Adrenal gland and paraganglia
Adrenal hyperfunction / hyperplasia
Pigmented adrenal cortical hyperplasia

Author: Nat Pernick, M.D. (see Authors page)

Revised: 1 March 2016, last major update February 2013

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PubMed Search: Pigmented[title] adrenocortical hyperplasia
Cite this page: Pigmented adrenal cortical hyperplasia. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/adrenalpigmented.html. Accessed December 7th, 2016.
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
Treatment
  • 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
Micro 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