Poster Presentation The Joint Annual Scientific Meetings of the Endocrine Society of Australia and the Society for Reproductive Biology 2017

Two cases of adrenocortical carcinoma (#256)

Alice Y Hong 1 , Anneke Graf 1 , Melissa Lee 1 , Dilshani Jayawardene 1 , David A Pattison 2 , Richard J MacIsaac 1 , Nirupa Sachithanandan 1
  1. Department of Endocrinology & Diabetes, St Vincent's Hospital Melbourne, Fitzroy, VIC, Australia
  2. Department of Nuclear Medicine & Specialised PET Services, Royal Brisbane and Women’s Hospital, Herston, Queensland, Australia

Adrenocortical carcinomas are rare but patients often present with advanced disease and display symptoms of hormone hypersecretion or tumour burden/mass effect. Here we present two cases of adrenocortical carcinoma to highlight the challenges of managing this condition.


Case 1: A 48 year old female initially presented with an incidental adrenal mass measuring 42mm. On triple-phase CT the mass was reported as an adrenal myelolipoma and no further followup was arranged. She represented 3 years later with abdominal bloating, facial plethora, hirsutism and weight gain. Investigations revealed hypercortisolism and hyperandrogenism in the setting of a 16cm adrenal mass with retroperitoneal lymphadenopathy but no distant metastases. She underwent an open right adrenalectomy and histology was consistent with a 17cm adrenocortical carcinoma with a high Ki-67 index of 40% and positive lymph nodes. Post-operative workup revealed residual local disease as well as pulmonary metastases. She then received adjuvant therapy with etoposide/doxorubicin/cisplatin and mitotane. Progressive disease was further treated with radionucleotide therapy (I131-metomidate), immunotherapy (PD-1 antibody BGB-A317) and sunitinib. Despite multiple lines of treatment, disease control was never achieved and the patient died 2 years following her initial surgery.


Case 2: A 35 year old female presented with weight gain, amenorrhoea, hirsutism and abdominal striae. Workup revealed hyperandrogenism and hypercortisolism with a large right adrenal mass. A 94mm adrenocortical carcinoma with a Ki-67 index of 30% was resected. She underwent adjuvant therapy with mitotane however follow-up imaging revealed new pulmonary and hepatic metastases. She received first-line chemotherapy with etoposide/doxorubicin/cisplatin as well as mitotane and metyrapone to control florid Cushing’s syndrome. She progressed to second-line chemotherapy with capecitabine/gemcitabine however died soon after.


An actionable mutation suitable for targeted therapy was not identified on next-generation sequencing in either case. These cases emphasise the need for improved treatments for metastatic disease.