ACC is a rare tumor with poor prognosis. Its low incidence hinders the development of a reliable database on clinical manifestations, prognosis, and the impact of different treatment options. The purpose of the German ACC Registry is to evaluate these aspects in a large retrospective series and to establish a prospective database to improve patient care and recruitment for future clinical trials.
In June 2003, a structured evaluation form collecting comprehensive information on diagnostic procedures, therapy, and follow-up was developed. All GANIMED (German Adrenal Network: Improving Medical research and Education) centers and the Study Group for Malignant Endocrine Tumors in Childhood were contacted to include patients (diagnosed after 1982) in this registry. A website (http:www.nebennierenkarzinom.de) was created containing all the information required for patient enrolment.
Ninety-five patients (females 63%; males 37%) were included in the registry until January 2004. The mean age at diagnosis was 39 years (range 0.3–83 years). Hormone excess was detectable in 55% of the patients. Three patients were diagnosed as stage I (< 5 cm), 32 as stage II (> 5 cm), 33 as stage III (advanced locoregional disease), and 27 as stage IV (metastasized disease) at the time of primary diagnosis. Mean tumor size at diagnosis was 11 ± 4 cm (3–21cm). Surgery was performed in all patients. Metastases occurred mainly in lung (47%), liver (30%), and bone (13%). The median survival of all patients was 33 (1–274) months. As expected, the survival differed significantly according to the stage.
Enrolment into the retrospective registry will be open until December 2004. This registry will allow a detailed analysis of prognostic factors and the present outcome in patients with ACC in Germany. The prospective registry will only include newly diagnosed patients with ACC. Its aims will include improvement in patient care and participation in international therapeutic trials such as the proposed First International Randomized trial in Metastastic Adrenocortical Cancer Treatment (FIRM-ACT).
