Exp Clin Endocrinol Diabetes 2013; 121 - OP1_05
DOI: 10.1055/s-0033-1336613

Transsphenoidal microsurgery for ACTH-dependent hypercortisolism: a consecutive series

UJ Knappe 1, C Jaspers 2, R Salbeck 3, W Saeger 4, M Engelbach 5, B Jacobs 6, WA Mann 7, J Feldkamp 8
  • 1Johannes Wesling Klinikum, Dep. of Neurosurgery, Minden, Germany
  • 2Johannes Wesling Klinikum, Dep. of Endocrinology, Minden, Germany
  • 3Johannes Wesling Klinikum, Institute of Radiology, Minden, Germany
  • 4Marienkrankenhaus Hamburg, Institute for Pathology, Hamburg, Germany
  • 5German Clinic for Endocrinology, Frankfurt, Germany
  • 6ENDOKRINOLOGIKUM, Osnabrück, Germany
  • 7ENDOKRINOLOGIKUM, Frankfurt, Germany
  • 8Klinikum Bielefeld, Department of Endocrinology, Bielefeld, Germany

The diagnosis of Cushing's disease (CD) is based on endocrinological parameters. In a certain amount of patients MRI fails to detect a pituitary tumor and extensive pituitary exploration may be necessary. We report on our consecutive series of 50 patients who underwent transsphenoidal surgery under the diagnosis of CD. Out of 50 patients (36 f, 14 m, age 20 – 71 ys, mean FU 40 ms) with typical endocrinological findings for ACTH-dependent hypercortisolism (42 primary cases), in 30 cases microadenomas were suspected or identified by MRI, in 5 cases macroadenomas were visible, and in 15 cases MRI (30%) was negative. IPSS was performed in 13 cases. Microsurgical transsphenoidal surgery was performed using neuronavigation (56 x MRI, 1 x CT) and intraoperative ultrasound (N = 47). Decline of serum-cortisol was monitored to assess early remission.

In 25 out of 37 cases (68%) with proven microadenomas HF-ultrasound identified the tumors as hyperechoic masses, 3 were negative, 5 false positive, and 6 were questionable. In the 7 out of 15 cases with false negative MRI, intraoperative ultrasound identified the adenomas correctly. In 44 out of 50 patients decline of cortisol revealed remission of CD (overall remission rate 88%). With proven microadenomas remission rate was 95.2% (40 out of 42), with 2 successfull early reoperations (remission rate 100%). In 2 cases pituitary exploration was negative (4%). In 40 out of 48 cases evaluated so far, anterior pituitary function was intact at follow up (83%). Out of 46 patients with remission 4 developed recurrent hypercortisolism (9%), including 2 out of 39 primary operations (recurrence rate 5%).

The diagnosis of CD remains to be based purely on endocrinological data. Only in equivocal results IPSS is indicated. In MRI-negative cases, intraoperative ultrasound may identify the adenoma and thus prevent harmful exploration of the gland. Surgery for CD should be performed by specialized pituitary surgeons.