• Also called primary pigmented nodular adrenocortical disease
  
• Rare cause of ACTH-independent Cushing’s syndrome (Mod Pathol 1992;5:23)
  
• May be familial, autosomal dominant and associated with Carney’s 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’s 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’s 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
  

• 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
  

• 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
  

• Zona reticularis and fasciculata type cells
  
• Abundant lipofuscin type bodies
  

• Melanoma
  

This information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must be interpreted in the context of a patient's clinical data using reasonable medical judgment. This website should not be used as a substitute for the advice of a licensed physician.

All information on this website is protected by copyright of PathologyOutlines.com, Inc. Information from third parties may also be protected by copyright. Please contact us at copyrightPathOut@gmail.com with any questions (click here for other contact information).