Transsphenoidal surgery in Cushing disease: 10 years of experience in 34 consecutive cases.
OBJECTIVE
Cushing disease is a rare disorder. Because of their small size the adrenocorticotropic hormone (ACTH)-producing tumors are often not detectable on neuroimaging studies. To obtain a cure with transsphenoidal surgery (TSS) may therefore be difficult. In this report the authors present 10 years of experience in the treatment of patients with Cushing disease who were followed up with the same protocol and treated by the same surgeon.


METHODS
Thirty-four patients, 26 of them female and eight of them male (mean age 40 years, range 13-74 years) were studied. All had obvious clinical signs and symptoms of Cushing syndrome. Magnetic resonance (MR) imaging was performed in all patients, and inferior petrosal sinus (IPS) sampling was done in 14. In 12 patients MR imaging indicated a pituitary tumor; 10 were microadenomas and two were macroadenomas. In six patients with no visible tumor, the results of IPS sampling supported the diagnosis. All patients underwent TSS; the mean follow-up duration was 6 +/- 0.5 years. Selective adenomectomy was performed in 32 and hemihypophysectomy in the other two patients. A cure was obtained in 31 patients (91%) after one TSS and in two more patients after further TSS; one patient was not cured despite two TSSs and one underwent bilateral adrenalectomy. Disease recurrence was seen in two patients after 3 years, and they were successfully treated with stereotactic gamma knife surgery. Half of the patients had an ACTH deficiency postoperatively, whereas one third had other pituitary hormone insufficiencies. There were no serious complications attributable to the surgical intervention.


CONCLUSIONS
Transsphenoidal surgery with selective adenomectomy is an effective and safe treatment for Cushing disease. In the patients presented in this study, the surgical outcome seemed to depend on careful preoperative evaluation and the surgeon's experience. For optimal results in this rare disease the authors therefore suggest that the endocrinological, radiological, and surgical procedures be coordinated in a specialized center.
Höybye
Charlotte
C
Department of Endocrinology and Diabetology, Karolinska Hospital, Stockholm, Sweden. charlotte.hoybye@ks.se
Grenbäck
Eva
E
Thorén
Marja
M
Hulting
Anna-Lena
AL
Lundblad
Lars
L
von Holst
Hans
H
Anggård
Anders
A
D000236:Adenoma / Q000150:complications* / Q000601:surgery*; D000293:Adolescent; D000328:Adult; D000368:Aged; D003480:Cushing Syndrome / Q000601:surgery*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D019635:Neurosurgical Procedures / Q000379:methods*; D010911:Pituitary Neoplasms / Q000150:complications* / Q000601:surgery*; D016634:Radiosurgery / Q000379:methods*; D012008:Recurrence; D012189:Retrospective Studies; D013100:Sphenoid Bone / Q000601:surgery; D016896:Treatment Outcome