Endocrine Abstracts

However ACTH secreting adenomas are one of the most common functional pituitary tumors, subtypes such as Crooke cell corticotroph adenoma(CCCA) are relatively rare. We present here a patient with CCCA who had severe hypercortisolism mimicking ectopic ACTH syndrome. A 69 years old female patient admitted to emergency department after a fall. On cranial MRI,’a 3.5x2.5x3cm mass appearance with a sellar-suprasellar location, which is compressing the optic chiasm, expanding the sella to cavernous sinuses’ was detected. It was learned that she gained weight in the last year and his blood sugar had been dysregulated. She had a buffalo hump, thin extremities and central obesity. No signs of abdominal wall striae, facial plethora and proximal myopathy were observed. The biochemistry and anterior pituitary hormone examination results are as in Table 1. The result of 1 mg dexamethasone suppression test was 43.56µg/dl. The midnight serum cortisol was found to be 33µg/dl, the midnight salivary cortisol was 2.24µg/dl and 24 hours urinary free cortisol level was 1718,35 mg/24 h. Spironolactone and metyrapone were started due to resistant hypokalemia and overt hypercortisolemia. In the multidisciplinary council; surgery was decided. After transsphenoidal surgery, focal staining with ACTH and thick membranous staining in more than 50% of cells were detected in the operation material. Pathology result was reported as compatible with CCCA. Metyrapone was discontinued in the post-operative period. The patient who developed central hypothyroidism and central diabetes insipidus was started on levothyroxine and desmopressin treatments. Hypercortisolemia could not be evaluated in the post-operative period;because after the surgery, dexamethasone was given to the patient with subdural hematoma secondary to falling, for anti-edema effect. CCCA are rarely seen tumors. They tend to be invasive and clinically aggressive. They may recur after surgery and may show resistance to re-operation or radiotherapy(1). In our case;there was a pituitary mass pressing on the surrounding tissues, severe hypercortisolemia and rapid progression of clinical and laboratory findings mimicking ectopic ACTH syndrome.

References: 1. Di Ieva A, Davidson JM, Syro LV, Rotondo F, Montoya JF, Horvath E, Cusimano MD, Kovacs K. Crooke’s cell tumors of the pituitary. Neurosurgery. 2015 May;76(5):616-22. doi: 10.1227/NEU.0000000000000657. PMID: 25635886