Cushing's Disease Due to Recurrent Cystic Macroadenoma

Crooke's tumor is a rare histological type of ACTH-producing adenomas. The most frequent clinical presentation is as Cushing's syndrome in middle-aged women and on other occasions as silent corticotropic adenomas. We present the case of a patient with recurrent Cushing's disease due to cystic Crooke's tumor.

Case Description:

A 44-year-old female patient, history of hypertension and smoking; She went to the emergency room due to a sudden onset holocranial headache, associated with nausea, vomiting and deterioration of bilateral visual acuity of one week of evolution. On physical examination: obesity, cushingoid facies, decreased bilateral visual acuity with greater involvement of the left eye.

Analytical: ACTH 111 pg/ml (0-46), 8am serum cortisol: 27.5 ug/ml (5-25), postdexa-1 cortisol: 17.70 ug/ml, urinary free cortisol: 249 and 389 ug/24h (7 - 96); DHEAS: 225 ug/dL; PRL: 31 ng/mL (1.9-25.0); IGF1: 114 ng/mL (101-267); FSH: 4.37 mIU/mL (2.8-11.3); estradiol: 41.8 pg/mL (0-160); TSH: 1.34 uIU/mL (0.4-4.0); FT4: 0.925 ng/dL (0.8-1.9). MRI of the pituitary gland revealed a 30 x 18 mm macroadenoma with a cystic appearance compressing the optic chiasm. She underwent transsphenoidal adenomectomy, The pathologist confirmed the presence of a cystic tumor of approximately 30x14 mm, with a friable consistency and with a yellowish citric content and little vascularity, the histopathology concluded adenoma with signet ring cells and the IHC was positive for ACTH, pankeratin, chromogranin, low CK7 molecular weight and vimentin. She presented with remission of hypercortisolism, improvement of vision up to 15 months after she presented cafelea again, visual compromise, ACTH: 149 pg/mL (7.2-63.5); urinary free cortisol: 398nmol/24h (30-197); The control MRI showed an expansive intrasellar process with a cystic appearance and mild signal hypointensity in T1, hyperintensity in T2, it measures 12 x 18 x 13 mm.

Discussion:

Crooke tumors in imaging studies usually present as macroadenoma (77.2%) with invasion of the cavernous sinuses (79.2%), although cases of metastasis (7.5%) have also been reported. Crooke's tumor can be silent or clinically functioning and usually has a torpid course with recurrences requiring multimodal treatment.