LAUSR

BACKGROUND Crooke cell adenomas (CCA) are rare potentially aggressive pituitary adenomas. Data regarding prevalence and clinical course is sparse. METHODS Methods consisted of a retrospective review of 59 consecutive functioning corticotroph adenomas (FCA) operated between October 2017-November 2020, and a literature review of CCA publications since 1991. RESULTS The prevalence of CCA among FCA at our institution was 8.5% (5/59). In the 4 other surgical cases series, prevalence of CCA ranged between 0%-6.8%. Our patients, 4 women and 1 man, mean age 46±11 years, presented with hypercortisolism (3/5), vision loss (1/5) and incidentally (1/5). All patients had elevated ACTH (151±54 pg/ml) and urinary free cortisol (830±796.5 μg/day) levels. Radiologically, 3 tumors were macroadenomas and 2 had cavernous sinus invasion. All patients achieved biochemical remission at 3 months postoperatively. One patient with a giant pituitary adenoma underwent fractionated radiation for residual tumor. During follow-up (range: 3.1-31.0 months), no patients had evidence of radiological or biochemical recurrence. The literature review (3 case series) and 21 case reports identified 22 FCA with outcome data. Additional treatments included reoperation (50%), radiation (59%), bilateral adrenalectomy (23%) and Temozolomide (36%). CONCLUSION We report a higher CCA prevalence among functioning ACTH-adenomas after the implementation of 2017 WHO classification. In our series and the literature, most CCAs were macroadenomas with high ACTH levels. Postoperative outcomes were excellent in our series, while some cases from the literature were refractory to standard treatments. Larger clinical and molecular studies are needed to identify patients at risk.