Endocrine Abstracts

A 38-year-old lady, who was 9 months post-partum, presented in 2008 with hirsutism, acne and abdominal discomfort. She was virilised and had an easily palpable right upper quadrant mass. Biochemistry revealed mild hypokalaemia (K 3.3 mmol/l), low albumin and gross elevation of serum androgens (androstenedione 93.9 nmol/l, DHAS 37.7 μmol/l and testosterone 13.7 nmol/l). UFC was 380 nmol/24 h but following 1 mg dexamethasone, cortisol suppressed fully. Urine steroid profile demonstrated increased excretion of androgen and progestogen metabolites and grossly elevated THS excretion. Renin and aldosterone were normal. On CT scanning a large right adrenal tumour was seen with liver metastases encompassing the IVC. Initial treatment comprised mitotane and operative de-bulking. Histology confirmed adrenocortical carcinoma (ACC), so she underwent four cycles of chemotherapy with mitotane + doxorubicin/ cisplatin/ etoposide, followed by radical radiotherapy to the adrenal bed (50 Gy in 25 fractions over 5 weeks). She was maintained on mitotane but in 2010 relapsed with liver metastases which were not amenable to further surgery. Radiofrequency ablation (RFA) of the liver metastases was then performed. In 2011 she developed lung and progressive liver metastases. Further RFA to liver and lung was administered, followed by repeated chemotherapy (with the same regimen), but disease progressed further. Sunitinib was initiated and the dose increased, which resulted in a partial response, although an area of apparent selective resistance in part of the liver was treated by nanoknife ablation. Recent CT suggests some disease progression, although androgens have normalised over the years (testosterone <0.5 nmol/l), a’dione 4.7 nmol/l, DHAS 2.9 μmol/l). There are little published data on RFA in metastatic ACC, but two small studies (both n=8) have demonstrated safety and short-term efficacy in terms of tumour shrinkage (Wood et al.), and median survival of 1.9 years post RFA (Ripely et al.) respectively. Pre-clinical studies exploring the use of sunitinib in ACC are promising (Lin et al., Kroiss et al.) and there are a few published cases and a small clinical trial of use of sunitinib in metastatic ACC reporting variable outcomes (Lee et al., Kroiss et al.). This is a highly unusual case of prolonged survival in aggressive ACC with the use of multiple therapeutic modalities, many of which have a paucity of clinical evidence.