Metastatic adrenocortical carcinoma

As I had mentioned in previous posts, earlier this summer I had two patients who were diagnosed in the same month with adrenocortical carcinoma. Both are young women who presented with Cushing’s disease, adrenal androgen production and a large adrenal mass. They underwent surgical resection and have felt much better since.

One had a follow up fluorodeoxyglucose PET/CT, which revealed no evidence of disease. We do not know if time will prove her cancer-free but this news was a relief for all of us.

The other, however, has had recurrence of symptoms. Her baseline Dehydroepiandrosterone sulfate before surgery was >500 mcg/dL, immediately after surgery it was 130 mcg/dL and over the last month it has increased to 787 mcg/dL (normal: 31 mcg/dL to 228 mcg/dL). Unfortunately, FDG-PET/CT confirmed what we dreaded to find: multiple new areas of metastatic disease throughout her abdomen and pelvis. We have sent her to a multidisciplinary adrenal cancer clinic at a tertiary referral center. Our hope is for her to be treated with an investigational regimen as part of a clinical research study.

The prognosis for metastatic adrenal cancer is dismal with many patients not surviving even one year. Because adrenal cancer is a rare disorder, it has been somewhat of an orphan disease in that not much new research has been done, simply because very few centers have a sufficient number of patients to perform such research.

Mitotane has been used alone and in combination with other chemotherapeutic agents to manage symptoms of hypercortisolism and delay the growth of metastatic disease. However, it is not curative. The usual dose of mitotane is 2 g to 4 g per day. Patients are initiated on 500 mg daily which is then increased as tolerated by 500 mg per week. Side effects include nausea, vomiting, rash, diarrhea, lethargy, sedation, dizziness, ataxia and others.

However, a case report of sustained remission with the kinase inhibitor sorafenib in stage 4 metastatic adrenocortical carcinoma was recently published online in Endocrine Practice. The patient was initiated on sorafenib and had resolution of lung metastasis, reduction in hepatic lesions, normalization of biochemical abnormalities and clinical improvement sustained for more than 28 months. Perhaps emerging treatments such as this will offer hope for our patients with adrenal cancer?

As I write this post, snow is gently falling on the pines outside my window. It would be easy for me to not think about my patients when I am at home and not at the clinic. When we speak about disease states we all too frequently lose ourselves behind objective talk about statistics, survival and response rates. It is one way that physicians cope with having to give bad news or treat incurable diseases. However, we must never forget that it is human beings we are treating - not diagnoses or disease states.

I wonder about my patient and her family. I hope she will receive encouraging news. She and her family are not only patients, they are neighbors who live in the same small western South Dakota town my family does. They know many of the same people we know. I cannot imagine what it must be like to be in their situation. What will her future hold? No one knows the answer to that question.

Of course, no one really knows what lies ahead in any of our futures. Any one of us could be faced with horrible news. Any day could be our last. As we are amidst the holidays, we must not forget to be grateful for the true blessings of life: our health, our happiness, and that of our family and friends. I wish you all the very best for the holidays and the New Year.