Posted by Thomas Repas, DO, FACP, FACE, CDE July 24, 2009 03:56 PM
Previously, I posted about a young woman who presented with rapid onset Cushing’s syndrome and a 9.0-cm adrenal mass. Her initial 24-hour urine-free cortisol was 1,095 mcg/24 hours (upper limits of normal, 45), one of the highest I have ever seen. She subsequently had surgical resection which confirmed moderately differentiated adrenocortical carcinoma as we had suspected. Fortunately, there was no evidence of extension beyond the adrenal gland or metastatic lymph nodes. Based on size, lack of extension outside the adrenal gland and no known metastasis, she is T2N0MX, stage 2 disease.
She is doing amazingly well. She has lost over 20 lbs, looks better and feels better, and her appetite has returned. We placed her on hydrocortisone for glucocorticoid replacement but are weaning her off as the previously suppressed contralateral adrenal recovers.
The question we have: Is there any persistent disease?
Adrenocortical cancer can be aggressive. Unfortunately, most patients present in stage III or IV. The prognosis for stage IV disease is dismal, with a five-year survival of only 15% to 25%. The only hope for cure is being fortunate enough to completely resect stage I or stage II disease.
The next step will be imaging by PET with F-18-fluorodeoxyglucose. Meta-analysis suggests that FDG-PET imaging is reliable in staging and follow-up of disease, with a sensitivity of 96% and specificity of 99%.
If persistent disease is detected, unfortunately, her prognosis will be poor. Multiple chemotherapeutic regimens have been tried, with limited results. One study reported that combination of etoposide, doxorubicin and cisplatin with mitotane (Lysodren, Bristol-Myers Squibb) had a response rate of as high as 54%. Most other studies have had much lower success rates.
She will be seeing our colleagues in oncology. I hope that we have cured this very pleasant young woman with our initial surgery. We will follow her very closely. If there is evidence of persistent disease, I will also advise her to be seen at a tertiary referral center for additional opinion and the possible option of participation in a clinical trial.
