Primary adrenocortical carcinoma: A retrospective review of The Cleveland Clinic Foundation experience between 1995 to 2002
18614 Background: Primary adrenocortical carcinoma (PAC) is a rare disease with a incidence of 1 per million. PAC’s are highly malignant tumors with guarded prognosis. Radical surgery is the treatment of choice. Methods: The pathology database and medical charts of these patients were retrospectively reviewed. Results: There were 14 females, 8 males, and 95% were caucasion. The mean age was 49.2 ± 11.8 years (females 51.6 years, males 45 years). The mean size of the tumor was 12.6 ± 5.1 cm (range of 3.6 to 25 cm). Thirteen patients (59.1%) presented with left and nine (40.9%) with right sided PAC. Seventy- two percent of the patients had abdominal, back, and/or flank pain. Forty five percent of patients had symptoms related to hypercortisolism and/or hyperandrogenism. Twelve patients (55%) had functional tumors (hypercortisolism 67%, hyperandrogenism 42%, and DHEA-S hypersecretion 42%) and ten were non-functional. Five percent of the tumors were classified as stage I, 33% were stage II, 38% were stage III, and 24% were stage IV. Twelve patients (55%) had distant metastases (liver 75%, lung 67%, retroperitoneum 33%, kidney 17%, contra-lateral adrenal gland 17%). All 22 patients had adrenalectomies (open 91%; laparoscopic 9%). The microscopic margin of resection were negative in 14 and positive in 6 patients. The information for 18 patients were evaluable for treatment. Eight patients received post-op therapy. Four patients received Mitotane (o,p’-DDD) alone (three died at 2, 29, and 213 months; and one is alive at 38 months after diagnosis). Three patients received both Mitotane and chemotherapy (two died at 3 and 43 months, one is alive at 140 months). One patient received only chemotherapy and died at 8 months. Ten patients received no post-op therapy. The estimated 5-year survival for the entire group was 48% (Kaplan-Meier survival estimate, 95% confidence interval). The estimated 5-year survival for stage III was 33%, and for stage IV was 30%. There were no deaths in patients with stage II group. The estimated 5-year survival for the treated group (8 patients) was 33% and 58% for the untreated group (10 patients). Conclusions: In PACs, surgery remains the main method of therapy. In our experience, Mitotane with or without chemotherapy did not improve survival. No significant financial relationships to disclose.