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Adrenocortical carcinoma
Other Resources UpToDate PubMed

Adrenocortical carcinoma

Contributors: April Randolph, Benjamin L. Mazer MD, MBA
Other Resources UpToDate PubMed

Synopsis

An adrenocortical carcinoma is a rare (1 case per 1 million adults) malignant tumor of the adrenal cortex. The tumor is typically unilateral and may have an irregular shape. Adrenal carcinomas may be found incidentally on imaging or may be identified after a patient shows signs of endocrine dysfunction, such as hypercortisolism or virilization. About half of adrenal carcinomas do not produce hormones, while the other half produce any combination of the normal adrenal cortical hormones. Adrenal carcinomas have a bimodal epidemiology. They are most common in children aged younger than 5 years and in middle-aged adults. Syndromes such as multiple endocrine neoplasia type 1 are uncommonly the cause of adrenal carcinoma.

Adrenocortical carcinoma has a chronic duration, lasting years. Prognosis varies based on staging of the cancer; however, nearly half of all patients have metastases at the time of initial diagnosis.

Codes

ICD10CM:
C74.90 – Malignant neoplasm of adrenal gland

SNOMEDCT:
255035007 – Adrenal carcinoma

Differential Diagnosis & Pitfalls

  • Adrenal adenoma – benign; typically round, homogeneous, and relatively small
  • Pheochromocytoma – typically highly vascular; may show cystic or hemorrhagic changes
  • Adrenal metastasis – typically bilateral
  • Adrenal cyst
  • Adrenal hemorrhage
  • Myelolipoma
  • Cushing syndrome

Best Tests

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Therapy

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References

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Last Updated:02/08/2023
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Adrenocortical carcinoma
A medical illustration showing key findings of Adrenocortical carcinoma : Virilization, Weight gain
Imaging Studies image of Adrenocortical carcinoma - imageId=7878499. Click to open in gallery.  caption: '<span>Post-contrast MRI of the abdomen demonstrates heterogeneously enhancing right adrenal mass.</span>'
Post-contrast MRI of the abdomen demonstrates heterogeneously enhancing right adrenal mass.
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