Cushing syndrome due to primary pigmented nodular adrenocortical disease: Findings at CT and MR imaging Academic Article uri icon

Overview

MeSH Major

  • Adrenal Cortex Diseases
  • Cushing Syndrome
  • Magnetic Resonance Imaging
  • Tomography, X-Ray Computed

abstract

  • Primary pigmented nodular adrenocortical disease (PPNAD) is a rare cause of Cushing syndrome in infants, children, and young adults. It is characterized by non-adrenocorticotropic hormone-dependent hypersecretion of cortisol by multiple, pigmented nodules of hyperplastic adrenocortical cells. With a single exception, adrenal glands have been described as normal with computed tomography (CT) in all previous series. Eight patients had Cushing syndrome due to surgically proved PPNAD. Four of the eight patients had stigmas of Carney complex (lentigines, calcified Sertoli cell tumors of the testes, and cardiac and soft-tissue myxomas). CT and/or magnetic resonance (MR) imaging demonstrated unilateral or bilateral nodularity in five of six patients examined. Macronodules (greater than 10 mm) were seen in the two oldest patients. As the clinical presentation of Cushing syndrome in this group of patients may be atypical (severe osteoporosis or short stature), the detection of multiple, small adrenocortical nodules with CT or MR imaging supports, or may even suggest, the diagnosis of PPNAD.

publication date

  • January 1989

Research

keywords

  • Academic Article

Identity

Language

  • eng

PubMed ID

  • 2748822

Additional Document Info

start page

  • 415

end page

  • 20

volume

  • 172

number

  • 2