Exp Clin Endocrinol Diabetes 2014; 122 - P119
DOI: 10.1055/s-0034-1372136

Adrenalectomy for central ACTH-dependent hypercortisolism: tap and scan

UJ Knappe 1, C Jaspers 2, R Salbeck 3, W Saeger 4, H Willenberg 5, R Santen 6, J Feldkamp 7, K Ehlenz 8
  • 1Johannes Wesling Klinikum, Department of Neurosurgery, Minden, Germany
  • 2Johannes Wesling Klinikum, Department of Endocrinology, Minden, Germany
  • 3Johannes Wesling Klinikum, Department of Radiology, Minden, Germany
  • 4Universitätskrankenhaus Eppendorf, Department of Neuropathology, Hamburg, Germany
  • 5Heinrich Heine University, Department of Endocrinology, Düsseldorf, Germany
  • 6German Clinic for Endocrinology, Frankfurt, Germany
  • 7Klinikum Bielefeld, Department of Endocrinology, Bielefeld, Germany
  • 8Gesundheitszentrum Martinshof, Department of Endocrinology, Gießen, Germany

Bilateral adrenalectomy (blAdx) is a possible treatment of not controllable ACTH-dependent hypercortisolism. The follow-up is not standardized.

Out of 70 patients undergoing surgery for ACTH-sectreting pituitary tumors 2 patients with Cushing's disease (CD) finally underwent blAdx and another 3 presented with Nelson's syndrome (NS).

Patient 1 (male, 62 ys) underwent surgery for CD in 2001 and reoperations for recurrences in 2006 and 2010. Due to persistence of CD, radiosurgery was performed in 2010, combined with administration of pasireotide, blAdx was performed in 2011. One year later ACTH was elevated 3-fold. MRI was not possible (pacemaker). Patient 2 (female, 32 ys) underwent surgery for CD elsewhere in 2010 without remission. After reoperation with remission in 2010 recurrence of hypercortisolism occurred after 14 months, and blAdx was performed in 2012. ACTH was elevated 3-fold after 1.5 years, with good suppression after dexamethasone, sellar MRI was negative. Patient 3 (female, 34 ys) had undergone negative pituitary exploration for CD elsewhere in 1999, and blAdx in 2003. In 2012 transsphenoidal surgery for a left parasellar infiltrative macroadenoma (diameter 21 mm) was performed, and ACTH declined from > 800 pg/ml to < 80 pg/ml. Gamma-knife radiosurgery was indicated but not yet performed, since no tumor remnant was visible in MRI. Case 4 (male, 43 ys) was suspected to suffer from ectopic ACTH-dependent hypercortisolism and underwent blAdx in 2011. Pituitary exploration was performed in 2012. ACTH declined from > 460 ng/ml to < 80 ng/ml. Sellar MRI was negative after 7 months. Patient 5 (female, 36 ys) underwent pituitary surgery for CD elsewhere in 2007 and blAdx in 2009. In 2013 she underwent adenomectomy of a 15 mm macroadenoma. ACTH declined from > 2000 ng/ml to < 450 ng/ml. Further tests are scheduled.

After blAdx detection of the ACTH levels and regular MRIs of the sellar region have to be performed to rule out the development of NS.