Failure of partial hypophysectomy as definitive treatment in cushing?s disease owing to nodular corticotrope hyperpiasia; report of four cases

[~MaryO~]

From http://www.springerlink.com/content/10734t6217735304/

 

Luiz R. Salgado, Berenice B. Mendon, Jayme Goldman, Mauro Semer, Mirta Knoepfelmacher, Ana M. Tsanaclis, Bernardo L Wajchenberg and Bernardo Liberman

(1) Endocrine Service, Hospital das Cl?nicas, P.O. Box 8091, 05403-900, S?o Paulo, Brazil

(2) Hospital das Cl?nicas, S?o Paulo University, S?o Paulo, Brazil

 

Abstract Nodular corticotrope hyperpiasia is a rare pathology causing Cushing?s syndrome owing to a primary pituitary disease or ectopic CRH production. In this study, we evaluated the laboratory and pathological findings and results of transsphenoidal pituitary surgery in four patients with Cushing?s disease. Dynamic tests of pituitary-adrenal function (dexamethasone suppression, metyrapone, CRH, and DDAVP tests) were done before and after transsphenoidal pituitary surgery. Plasma and total urinary cortisol, serum 11-deoxycortisol, and plasma ACTH were determined by RIA. Hormonal dynamic tests and radiologic studies were compatible with a pituitary ACTH source. The transsphenoidal surgery revealed the presence of corticotrope hyperpiasia confirmed by immunoperoxidase stain and a preserved reticulum framework in the removed pituitary tissue of these four patients. The pituitary surgery led to a short period of improvement in two of the patients (1 and 4), a 3-yr remission in one patient (patient 2), and no improvement in one (patient 3). We conclude that although our patients appear to have inadequate suppression with high-dose dexamethasone, there is no way to diagnose this pathology presurgically, and that total hypophysectomy, bilateral adrenalectomy, and irradiation are the only alternatives for definitive treatment. A CRH-secreting ectopic tumor could not be found in our patients either before or after surgery in the follow-up period.

 

Key Words ACTH-dependent hypercortisolism - nodular corticotrope hyperpiasia - adrenalectomy - hypophysectomy

[ADDflower]

Gee... that sounds like a lot of "us" on the board these days...

 

I wish some of these doctors would come ask people from this board to volunteer medical records for their studies. I can't think of too many that would say no.

[Kathryn G]

MaryO,

Thanks for posting this. I know that from articles I have read that treating hyperplasia is much more difficult. Hopefully more knowledge is gained down the road and other people do not need to suffer.

Kate G