sleeper M, Klibanski A (1998) Biochemical assessment of Cushing’s disease in patients with corticotroph macroadenomas. J Clin Endocrinol Metab 83(5):1619–1623
Pereira AM, van Aken MO, van Dulken H, Schutte PJ, Biermasz NR, Smit JW, Roelfsema F, Romijn JA (2003) Long-term predictive value of postsurgical cortisol concentrations for cure and risk of recurrence in Cushing’s disease. J Clin Endocrinol Metab 88(12):5858–5864
Rollin GA, Ferreira NP, Junges M, Gross JL, Czepielewski MA (2004) Dynamics of serum cortisol levels after transsphenoidal surgery in a cohort of patients with Cushing’s disease. J Clin Endocrinol Metab 89(3):1131–1139
Hammer GD, Tyrrell JB, Lamborn KR, Applebury CB, Hannegan ET, Bell S, Rahl R, Lu A, Wilson CB (2004) Transsphenoidal microsurgery for Cushing’s disease: initial outcome and long-term results. J Clin Endocrinol Metab 89(12):6348–6357. doi:10.1210/jc.2003-032180
Nakane T, Kuwayama A, Watanabe M, Takahashi T, Kato T, Ichihara K, Kageyama N (1987) Long term results of transsphenoidal adenomectomy in patients with Cushing’s disease. Neurosurgery 21(2):218–222
Valassi E, Biller BM, Swearingen B, Pecori Giraldi F, Losa M, Mortini P, Hayden D, Cavagnini F, Klibanski A (2010) Delayed remission after transsphenoidal surgery in patients with Cushing’s disease. J Clin Endocrinol Metab 95(2):601–610. doi:10.1210/jc.2009-1672
De Tommasi C, Vance ML, Okonkwo DO, Diallo A, Laws ER Jr (2005) Surgical management of adrenocorticotropic hormone-secreting macroadenomas: outcome and challenges in patients with Cushing’s disease or Nelson’s syndrome. J Neurosurg 103(5):825–830. doi:10.3171/jns.2005.103.5.0825
Mampalam TJ, Tyrrell JB, Wilson CB (1988) Transsphenoidal microsurgery for Cushing disease. A report of 216 cases. Ann Intern Med 109(6):487–493
Ciric I, Zhao JC, Du H, Findling JW, Molitch ME, Weiss RE, Refetoff S, Kerr WD, Meyer J (2011) Transsphenoidal surgery for Cushing’s disease: experience with 136 patients. Neurosurgery. doi:10.1227/NEU.0b013e31822dda2c
Zhang S, Ye X, Shan Q, Zhang W, Ye L, Cui Y (1999) Effects of acupuncture on the levels of endothelin, TXB2, and 6-keto-PGF1 alpha in apoplexy patients. J Tradit Chin Med 19(1):39–43
Alarifi A, Alzahrani AS, Salam SA, Ahmed M, Kanaan I (2005) Repeated remissions of Cushing’s disease due to recurrent infarctions of an ACTH-producing pituitary macroadenoma. Pituitary 8(2):81–87. doi:10.1007/s11102-005-2961-8
Kamiya Y, Jin-No Y, Tomita K, Suzuki T, Ban K, Sugiyama N, Mase M, Sakuma N, Kimura G (2000) Recurrence of Cushing’s disease after long-term remission due to pituitary apoplexy. Endocr J 47(6):793–797
Le Nestour E, Abecassis JP, Bertagna X, Bonnin A, Luton JP (1994) Silent necrosis of a pituitary corticotroph adenoma revealed by timely magnetic resonance imaging: a cause of spontaneous remission of Cushing’s disease. Eur J Endocrinol 130(5):469–471
Rotman-Pikielny P, Patronas N, Papanicolaou DA (2003) Pituitary apoplexy induced by corticotrophin-releasing hormone in a patient with Cushing’s disease. Clin Endocrinol 58(5):545–549
Sasaki M, Funayama H, Asano T, Kasono K, Namai K, Tamemoto H, Ueno S, Ota M, Kawakami M, Shinoda S, Ishikawa SE (2003) Full-blown Cushing’s disease after an episode of pituitary apoplexy. Endocr J 50(5):501–506
Mercado-Asis LB, Oldfield EH, Cutler GB Jr (1995) Pituitary tumor hemorrhage in Cushing disease. Ann Intern Med 122(3):189–190
Fraser LA, Lee D, Cooper P, Van Uum S (2009) Remission of acromegaly after pituitary apoplexy: case report and review of literature. Endocr Pract 15(7):725–731. doi:10.4158/EP09126.CRR
Thomas N, Simon R, Chacko G, Chacko AG, Chandy MJ, Seshadri MS (1999) Regression of acromegaly following pituitary apoplexy. Neurol India 47(2):161–162
Arafah BM, Harrington JF, Madhoun ZT, Selman WR (1990) Improvement of pituitary function after surgical decompression for pituitary tumor apoplexy. J Clin Endocrinol Metab 71(2):323–328
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Choudhry, O.J., Choudhry, A.J., Nunez, E.A. et al. Pituitary tumor apoplexy in patients with Cushing’s disease: endocrinologic and visual outcomes after transsphenoidal surgery. Pituitary 15, 428–435 (2012). https://doi.org/10.1007/s11102-011-0342-z
Published:
Issue Date:
DOI: https://doi.org/10.1007/s11102-011-0342-z