Long Term Results of Transsphenoidal Adenomectomy in Patients with Cushing's Disease

Abstract

As part of an ongoing series, 100 patients with Cushing's disease underwent transsphenoidal operations. Pituitary adenomas were confirmed in 93 patients, and initial remission was achieved in 86 (92%) of them. Hypercortisolemia was not corrected in 7 patients, and in 4 this was due to invasive adenomas. These patients were subjected to irradiation, medical treatment, or both after operation. Only 7 of the 100 patients had no pituitary adenoma found at operation, and they obtained no clinical remission even after partial or subtotal hypophysectomy. Follow-up review, with an emphasis on endocrinological studies, was performed on these patients for a mean period of 38 months. Seventy-eight patients were in long term remission after operation and had restoration of noncorticotropic hormone secretion as well as pituitary-adrenal function. Recurrence was noted in 8 patients after 19 to 82 months in remission. In all of these patients, pituitary adenomas were verified by reoperation and no case of corticotrophic cell hyperplasia was noted. We conclude that late recurrence of Cushing's disease may occur after adenoma removal and is due to the regrowth of adenoma cells left behind in the peritumoral tissue at the first operation. In view of the overall remission rate, transsphenoidal adenomectomy is considered a highly effective treatment for Cushing's disease.

Supra- and Extrasellar Pituihry Microadenoma as a Cause of Cushing's Disease.

“…The rate of negative pituitary exploration was between 5 and 7% [1,14], and the rate of postoperatively established extracranial ectopic ACTH-producing tumor was about 2% [1,14]. [7,81, in the cavernous sinus [11] or in the supraand extrasellar region as in our case.…”

“…In several institutions the initial remission rate after transsphenoidal microsurgery in cases preoperatively diagnosed with Cushing's disease is between 79 and 86% [1,14,15]. The rate of negative pituitary exploration was between 5 and 7% [1,14], and the rate of postoperatively established extracranial ectopic ACTH-producing tumor was about 2% [1,14].…”

“…ACTH-producing pituitary adenomas causing Cushing's disease are usually small and located in the sella turcica [1], but many papers have reported that pituitary adenomas may be located in various extrasellar parts of the cranial cavity [2][3][4][5][6][7][8][9][10][11]. We report the first case of Cushing's disease caused by supra-and extrasellar microadenoma, which originated in the anterior pituitary and extended upward without connecting to the stalk.…”

Adrenal Insufficiency after Incomplete Resection of Pituitary Macrocorticotropinoma of Cushing's Disease. Role of High Molecular Weight ACTH.

“…The reference range was 2.0-11.5 pmol/l. Cross reactivity with j3-LPH was 0.01%, and was far less than 0.01% with other POMC-related peptides, [11][12][13][14][15][16][17][18][19][20][21][22][23][24][1][2][3][4][5][6][7][8][9][10]. Plasma cortisol was measured by a Gammacoat cortisol kitTM (Incstar Co., Stillwater, MN, USA), and the reference range was 110-505 nmol/1.…”

“…According to the numerous reports on large populations of patients with Cushing's disease [1][2][3][4][5][6][7][8][9][10][11] including children and adolescents [10,11 ], pituitary macroadenomas larger than 10 mm in diameter could be found in 261 of a total of 1621 patients (16.1%). However, among children and adolescents, the percentage of macroadenoma was found to be much less than this series for adults [10][11][12].…”