Case 53 - Discussion

Uploaded: 2007-06-22, Updated: 2007-12-27

 

Adrenal Cortical Hyperplasia

CLINICAL FEATURES

  • Can be diffuse, localized with formation of one or more nodules;

  • Almost always bilateral;

  • Most cases of diffuse cortical hyperplasia are ACTH dependent, over production from the pituitary gland or a lung tumor;

  • Most cases of nodular cortical hyperplasia are unrelated to ACTH production, and are ACTH independent or adrenal dependent;

  • A dominant macronodule might be regarded as an adenoma even without symptoms or coexistence of diffuse or micronodular hyperplasia.

GROSS FINDINGS

  • Often bilaterally diffuse or multiple nodules;

  • Nodules are commonly extrusions of cortex, may project in a hemispheric or "detached" nodule depending on the plane of section;

  • The nodules appear discrete and sharply demarcated, and some dominant macronodules may appear encapsulated.

MICROSCOPIC FINDINGS

  • Variable architectural patterns: alveolar, trabecular, gyriform ribbon-like, pseudoglandular;

  • Nodules are not well-demarcated and blend almost imperceptibly with the adjacent hyperplastic cortex;

  • Some nodules are almost entirely composed of lipid-rich cells (yellow nodules grossly);

  • Variable mixture of pale-staining, lipid-rich vacuolated cells and lipid-depleted cells with compact, eosinophilic cytoplasm;

  • Rarely cells with "ballooned" vacuolated cytoplasm are present;

  • Nuclear pleomorphism is often inconspicuous and mitotic figures are rare;

  • Pseudoglandular foci may be present with stringy lightly basophilic material, but PAS- and glycogen-;

  • Occasionally, lipomatous or myelipomatous foci, or rare bony metaplasia.

CLASSIFICATION

  • Diffuse hyperplasia

  • Nodular hyperplasia

    • Micronodular (<1.0cm)

    • Macronodular (>1.0cm)

    • Combined micronodular and macronodular

  • Combined diffuse and nodular hyperplasia

  • Macronodular hyperplasia with marked adrenal enlargement

  • Primary pigmented nodular adrenocortical disease: part of Carney's syndrome

  • Incidental pigmented nodules

DIFFERENTIAL DIAGNOSES

  • Adrenal adenoma: usually single, unilateral and well-demarcated.

REFERENCES

  • AFIP, tumor of the adrenal glands and extra-adrenal paraganglia, series 3;

  • Rosai and Ackerman's Surgical Pathology, 9th edition.