Introduction: Carney Complex (CNC) is a familial neoplasia syndrome that is associated with pituitary-associated hypersecretion of growth hormone (GH; acromegaly). The underlying cause of pituitary GH-hypersecretion and its management have been incompletely defined. To provide biologic insight into CNC-associated pituitary pathology and improve management, we analyzed findings in CNC patients that underwent transsphenoidal surgery.
Methods: Consecutive CNC patients at the National Institutes of Health with acromegaly and imaging evidence of a pituitary adenoma(s) that underwent transsphenoidal resection of tumor(s) were included. Prospectively acquired magnetic resonance (MR)-imaging, biochemical, surgical and histology data were analyzed.
Results: Seven acromegalic CNC patients (2 male, 5 female) were included. Mean age at surgery was 29.7 years (range, 18 to 44 years). Mean follow-up was 4.7 years (range, 0.2 to 129 months). MR-imaging revealed a single pituitary adenoma in 4 patients and multiple pituitary adenomas in 3 patients. While patients with single discrete pituitary adenomas underwent selective adenomectomy, patients with multiple adenomas underwent selective adenomectomy of multiple tumors (1 patient), as well as partial (1 patient) or total hypophysectomy (1 patient). All adenomas were either GH- and PRL-positive or exclusively PRL-positive. Pituitary tissue surrounding the adenomas in patients with multiple adenomas revealed hyperplastic GH- and PRL-positive tissue.
Conclusions: CNC-associated acromegaly results from variable pituitary pathology, including singular GH-secreting adenomas or multiple GH-secreting adenomas and/or GH-hypersecretion of the pituitary gland surrounding multiple adenomas. While selective adenomectomy is the preferred treatment for cases of GH-secreting adenomas, multiple adenomas with associated pituitary gland GH-hypersecretion may require partial or complete hypophysectomy to achieve biochemical remission.
Patient Care: It will provide understanding into the clinical and surgical management of acromegaly in Carney Complex.
Learning Objectives: 1. To develop management strategies for treatment of pituitary adenomas in Carney Complex.
2. To better understand the pathobiology of pituitary adenomas in Carney Complex.
3. To better understand management of endocrinopathy in Carney Complex.