Management of ACTH-secreting supradiaphragmatic adenomas

Dam-Hieu, Phong; Irthum, Bernard; Seizeur, Romuald; Roudaut, Nathalie; Besson, Gérard

doi:10.1016/j.clineuro.2007.04.009

« Previous Next »Clinical Neurology and Neurosurgery
Volume 109, Issue 8 , Pages 698-704, October 2007

Management of ACTH-secreting supradiaphragmatic adenomas

Phong Dam-Hieu
- Department of Neurosurgery, University of Bretagne occidentale, Faculty of Medicine, F 29609 Brest, France
- Corresponding author at: Department of Neurosurgery, University Medical Center, Bd Tanguy-Prigent, F29609 Brest France. Tel.: +33 298 347 689; fax: +33 298 342 337.
Bernard Irthum
- Department of Neurosurgery, University of Auvergne, Faculty of Medicine, F 63000 Clermont-Ferrand, France
Romuald Seizeur
- Department of Neurosurgery, University of Bretagne occidentale, Faculty of Medicine, F 29609 Brest, France
Nathalie Roudaut
- Department of Endocrinology, University of Bretagne occidentale, Faculty of Medicine, F 29609 Brest, France
Gérard Besson
- Department of Neurosurgery, University of Bretagne occidentale, Faculty of Medicine, F 29609 Brest, France

Received 4 February 2007; received in revised form 13 April 2007; accepted 19 April 2007.

Abstract

Supradiaphragmatic adrenocorticotropic hormone (ACTH) secreting pituitary adenomas are exceptionally encountered (14 cases previously described) and raise issues concerning their nosology and management. If surgery is the treatment of choice, the nature of surgical approach (craniotomy, transsphenoidal approach) remains controversial. To illustrate this issue, we presented two cases of supradiaphragmatic ACTH secreting pituitary adenomas successfully excised via a subfrontal approach. Both patients were female (20 and 41years) and had a typical Cushing's syndrome. MRI revealed, in both cases, a suprasellar mass in contact with the pars tuberalis of the pituitary. In the first case, the patient underwent initially a transsphenoidal approach with negative exploration and subsequent partial hypophysectomy. One year later, the patient was operated on again via a subfrontal approach, allowing excision of a supradiaphragmatic adenoma and a complete cure of Cushing's disease. In the second case, the patient underwent initially a subfrontal approach and was definitely cured. In both cases, the diaphragma sellae was found to be intact and the pituitary stalk could be preserved. Postoperative MRI demonstrated a clearly visible intact pituitary stalk in conjunction with normal aspect of the pituitary. Supradiaphragmatic pituitary adenomas are most likely adenomas of the pituitary stalk with extra-axial development. Surgery remains the treatment of choice. Should the superior approach be preferred, the transsphenoidal-transtuberculum sellae approach may represent a viable alternative when performed by a well-trained surgical team. Surgery may be difficult, and drawbacks are non negligible, particularly in elderly patients. In these cases, stereotactic radiosurgery should receive more consideration.