Adrenal gland & paraganglia
Adrenal hyperfunction / hyperplasia
Pigmented adrenal cortical hyperplasia
Author: Nat Pernick, M.D.
Last author update: 1 February 2013
Last staff update: 4 June 2019
Copyright: 2003-2019, PathologyOutlines.com, Inc.
PubMed Search: Pigmented[title] adrenocortical hyperplasia
Table of Contents
Definition / general | Treatment | Gross description | Microscopic (histologic) description | Electron microscopy description | Differential diagnosisCite this page: Pernick N. Pigmented adrenal cortical hyperplasia. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/adrenalpigmented.html. 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
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
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
