gms | German Medical Science

Objective: Thunderclap headache and visual disturbances are clinical features of pituitary apoplexy (PA), the sudden infarction of and/or hemorrhage into a preexisting pituitary adenoma. Not all adenoma subtypes seem to be at equal risk for PA, which occurs most often in non-functioning pituitary macroadenomas. Adenocorticotroph hormone (ACTH) secreting tumors leading to the clinical entity of Cushing’s disease (CD) are almost exclusively microadenomas and are most often diagnosed via the typical clinical features of the underlying endocrinopathy. In the literature, only isolated cases of PA in ACTH-secreting pituitary adenomas are reported, the largest series so far comprising 4 patients.

Methods: Within the framework of a retrospective analysis, we reviewed the chart records of patients with proven PA (by means of MRI imaging and/or histology) referred to the neurosurgical department of our university with respect to the typical clinical features of CD, positive ACTH-immunostaining and/or recurrence of CD after PA in order to identify infarcted ACTH-secreting adenomas.

Results: Of 69 patients with PA (31 male, 38 female), 7 presented prior to PA with the typical clinical features of CD and in two cases with positive immunostaining for ACTH-secreting pituitary adenoma there were no clinical signs of hypercortisolism. Histopathology revealed positive ACTH-immunostaining in 7 cases and only necrosis in 2 cases. All patients underwent transsphenoidal surgery. In 2 patients clinical features of CD in addition to biochemical disease recurrence were seen 7-9 years after PA, necessitating medical or radiotherapy.

Conclusion: The in proportion to the overall study group high number of patients with PA and underlying CD/ACTH-staining adenomas leads us to conclude that hemorrhage into this adenoma type is not such a rare entity as previously thought. Specific risk factors, like coagulation disorders and metabolic syndrome may play a role in the pathophysiology of PA in patients with CD. Surgical removal of the pituitary mass does not only allow decompression of the optic chiasm but also the collection of tissue samples, which is crucial for further therapy. Moreover, neurosurgeons should be reminded to take a careful clinical history, taking into account endocrinological signs and symptoms. Patients must be counseled to adhere to long-term neurosurgical and endocrinological follow-up.