Aim: ACC is an extremely rare heterogeneous aggressive disease. Clinical presentation are various. The prognosis is very poor. There are limited data to define prognostic markers for survival beyond stage.
The aim of this study is to describe the experience of our institution with patients affected by ACC.
Material and methods: This study was performed on 13 patients aged between 26 and 76 years, from 1992 to 2009 who turned to our Institution for an adrenal carcinoma.
Results: Only 15% of patients was asymptomatic at the time of onset. The 85% reported symptoms attributable to cortisol or androgen hypersecretion, to the tumour mass, gastrointestinal and more aspecific symptoms. The CT showed that in 15.4% the tumour was smaller than 5 cm, in 46.2% it was between 5 and 10 cm, in 23% the size ranged from 10 to 15 cm and the 15.4% of the masses had a diameter greater than 15 cm. The 38.5% of these masses were not secreting, the 23% secreted cortisol, the 15.4% secreted androgens and the 23.1% had a mixed androgen-cortisol secretion. No mass secreted mineralocorticoids or estrogens. Eleven patients underwent surgery: laparotomy in 8 cases and laparoscopy in 3 cases. It was possible to perform a radical resection in 72.7% of cases. The 46.2% of patient underwent adjuvant therapy with mitotane. In 4 patients mitotane was administered with different patterns of systemic cytotoxic chemotherapy. In our series, 69.2% of patients are deceased. The median survival is 28.9±11.1 months. The time to relapse is 14±16.9 months.
Conclusion: Our data agree with literature, describing a highly aggressive disease with a poor prognosis. Even in our series, the most important prognostic factor is the radical surgery that significantly influence the outcome of the disease.
Prague, Czech Republic
24 - 28 Apr 2010
European Society of Endocrinology