Endocrine Abstracts

Introduction: Adrenocortical carcinoma (ACC) is a rare but devastating malignancy. We performed a review to determine outcome in our patient cohort.

Of 20 patients 14 were females, six males. Median overall survival was 27.5 months (range 1–168), nine being deceased. Mean age at presentation 52.3 years (range 18–71). The majority of cancers were large; 5% ≤5 cm, 40% 5–10 cm, 55 >10 cm. 25% had stage IV disease, 10% stage III, 55% stage II, 10% stage 1. 75% of 16 with histology had Weiss score >2. The functionality of tumours was determined using blood tests and urinary steroid profiles. 70% were functioning. 67% tumours in males were secretory (glucocortiocoids only). 93% tumours in females secreted a range of hormones; glucocorticoids 41%, androgens 41%, mineralocorticoids 11%, other precursors 11%.

Of 16 adrenalectomies, five underwent laparoscopic adrenalectomy, eight laparotomy, three procedures unknown. Surgical procedure did not affect survival.

Fifteen patients were treated with mitotane; seven as adjuvant therapy after surgery, six combined with chemotherapy, two as monotherapy for recurrence when unfit for chemotherapy. Mean duration of Mitotane was 18.3 months (range 1–70). Therapeutic level (14–20 μg/dl) was achieved in only five due to side effects.

One patient underwent post-operative radiotherapy to the adrenal bed. Seven patients had palliative chemotherapy with EDP (Etoposide, Doxorubicin, Cisplatin) and Mitone 1st line. Two required 2nd line Streptozocin regimes. Median overall survival was 18 months (range 2–30). Eleven of all patients suffered adverse effects to systemic therapies.

Of 11 patients under surveillance 6 are in remission, 3 having completed >2 years mitotane at therapeutic levels. Five patients are undergoing treatment for recurrence or disease progression, three undergoing palliative chemotherapy.

Conclusions: In our small cohort, ACC has a poor prognosis. Further multicentre studies are needed to answer outstanding questions regarding adjuvant mitotane therapy. New treatments are needed to improve the dismal prognosis.