MON-521 - Hernia, Hormones and A Hidden Malignancy: A Case of Androgen-Secreting Adrenocortical Carcinoma

Background:
Pure androgen-secreting adrenocortical carcinomas are a rare subset of functional adrenal malignancies, accounting for approximately 2–6 % of all adrenocortical carcinomas. We report an unusual case of a large androgen-secreting adrenocortical carcinoma presenting as an incarcerated umbilical hernia.

Case Description:
A 45-year-old female presented in April 2025 with progressive abdominal pain and an incarcerated umbilical hernia. Physical examination revealed moderate facial hirsutism without cushingoid features. Imaging demonstrated a 15.6 cm heterogeneous left upper quadrant mass inseparable from the left kidney with non-visualization of the adrenal gland. Hormonal evaluation revealed markedly elevated androgens including serum dehydroepiandrosterone sulfate level more than 5000 μg/dL, testosterone level of 238 ng/dL, and androstenedione level of 801 ng/dL. Cortisol production was normal. Following urgent hernia repair, she underwent open left adrenalectomy with en bloc distal pancreatectomy and splenectomy. Pathology confirmed a 16 cm adrenocortical carcinoma with lympho-vascular invasion, margins negative for tumor, and nine lymph nodes negative for metastasis. Immunohistochemistry showed focal Ki-67 proliferation index up to 30% and focal weak p53 expression. Post-operatively, androgen levels normalized and adjuvant mitotane therapy was initiated in July 2025 with appropriate steroid coverage. In January 2026, she reported new-onset abdominal pain. Repeat CT imaging revealed metastatic disease involving the right hepatic lobe, anterior pararenal, and left perinephric spaces, not present on initial imaging.


Discussion:
This case highlights classic features of pure androgen-secreting adrenocortical carcinoma, including virilization without hypercortisolism, markedly elevated dehydroepiandrosterone sulfate levels, and large tumor size. High Ki-67 proliferation index and stage III disease classify this tumor as high-risk for recurrence. Complete surgical resection remains the only curative treatment, although locally advanced disease often requires multi-visceral resection. Despite ongoing adjuvant mitotane therapy, early metastatic recurrence occurred.

Conclusion:
Pure androgen-secreting adrenocortical carcinoma is a rare and aggressive malignancy requiring high clinical suspicion in women with rapid-onset virilization and severe hyperandrogenism. Complete surgical resection remains the cornerstone of therapy, while high Ki-67 proliferation index predicts increased recurrence risk even after complete resection. This case underscores the aggressive nature of pure androgen-secreting adrenocortical carcinoma and reinforces the need for early recognition, multidisciplinary management and close post-operative surveillance.

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