30 Jun 2022

7/20/20: Cortisol producing adrenocortical carcinoma

In morning report today, PGY3 Brian Horwich presented a rare case of a 59 year old F with history of hypothyroid and hypertension presenting with 5 days of bilateral lower extremity edema. Review of system was otherwise negative. Physical exam notable for facial plethora, mild hirsuitism and 2+ pitting edema to knees bilaterally.

BMP was significant for the following:

Na: 137/K: 2.3/Cl: 81/HCO3: 41/BUN: 24/Cr: 0.99 < Glucose: 183

VBG: 7.53/49/39/41, lactate 3.5

Cardiac enzyme normal and TTE showed normal EF, heart chambers and valvular function. Bilateral LE duplex was negative for DVTs. Abdominal US showed hepatic steatosis and indeterminate 4.1cm hypoechoic structure with internal echogenicity adjacent or within R hepatic lobe.

CT Chest/Abdomen/Pelvis had the following findings:

Given her lab derangements, Endocrine was consulted on hospital day 1 and recommended hypercortisolism, hyperaldosteronism workup.

Hormone results:

  • ACTH: 5 (normal 6-50)
  • AM cortisol: 45 (normal: 4.8-19.5)
  • PM cortisol: 47.7 (normal: 2.5-11.9)
  • Aldosterone: 80 (high)
  • Renin: 3.89 ng/ml/hr (normal: 0.25-5.82)
  • Serum metanephrines: 37 pg/ml (normal <57), Serum normetanephrines: 52 pg/ml (normal <148)

The patient was started on spironolactone and ketoconazole. She received an adrenal mass biopsy with pathology consistent with adrenocortical carcinoma. Plan with after tumor board is to initiate patient on EDP + Mitotane.

Take home points:

  1. Adrenocortical carcinoma is a rare malignant neoplasm, arising typically between 40-60 years of age at presentation, female predominant (60%) and majority (60%) are functional (hormone producing).
  2. Treatment is surgical if limited to abdomen with adjuvant medical therapy and if advanced (metastatic), recommend medical therapy with mitotane + EDP (etoposide, doxorubicin, cisplatin).

Sentinel Article:

European Society of Endocrinology Clinical Practice Guidelines on the management of adrenocortical carcinoma in adults, in collaboration with the European Network for the Study of Adrenal Tumors

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