Adrenal gland and paraganglia
Adrenocortical adenoma
Tumors associated with Cushing disease

Author: Carmen Perrino, M.D. (see Authors page)

Editor: Debra Zynger, M.D.

Revised: 20 January 2016, last major update February 2014

Copyright: (c) 2003-2016, PathologyOutlines.com, Inc.

PubMed Search: Adenomas [title] Cushing's syndrome
General
  • Adrenal cortical adenoma with associated hypercortisolism and clinical manifestations of Cushing’s syndrome
Terminology
  • Adrenal cortical adenoma (ACA)
  • Hypercortisolism: sometimes used synonymously for Cushing’s syndrome
  • Pre-clinical/sub-clinical Cushing’s syndrome: hypercortisolism in the context of an incidentally discovered adrenal mass without overt clinical manifestations of Cushing’s syndrome (Arq Bras Endocrinol Metabol 2007;51:1272)
  • Primary hypercortisolism: due to secretion of cortisol by the adrenal gland versus secondary hypercortisolism: due to increased secretion of ACTH by pituitary or to secretion of cortisol by an ectopic tumor
Epidemiology
Sites
  • Adrenal cortex - zona fasciculata
Pathophysiology
  • ↑Cortisol → ↓corticotropin releasing hormone (CRH), ↓adrenocorticotropic hormone (ACTH); ↑blood glucose
Etiology
  • Neoplastic proliferation of functioning adrenal cortical cells producing cortisol
Clinical Features
  • Central obesity, moon facies, plethora, striae, thin skin, easy bruising, hirsutism, telangiectasias, hyperhidrosis, myopathy, osteoporosis, decreased libido
Diagnosis
  • Discrete adrenal lesion in the context of hypercortisolism and the clinical manifestations of Cushing’s syndrome
Laboratory
  • ↑Cortisol, ↓CRH, ↓ ACTH, hyperglycemia
Radiology Description
Prognostic Factors
  • In general, most reliable factors to exclude an adrenal cortical carcinoma are size, necrosis, mitotic activity, and atypical mitoses (Mod Pathol 2011;24:S58)
  • When distinguishing ACA from carcinoma (ACC), several criteria including the Weiss and Modified Weiss have been proposed (see Adrenal cortical adenoma – general)
Case Reports
Treatment
  • Surgical resection
Clinical Images

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Chubby cheeks, moon facies and central obesity

Post-operative

Moon facies, acne, bitemporal excess hair

CT of abdomen

Adrenal CT scan

Gross Images

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Resected adrenal mass

Well circumscribed ACA

Micro Description
  • Pushing border, may be unencapsulated or surrounded by fibrous pseudocapsule
  • Architectural patterns: nesting, alveolar, cords, trabeculae
  • In comparison to uninvolved adrenal cortex, cells are large with distinct cytoplasm
  • Vacuolated, clear-appearing cytoplasm that is lipid rich
  • Distinct cell borders
  • Nuclei are relatively uniform; occasionally may have focal cytologic atypia
Micro Images

4 month old girl

Virtual Slides

ACA and Cushing’s syndrome

Cytology Description
Negative Stains
  • EMA, CEA, B72.3, S100, chromogranin (stains adrenal medulla), vimentin, carbonic anhydrase IX (CAIX)
Electron Microscopy Description
Molecular / Cytogenetics Description
Differential Diagnosis