Endocrine Abstracts

Adrenocortical cancer is a rare, aggressive disease, requiring a fast and multidisciplinary approach. The genetics is heterogeneous so is also the clinical course. In the last 10 years, we have diagnosed and treated a number of 20 cases, aged at diagnosis between (34–74 years) (mean 55.9±12.87), followed up between (2–77 months) (mean 25.75±23.58). The maximal diameter of the tumor was (5.5–25) (mean 10.95±5.43). The secretion profile was Cushing syndrome in 11 cases, androgen secreting tumor 1 cases, mineralocorticoid secreting tumor in 2 cases, while 6 cases were nonfunctional. Hypokaliemia was encountered in 2 cases. At diagnosis, the majority of cases (15) had local invasion, and 8 of which needed also nephrectomy and or splenectomy. All were submitted to open surgery. The Weiss score distribution of cases (3–8) was available in 13 patients. After the first surgery, 17 cases were submitted to oncologic approach using mitotane, which was gradually increased to target recommended dosage (14–20 mg/dl). Additional chemoterapeutic agents were used in 5 cases. The follow-up was done at 3 months with hormonal assays as well as oncologic imaging (CT scan). A number of 15 cases developed primary adrenocortical failure and requested oral substitution with hydrocortisone, on average with 35 mg/day in 2 or 3 administrations with doses higher than those requested for other causes of Addison disease. Relapse cases were submitted again to surgery in 7 patients. Mean progression-free survival was 3–6 months and mean mortality was at 25 months from diagnosis. Prognosis was related to Weiss score, but also to compliance to treatment. In conclusion, adrenocortical cancer is a heterogeneous disease, with high mortality and poor prognosis, requiring multidisciplinary approach.