Adrenal gland and paraganglia
Tumors associated with Cushing disease

Topic Completed: 1 February 2014

Minor changes: 16 January 2020

Copyright: 2003-2020,, Inc.

PubMed Search: Adenoma [title] Cushing syndrome adrenal gland

Carmen Perrino, M.D.
Debra Zynger, M.D.
Page views in 2019: 606
Page views in 2020 to date: 246
Cite this page: Perrino C, Zynger D. Tumors associated with Cushing disease. website. Accessed May 27th, 2020.
Definition / general
  • Adrenal cortical adenoma with associated hypercortisolism and clinical manifestations of Cushing syndrome
  • Adrenal cortical adenoma (ACA)
  • Hypercortisolism: sometimes used synonymously for Cushing syndrome
  • Pre-clinical/sub-clinical Cushing syndrome: hypercortisolism in the context of an incidentally discovered adrenal mass without overt clinical manifestations of Cushing 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
  • Adrenal cortex - zona fasciculata
  • ↑Cortisol → ↓corticotropin releasing hormone (CRH), ↓adrenocorticotropic hormone (ACTH); ↑blood glucose
  • 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
  • Discrete adrenal lesion in the context of hypercortisolism and the clinical manifestations of Cushing syndrome
  • ↑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
  • Surgical resection
Clinical images

Images hosted on other servers:

Chubby cheeks, moon facies and central obesity


Moon facies, acne, bitemporal excess hair

CT of abdomen

Adrenal CT scan

Gross images

Images hosted on other servers:

Resected adrenal mass

Well circumscribed ACA

Microscopic (histologic) 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
Microscopic (histologic) images

Images hosted on other servers:

4 month old girl

Virtual slides

Images hosted on other servers:

ACA and Cushing 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
Back to top