Pituitary apoplexy in patients with
adrenocorticotropic hormone (
ACTH) producing
tumors is a rare occurrence. We report four patients with
Cushing's disease harboring
ACTH-secreting macroadenomas who presented with
pituitary apoplexy. We report the endocrinologic and visual outcomes of these patients after emergent transsphenoidal surgery. A retrospective chart review was performed in 4 patients who presented with
pituitary apoplexy from
hemorrhage into an
ACTH-secreting pituitary adenoma. The patient charts were reviewed for clinical presentation, neuroimaging findings, intraoperative surgical findings, pathologic findings, and postoperative endocrinologic and visual outcomes. All patients presented with acute
headaches,
nausea,
vomiting, and visual loss from optic compression. MR imaging demonstrated a hemorrhagic macroadenoma that was confirmed at surgery. All patients underwent emergent transsphenoidal
decompression (within 24 h of presentation). One of these underwent an additional
craniotomy to resect
residual tumor. Postoperatively, all patients showed significant improvement in visual acuity and visual fields with biochemical remission confirmed on laboratory testing. Significant
weight loss as well as resolution of diabetes and
hypertension was noted in all cases. All four patients remained in biochemical remission at their most recent follow-up visit (mean 40 months, range: 24-72 months). Excellent endocrine and visual outcomes can be achieved after emergent transsphenoidal surgery in patients with
Cushing's disease presenting with
pituitary apoplexy. Although the cure rates of non-apoplectic
ACTH macroadenomas are generally poor, higher rates of remission can be achieved in cases of
pituitary apoplexy. This may be partly due to the effects of
tumor infarction.