Adrenal (Excluding Mineralocorticoids)

SAT-488 - Adrenocortical Carcinoma (ACC) Presenting with Severe Mineralocorticoid Excess and Partial Adrenal Insufficiency: A Possible DOC-Producing Tumor

Saturday, June 13, 2026

9:30 AM - 4:30 PM CT

Location: ENDOExpo; Poster Floor

Abstract Poster Presenter(s)

Tal Netser, MD (she/her/hers)

Endocrinology Fellow
ICHILOV/SOURASKY MEDICAL CENTER
Herzeliya, ISRAEL

Senior Author/Primary Investigator(s)

Tal Netser, MD (she/her/hers)

Endocrinology Fellow
ICHILOV/SOURASKY MEDICAL CENTER
Herzeliya, ISRAEL

Co-Author(s)

RH

Rabia Hamadi, MD

physician
TEL-AVIV Sourasky Medical Center
haifa, Israel
IW

Ido Wolf, MD

Director of Oncology Division
Tel Aviv Sourasky Medical Center
tel aviv, Israel
YG

Yona Greenman, MD PhD (she/her/hers)

Head of Institute of Endocrinology, Diabetes, Metabolism and Hypertension, Tel Aviv Sourasky Medical
Tel Aviv Sourasky Medical Center
Tel Aviv, Israel
SM

Sher Matsri, MD

Institute of Endocrinology, Metabolism and Hypertension, Tel Aviv-Sourasky Medical Center
Tel Aviv, HaMerkaz, Israel
NY

Noam Yaniv, MD

Tel Aviv Sourasky Medical Center
Tel Aviv, Israel

Senior Author/Primary Investigator(s)

EO

Etty Osher, MD, PHD

Physician
Tel Aviv Sourasky Medical Center, Tel Aviv, Israel
Rishon, Israel

Co-Author(s)

EO

Etty Osher, MD, PHD

Physician
Tel Aviv Sourasky Medical Center, Tel Aviv, Israel
Rishon, Israel

Disclosure(s):

Tal Netser, MD: No financial relationships to disclose

Background:
Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy, most commonly associated with cortisol and/or androgen excess. Isolated mineralocorticoid hypersecretion in ACC is rare and typically attributed to aldosterone secretion or, more frequently, to excess production of mineralocorticoid active precursors such as 11-deoxycorticosterone (DOC) due to steroidogenic enzyme dysregulation. This entity may be overlooked, as aldosterone levels are not markedly elevated.

Case Presentation:
A 61 year old woman with a history of vitiligo and breast cancer presented with severe rhabdomyolysis, profound hypokalemia, and refractory hypertension. Evaluation revealed renal potassium wasting, suppressed renin, and low-normal aldosterone levels. Morning cortisol was low, with an abnormal cosyntropin 250 µg stimulation test, consistent with partial adrenal insufficiency. Androgens and estradiol were low, 17-hydroxyprogesterone was elevated, suggesting disordered steroidogenesis. Imaging demonstrated a 6.0 × 7.4 cm right adrenal mass with retroperitoneal lymphadenopathy and hepatic lesions. The patient required high dose mineralocorticoid receptor antagonists and aggressive potassium supplementation.
She underwent right adrenalectomy. Pathology reveled high grade ACC with lymphovascular invasion, necrosis, Ki 67 index 25%, Weiss score 7, positive margins (R1), ENSAT stage II disease. Early post operative recurrence of hypertension and hypokalemia occurred, imaging demonstrated unresectable retroperitoneal and hepatic mass. Systemic chemotherapy and immunotherapy were initiated.
Literature Review:
To date 8 cases of DOC secreting ACC have been reported (1974–2019). Patients typically presented with severe hypertension and hypokalemia mimicking primary aldosteronism, despite suppressed renin and normal or low aldosterone. Clinical outcomes were heterogeneous. Notably, 2 of 8 cases documented suppressed cortisol production, supporting disrupted steroidogenesis with diversion towards DOC synthesis.

Conclusion:
This case highlights a rare ACC phenotype characterized by severe mineralocorticoid excess and concomitant impaired cortisol synthesis, likely mediated by DOC rather than aldosterone. Recognition of DOC driven mineralocorticoid hypertension is essential, as reliance on aldosterone levels alone may be misleading, and life threatening adrenal insufficiency may coexist, with important diagnostic and therapeutic implications.

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