Adrenal cortical adenoma: the fourth component of the Carney triad and an association with subclinical Cushing syndrome

Am J Surg Pathol. 2013 Aug;37(8):1140-9. doi: 10.1097/PAS.0b013e318285f6a2.


The Carney triad is the combination of gastric stromal sarcoma, pulmonary chondroma, and extra-adrenal paraganglioma. Herein, we describe the clinical, imaging, pathologic, and follow-up findings from 14 patients for a fourth component of the syndrome, adrenal adenoma. The adrenal neoplasm was asymptomatic and usually a late finding. Results of adrenocortical function tests were normal. Computed tomography revealed low-density adrenal masses that were consistent with adenomas. Bilateral lesions were present in 4 patients. In 13 of the 14 patients who underwent surgery, resected adrenal glands and biopsy specimens featured 1 or more circumscribed, yellow tumors, up to 3.5 cm in diameter, composed of well-differentiated polygonal cells with clear vacuolated cytoplasm and a smaller component of eosinophilic cells. The extratumoral cortex had combinations of normal histologic features, discrete clear cell micronodules, zonal clear cell hypertrophy, and marked atrophy. The lesion in the 14th patient was different, grossly and microscopically resembling the usual sporadic cortisol-secreting adenoma. After the tumor was excised, the patient required glucocorticoid support. None of the tumors recurred or metastasized. Fourteen additional patients had unilateral or bilateral adrenal tumors consistent with adenomas detected by imaging studies.

Publication types

  • Research Support, N.I.H., Intramural

MeSH terms

  • Adolescent
  • Adrenal Cortex Function Tests
  • Adrenal Cortex Neoplasms / chemistry
  • Adrenal Cortex Neoplasms / pathology*
  • Adrenal Cortex Neoplasms / surgery
  • Adrenalectomy
  • Adrenocortical Adenoma / chemistry
  • Adrenocortical Adenoma / pathology*
  • Adrenocortical Adenoma / surgery
  • Adult
  • Asymptomatic Diseases
  • Biomarkers, Tumor / analysis
  • Biopsy
  • Carney Complex / metabolism
  • Carney Complex / pathology*
  • Carney Complex / surgery
  • Child
  • Cushing Syndrome / metabolism
  • Cushing Syndrome / pathology*
  • Cushing Syndrome / surgery
  • Diagnostic Imaging / methods
  • Female
  • Humans
  • Immunohistochemistry
  • Male
  • Middle Aged
  • Predictive Value of Tests
  • Treatment Outcome
  • Young Adult


  • Biomarkers, Tumor