Adrenocorticotropin-secreting pituitary macroadenomas: expanding the clinical spectrum.
ACTH-secreting macroadenomas can lead to severe systemic disease and require lifelong follow-up after treatment.
Where it sits
this study against the rest of the pasireotide corpusSummary and findings
Three patients with adrenocorticotropin-secreting pituitary macroadenomas were described, all presenting with hypercortisolism and systemic complications. Biochemical testing confirmed ACTH-dependent hypercortisolism, and all patients underwent endoscopic transsphenoidal surgery. One patient achieved remission while two developed recurrence.
Abstract
Cushing disease (CD) secondary to adrenocorticotropin (ACTH)-secreting pituitary macroadenomas is uncommon, accounting for 10% to 15% of cases. We describe 3 patients from South Africa who presented with overt clinical features of hypercortisolism and considerable systemic complications, including hypertension, diabetes mellitus, and osteoporosis. Biochemical testing confirmed ACTH-dependent hypercortisolism with hypopituitarism and mild hyperprolactinemia consistent with stalk effect. Magnetic resonance imaging demonstrated pituitary macroadenomas, measuring 10 mm or greater, with parasellar extension. Mass effect was observed in 1 patient resulting in cranial nerve III palsy, while the others had visual field deficits secondary to optic chiasm compression. All patients underwent endoscopic transsphenoidal surgery, with 1 achieving remission and 2 developing recurrence. These cases highlight that ACTH-secreting macroadenomas can present with severe systemic disease and significant mass effect, and emphasize the importance of lifelong follow-up.
Background
This paper addresses the clinical presentation and complications associated with ACTH-secreting pituitary macroadenomas, which are a rare cause of Cushing disease. Prior knowledge indicates that these tumors can lead to significant systemic issues, but detailed case studies are limited. Understanding the clinical spectrum of these macroadenomas is crucial for improving diagnosis and management.
Methods
The study describes three patients from South Africa diagnosed with ACTH-secreting pituitary macroadenomas. All patients presented with overt hypercortisolism and underwent endoscopic transsphenoidal surgery. The primary outcome measured was surgical remission, while secondary outcomes included recurrence rates and complications.
Results
One patient achieved remission post-surgery, while two patients experienced recurrence. The abstract does not provide specific numeric findings related to hormonal levels or other quantitative measures.
Interpretation
The findings align with existing literature that highlights the severe systemic effects of ACTH-secreting macroadenomas. However, the clinical significance of the remission rate is uncertain due to the small sample size and lack of comprehensive follow-up data. The study's limitations, including the absence of long-term outcomes and the small n, restrict the ability to generalize these findings.
Key findings
- ACTH-secreting pituitary macroadenomas account for 10% to 15% of Cushing disease cases.
- Pituitary macroadenomas measured 10 mm or greater with parasellar extension.
- One patient experienced cranial nerve III palsy due to mass effect.
- Two patients developed recurrence after surgery.
Limitations
- small n=3 case series
- no long-term follow-up data reported
- lacks quantitative hormonal data
- single-site study