Adrenal gland & paraganglia

Adrenal hyperfunction / hyperplasia

Pigmented adrenal cortical hyperplasia

Last author update: 1 February 2013
Last staff update: 4 June 2019

Copyright: 2003-2019,, Inc.

PubMed Search: Pigmented[title] adrenocortical hyperplasia

Nat Pernick, M.D.
Page views in 2022: 762
Page views in 2023 to date: 66
Cite this page: Pernick N. Pigmented adrenal cortical hyperplasia. website. Accessed January 29th, 2023.
Definition / general
  • Also called primary pigmented nodular adrenocortical disease
  • Rare cause of ACTH-independent Cushing syndrome (Mod Pathol 1992;5:23)
  • May be familial, autosomal dominant and associated with Carney complex [cutaneous abnormalities in 80% (lentigenes, blue nevi, ephelides, myxomas), cardiac myxomas (72%, may be life threatening), large cell calcifying Sertoli tumors and Leydig cell tumors of testis (56%), primary pigmented nodular adrenocortical disease with Cushing syndrome (32-45%), myxoid fibroadenomas of breast in females (42%), growth hormone secreting pituitary adenomas (10%), uterine myxomas (8%), oral cavity myxomas (8%) and psammomatous melanotic schwannomas (5%); also neurofibromatosis, cerebral hemangioma]; associated with 2p16 abnormalities
  • Sporadic and non-familial patients are usually infants or age < 30 years
  • Laboratory: moderately elevated plasma cortisol but no diurnal rhythm, resistant to dexamethasone suppression and low / undetectable plasma ACTH
  • Note: Carney complex is also called LAMB syndrome (Lentigenes, Atrial myxomas, Mucocutaneous myxomas, Blue nevi), NAME syndrome (Nevi, Atrial Myxomas, Myxoid neurofibroma, Ephelides) or Swiss syndrome
  • Am J Surg Pathol 1989;13:921, Am J Surg Pathol 1984;8:335
  • Bilateral adrenalectomy
Gross description
  • Variably sized adrenal glands with multiple brown-black pigmented cortical nodules, 1 mm to 3 cm and atrophy of adjacent cortical tissue
  • Nodules may extend into corticomedullary junction or periadrenal fat
Microscopic (histologic) description
  • Sharply circumscribed, but unencapsulated nodules composed of large eosinophilic lipid-poor cells similar to zona reticularis, but often with enlarged pleomorphic nuclei, prominent nucleoli and prominent lipofuscin deposits
  • Also lipid-rich fasciculata-like cells
  • May have focal necrosis, mitotic activity, trabecular growth pattern, myelolipomatous change and lymphocytic infiltrates
Electron microscopy description
  • Zona reticularis and fasciculata type cells
  • Abundant lipofuscin type bodies
Differential diagnosis
  • Melanoma
Back to top
Image 01 Image 02