This is a guy looking sort of downcast and the caption reads, “Unfortunately there’s no cure. There’s not even a race for the cure.” Adrenal cancer is kind of like that. There is no cure unfortunately and it’s a rare enough cancer so it hasn’t gotten a lot of publicity. I’m not sure if there’s even a web site for adrenal cancer or not. Peak incidences is middle age, in the 40’s and 50’s, and there are two basic kinds of clinical presentations. Folks with nonfunctioning tumors; the tumors are generally going to be quite large at the time of presentation and the presentation will relate to clinical manifestations attributable to an abdominal mass.
These tumors tend to spread by direct invasion of surrounding structures as well as hematogenously. Alternately the tumors could present as a functioning syndrome. And they are about split, non-functioning tumors in most series compromise about 40-50% of the series and functioning tumors the remaining 50-60%. Among the functioning syndromes one can see Cushing’s syndrome with all the stigmata of hypercortisolism, virilization – either alone or virilization together with Cushing’s syndrome – comprise another large group of patients. Considerably less common might be patients who present with symptoms of mineral corticoid excess alone, hypertension, alkalosis, that sort of thing. Also quite common is the occasional man who presents with feminization as a result of an estrogen-secreting adrenal carcinoma.
This is a scan of a patient we saw several years ago, who presented with florid Cushing’s syndrome, had this very large mass that appeared to be arising from the adrenal gland. It was infiltrating into the vena cava, there were hepatic mets and very very extensive disease at the time of presentation. Unfortunately this patient did not do well and never really recovered from her surgery.
Indeed, surgery is the major therapeutic modality for this. The overall outlook in this disease is not good, but probably the single most important factor in terms of outcome is the resectability of the tumor. And this is just a chart of tumors that were completely resected versus those that were not. Among the chemotherapies for adrenal cancer, the agent that is used most commonly is mitotane, which has been shown to have some activity in these tumors. It’s a difficult drug to take. A couple of things to keep in mind if patients are on mitotane, since it is going to block adrenal hormone production, the patient should be on hydrocortisone to prevent adrenal insufficiency. There are some studies to suggest that monitoring serum levels is helpful and that there is sort of a fairly narrow therapeutic range. That folks who have levels less than 14 mg/L tend to have less effective tumor response, whereas side effects tend to become increasingly a problem for those with levels above 20 mg. So there is a role for monitoring, if you can do so.
Side effects for mitotane include weakness, somnolence, confusion, lethargy, headache, anorexia, nausea, diarrhea. Neurologic side effects are also seen, including ataxia and dysarthria. As I said, in general … you know, for us endocrinologists, when we have to deal with this, this is not an easy drug for patients. Frequently it will be necessary in these patients, in addition to attempting to do what one can to control the tumor, to in addition attempt some type of medical therapy to ameliorate their hypercortisolism, or perhaps their hyperandrogenism. And among the agents that can be used are ketoconazole, aminoglutethimide, metyrapone, and the RU486 steroid receptor blocking agent can also be used. But again, in general, I would say this is a disease in which the major treatment is surgical and which can be a very very difficult disease to deal with.