Extrapituitary adenomas in Cushing's disease (CD) are less frequently than pituitary
adenomas. Only a few cases of parasellar adenomas are reported in the literature.
We present another rare localization of an extrapituitary adenoma and CD.
A 41- year-old woman presented with increased sweating, arterial hypertension, secondary
amenorrhea, acne, alopecia, headache and leg weakness. The only known previous illnesses
were Hashimoto's Thyroiditis and Iron-deficiency anemia. Cushing's syndrome was confirmed
by elevated levels of serum cortisol, serum ACTH and urinary free cortisol and insufficient
suppression by dexamethasone suppression test (DST). Pituitary MRI, thoracic CT scan
and somatostatin receptor scintigraphy showed no lesion. Inferior sinus petrosus sampling
revealed a central-to-peripheral ACTH gradient suitable for Cushing's disease. In
consequence, endoscopic transsphenoidal exploration and partial resection of the pituitary
was performed. After resection the patient's symptoms persisted. In the further course,
a therapeutic attempt with ketoconazole, pasireotide and cabergoline failed. Therefore,
transsphenoidal hypophysectomy was done. After this, the biochemical analysis still
showed pathological disorders of the corticotrope axis. A newly performed inferior
sinus petrosus sampling indicated furthermore a Cushing's disease. In consequence,
all performed imaging procedures were reevaluated. A lesion in the ethmoidal sinus
was found by 18-FDG-PET scan and MRI. It was first misinterpreted as a polyp. Fifteen months after
the beginning of symptoms and nine months after first detection of hypercortisolism,
resection of the lesion was performed. The histopathological finding was an ACTH positive
extrapituitary adenoma. Post-operative cortisol was below the normal range and ACTH
was not detectable.
