Endocrine Abstracts

Adrenocortical carcinoma (ACC) is a rare malignancy with an incidence of 0.7–2.0 cases/million habitants/year, often with a dismal prognosis. Patients present with abdominal symptoms or symptoms of hormone excess. However, 15% are diagnosed incidentally. (1) A 59y male was referred with progressive lethargy, weight loss, dyspepsia and abdominal distension over the past 2 years. He was dismissive of ‘the elephant in the room’ and in denial. He was pushed to seek medical attention by his family when abdominal mass and cachexia could no longer be ignored. His weight was 63 kg, BMI 20.2 kg/m². He looked emaciated with no Cushing’s stigmata.There was a large firm mass on the entire left half of his abdomen extending midline. CT chest/abdomen/pelvis showed 23 cm mass arising from the left adrenal gland, heterogenous with punctate calcification and central necrosis. Mass was hormonally non-functional apart from incomplete cortisol suppression on 1 mg overnight dexamethasone suppression test – 86 nmol/l (<50).

Results: Normetanephrine 1.67 μmol/24 h (0–3), Urine metanephrine 0.49 μmol/24 h (0–1.40), Urine 3-methoxytyramine 0.75 μmol/24 h (0.57–2.39), DHEA 12.9 μmol/l (1.3–9.8), testosterone 4.8 nmol/l, androstenedione 12.2 nmol/l (2.8–10.5), SHBG 30.9 nmol/l (15–48), FAI 13.9 (34–106), 17OHP 2.2 nmol/l, aldosterone renin ratio 30 pmol/mIU.

FDG PET CT showed a large avid necrotic left upper abdominal mass with SUV max 7 inseparable from the left adrenal gland. He underwent an open adrenalectomy. 6 kg organised mass with central necrosis was fully excised measuring 37×21×21 cm. Post-operative period was complicated by hyponatraemia despite Hydrocortisone cover. Histology showed positive staining for calretinin, inhibin and melan-A. Staining for PAX8, S100, chromogranin was negative. The appearances were consistent with ACC, Weiss Score 5 and ki-67- 5.9%, T1, N0, M0, R0. Post-operatively he was very well, off steroids and back at work part-time. He was offered adjuvant mitotane but decided not to receive it. His circumstances were borderline-he did not meet ESMO guidelines for mitotane, although the ESE guidelines would suggest, that it could be considered ‘on a case-by-case basis’.

Conclusion: To our knowledge, this is the largest and the second heaviest ACC ever reported. This case highlights a very wide heterogenicity in ACC behaviour from a very aggressive to indolent disease. A combination of patient psychological factors leading to delayed presentation and indolent behaviour of this ACC allowed it to reach colossal proportions, something we are unlikely to see again.

References: 1 Rossella Libé Adrenocortical carcinoma (ACC): diagnosis, prognosis, and treatment Front Cell Dev Biol. 2015; 3: 45.