Endocrine Abstracts (2017) 49 EP36 | DOI: 10.1530/endoabs.49.EP36

Transformation from Addison's disease to adrenocortical carcinoma presented as Cushing's syndrome with androgenisation

Antonela Sabati Rajic, Kristina Groti Resman, Tomaž Kocjan & Marija Pfeifer

Department of Endocrinology, University Medical Centre Ljubljana, Ljubljana, Slovenia.

We present a female patient 56 years old, who was treated at the endocrinology department from the year 2006. The diagnosis of Addison’s disease was established and she was properly treated. At the same time primary hypothyroidism was diagnosed, also started treating it properly. She was regularly controlled more often at the first time, than yearly at outpatient clinic. In September 2015 she was in menopause. A few months before she noticed oedematous legs and arms and diffuse bruising. Hypertension was also noticed for the first time and needed therapy. Her voice was different (became deep), she noticed hair loss and hirsutism, as well. She thought it was all in correlation with menopause. At the control examination she had hypokalemia, hypernatremia, hypertension, osteopenia with clear clinical aspect of Cushing. The abdominal ultrasound, CT scan and FDG-PET scan showed huge (13.8 cm in diameter) heterogeneous tumour mass in the right adrenal gland. The tumour infiltrates inferior vena cava and liver. Pelvic bone metastases were diagnosed also. CT angiography showed tumour thrombus and pulmonary embolism. Tumour biopsy showed adrenocortical carcinoma. The hormonal tests showed co-secretion of cortisol and androgens (Cushing’s syndrome and androgenisation). Tumour was inoperable so tumour arterial embolisation was performed with idea of debulking. The patient was treated with mitotane in combination with systemic chemotherapy (EDP regiment: etoposide, doxorubicin), antiandrogen therapy per os and metirapon. Because of the important hepatotoxicity mitotane was discontinued soon. She needed also hydrocortisone supplementation transitory, for the short time. The patient died due to the disease progression after one year.

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