Virilizing Adrenocortical Carcinoma with Liver Metastases: A Case Report

Authors

DOI:

https://doi.org/10.34635/rpc.1184

Keywords:

Adrenocortical Carcinoma, Liver Neoplasms/secondary, Mitotane, Neoplasm Metastasis, Virilism

Abstract

Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy with an incidence of approximately 1-2 cases per million population per year. Clinical presentation varies widely and may result from hormone hypersecretion or mass effect. Virilization in women is highly suggestive of ACC. Prognosis is strongly related to tumor stage, as metastatic disease is associated with poor outcomes. We report the case of a 41-year-old woman who presented with a 10-month history of abdominal and lumbar pain, amenorrhea, hirsutism, and progressive virilization. Physical examination revealed male-pattern hair distribution and a palpable abdominal mass. Laboratory tests showed markedly elevated androgen levels, including total testosterone and dehydroepiandrosterone sulfate. Computed tomography revealed a large right adrenal mass measuring 10×16 cm with compression of adjacent structures. Positron emission tomography demonstrated intense metabolic activity and suggested hepatic invasion. After multidisciplinary discussion, the patient underwent en bloc resection including right adrenalectomy, right hepatectomy, and cholecystectomy. Histopathological examination confirmed a high-grade adrenocortical carcinoma with a Ki-67 proliferation index of approximately 40% and hepatic metastases (ENSAT stage IV). Early postoperative imaging revealed further hepatic disease progression. The patient subsequently received systemic therapy with mitotane and chemotherapy but developed treatment-related toxicity and progressive metastatic disease. She was referred to palliative care and died six months after surgery.

ACC remains a rare but highly aggressive malignancy. Although complete surgical resection is the cornerstone of treatment, patients with stage IV disease have a poor prognosis despite multimodal therapy. This case highlights the aggressive natural history of metastatic ACC and the challenges associated with its management.

Downloads

Download data is not yet available.

References

Fassnacht M, Puglisi S, Kimpel O, Terzolo M. Adrenocortical carcinoma: a practical guide for clinicians. Lancet Diabetes Endocrinol. 2025;13:438-52. doi:10.1016/S2213-8587(24)00378-4

Funder JW, Carey RM, Mantero F, Murad MH, Reineke M, Shibata H, et al. The management of primary aldosteronism: case detection, diagnosis, and treatment: an Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2016;101:1889-916. doi:10.1210/jc.2015-4061

Kuhlman JJ, Rao S, Sacchi de Camargo Correia G, Wolf E, Tawfiq R, Li S, et al. Assessment of survival and role of chemotherapy in patients with adrenocortical carcinoma: Insights from SEER Database (2000-2021). J Clin Oncol. 2025;43:16_suppl. doi:10.1200/JCO.2025.43.16_suppl.e16610

Nastos C, Papaconstantinou D, Paspala A, Pararas N, Vryonidou A, Pikouli A, et al. The impact of adrenocortical carcinoma hormone secreting status as a predictor of poor survival: a systematic review and meta-analysis. Langenbecks Arch Surg. 2024;409:316. doi:10.1007/s00423-024-03507-5

Ng L, Libertino JM. Adrenocortical carcinoma: diagnosis, evaluation and treatment. J Urol. 2003;169:5-11. doi:10.1016/S0022-5347(05)64023-2

Fassnacht M, Dekkers OM, Else T, Baudin E, Berruti A, de Krijger RR, et al. 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. Eur J Endocrinol. 2018;179:G1-46. doi:10.1530/EJE-18-0608

Jasim S, Habra MA. Management of Adrenocortical Carcinoma. Curr Oncol Rep. 2019;21:20. doi:10.1007/s11912-019-0773-7

Dy BM, Strajina V, Cayo AK, Richards ML, Farley DR, Grant CS, et al. Surgical Resection of Synchronously Metastatic Adrenocortical Cancer. Ann Surg Oncol. 2015;22:146-51. doi:10.1245/s10434-014-3944-7

Wangberg B, Khorram-Manesh A, Jansson S, Nilsson B, Nilsson O, Jakobsson CE, et al. The long-term survival in adrenocortical carcinoma with active surgical management and use of monitored mitotane. Endocr Relat Cancer. 2010;17:265-72. doi:10.1677/ERC-09-0190

Berruti A, Terzolo M, Sperone P, Pia A, Della Casa S, Gross DJ, et al. Etoposide, doxorubicin and cisplatin plus mitotane in the treatment of advanced adrenocortical carcinoma: a large prospective phase II trial. Endocr Relat Cancer. 2005;12:657-66. doi:10.1677/erc.1.01025

Veltri A, Basile D, Calandri M, Bertaggia C, Volante M, Porgiglia F, et al. Oligometastatic adrenocortical carcinoma: the role of image-guided thermal ablation. Eur Radiol. 2020;30:6958-64. doi:10.1007/s00330-020-07019-w

Miller BS, Gauger PG, Hammer GD, Giordano TJ, Doherty GM. Proposal for modification of the ENSAT staging system for adrenocortical carcinoma using tumor grade. Langenbecks Arch Surg. 2010;395:955-61. doi:10.1007/s00423-010-0698-y

Downloads

Published

2026-10-19

Issue

Section

Clinical Case

Most read articles by the same author(s)