SPONTANEOUS RESOLUTION OF PRIMARY HYPERCORTISOLISM OF CUSHING DISEASE AFTER PITUITARY HEMORRHAGE

Abstract

Objective: To describe a case of spontaneous resolution of primary hypercortisolism from Cushing disease (CD) due to pituitary apoplexy (PA).

Methods: Clinical, laboratory, and pathologic information are described.

Results: A 59-year-old female presented with a headache, a 2.3 cm sellar mass with a questionable hemorrhagic component, and clinical signs of hypercortisolism. On further evaluation, she had an increased 24-hour urine free cortisol, abnormal serum cortisol during a low dose dexamethasone suppression test, and an elevated plasma adrenocorticotropic hormone (ACTH), consistent with pituitary CD. As she was being prepared for surgical resection, she was noted to have spontaneous biochemical remission associated with resolution of her symptoms of hypercortisolism, and a repeat magnetic resonance imaging scan showed shrinkage of the sellar mass. She has been managed conservatively since and remains in clinical/biochemical remission until present time, 18 months following her initial presentation.

Conclusion: We report a case of spontaneous resolution of CD from symptomatic hemorrhage within an ACTH-secreting pituitary adenoma, or PA. This has been rarely reported in the medical literature. The fact that she did not pass through a phase of adrenal withdrawal, makes us suspect a residual functional adenoma within or around the sella which may eventually grow, causing her disease to recur, as has been reported. Hence, continued monitoring will be required.

Fig. 1.

MRI of the pituitary at the time of initial presentation showing an indeterminate sellar/suprasellar heterogeneous mass measuring up to 2 cm (shown by arrows). A, Coronal section without contrast. B, Sagittal section without contrast. C, Coronal section with contrast demonstrating lack of enhancement. D, Sagittal section with contrast demonstrating lack of enhancement. There is extension to the optic chiasm. Original differential diagnosis per neuroradiology included an atypical pituitary macroadenoma, less likely craniopharyngioma, germ cell tumor, or metastasis. MRI = magnetic resonance imaging.

Fig. 2.

Follow-up MRI with contrast of the pituitary showing an interval decrease in the size of the pituitary lesion, retrospectively suggestive of interval resorption of a hemorrhage secondary to an underlying adenoma. A and B, At 2 months. C and D, At 4 months. Hypoenhancing region below the gland is noted, scalloping the sphenoid bone suggestive of a residual adenoma, measuring approximately 8 mm in diameter. There is no residual suprasellar extension or mass effect on the optic chiasm. E and F, Most recent MRI with contrast at 12 months, showing an overall continued mild improvement in the size of the pituitary gland with a slight concavity of its superior surface. Additionally noted was a minimally improved 6 mm focus of delayed enhancement within the pituitary gland, reflecting a residual microadenoma. MRI = magnetic resonance imaging.