Endocrine Abstracts (2017) 50 P032 | DOI: 10.1530/endoabs.50.P032

Large functional adrenocortical carcinoma presenting with hyperandrogenism and hypercortisolism

Haider Khan & Ioannis Dimitropoulos


Derriford Hospital, Plymouth, UK.


Introduction: Adrenocortical carcinomas (ACCs) are rare; incidence is approximately 1–2 per million population per year. These arefrequently aggressive tumours that may or may not be functional. The primary potentially curative treatment for ACC is surgery with currently mixed opinion among experts regardingadjuvant Mitotane treatment.

The Case: 60 years old female was seen in clinic for work up of incidental adrenal mass. CT abdomen showed a very large adrenal tumour measuring 21×16×14cmswithout evidence of metastases.

She had 5–6 month history of rapid onset facial hirsutism and frontal balding as well as clinical features of Cushings. She was alsonormokalaemic and hypertensive despite good concordance with three anti-hypertensives. Her 2×24 hour urine collections for Catecholamines and free Cortisol were normal. Her serum androgen levels weremarkedly elevated.DST failed to suppress Cortisol (564 nmol/L) with ACTH of 12 ng/L.

CT and PET scans didn’t demonstrate distant metastases. She underwent right open adrenalectomy with steroid cover. Histopathology confirmed adrenocortical carcinoma with a higher proliferation rate of approximately 10–20% on MIB-1 immunostainingbut no capsular invasion.

Post-surgery her symptoms of hyperandrogenaemia, hypercortisolism and hypertension improved significantly. She was started on Adjuvant Mitotane treatment by oncologist. Repeat CT scan and PET scan didn’t show recurrence.

Discussion: In this case, the adrenal tumour size was unusually large and interstingly without capsular invasion or metastases. The role of post-op Mitotane therapy is debatable. Our patient has responded well to surgery and Mitotane treatment. Evidence for adjuvant Mitotane interms of extending recurrence-free survival is based on a few retrospective studies. However, due to the frequentlyaggressive nature of adrenocortical tumours, it is generally recommended, as well as avoiding delays in the initiation of such treatment.

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