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World Neurosurg. 2021 Nov 17; doi: 10.1016/j.wneu.2021.11.049. Epub 2021 Nov 17.

Functioning Crooke Cell Adenomas: Case Series and Literature Review.

World neurosurgery

Erica A Giraldi, Stewart G Neill, Pia Mendoza, Amit Saindane, Nelson M Oyesiku, Adriana G Ioachimescu

Affiliations

  1. Emory University School of Medicine, Department of Medicine, Division of Endocrinology, Metabolism and Lipids, 1365 Clifton Rd NE, Atlanta, GA 30322, USA; Emory University School of Medicine, Department of Neurosurgery, 1365 Clifton Rd NE, Atlanta, GA 30322, USA. Electronic address: [email protected].
  2. Emory University School of Medicine, Department of Pathology and Laboratory Medicine, 1364 Clifton Rd NE, Atlanta, GA 30322, USA.
  3. Emory University School of Medicine, Department of Radiology, 1364 Clifton Rd NE, Atlanta, GA 30322, USA.
  4. Emory University School of Medicine, Department of Medicine, Division of Endocrinology, Metabolism and Lipids, 1365 Clifton Rd NE, Atlanta, GA 30322, USA; Emory University School of Medicine, Department of Neurosurgery, 1365 Clifton Rd NE, Atlanta, GA 30322, USA.

PMID: 34800730 DOI: 10.1016/j.wneu.2021.11.049

Abstract

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.

Copyright © 2021 Elsevier Inc. All rights reserved.

Keywords: Crooke cell adenoma; Cushing’s disease; pituitary adenoma; silent ACTH adenomas

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