Category: Adrenal Disorders
Adrenocortical carcinoma (ACC) is a rare disease with limited therapeutic options. Stage IV disease is characterized by distant metastasis and has an unfavorable prognosis (5-year survival 0-28%). A proposed modification to European Network for the Study of Adrenal Tumours (ENSAT) staging incorporates histologic grade (defined primarily by mitotic rate) to more accurately predict outcomes.
We present the case of a 45 year old man diagnosed with ENSAT stage IV adrenocortical carcinoma with unresectable secretory pulmonary metastases.
We present the clinical, biochemical, and imaging findings of our patient and review the relevant literature for the management and prognosis of stage IV adrenocortical carcinoma.
En-bloc resection of the 10 kg adrenal mass was carried out. Postoperative histology was consistent with malignant disease, however, mitotic index was 11-12/50 HPF. Postoperatively, the patient received mitotane therapy for a period of 26 months. 12 months after surgery, patient’s disease progressed in the form of new hepatic metastases. The patient underwent 8 cycles of chemotherapy with etoposide and cisplatin while continuing mitotane at a reduced dose. After initially responding to chemotherapy, hepatic metastases progressed after the 7th cycle. The patient was lost to follow-up and discontinued all medications for a period of 33 months after which he presented with progression of the pulmonary metastatic nodules leading to hemoptysis. There was however, no evidence of disease recurrence at the primary site. The patient elected to stay off medication and continues to work and lead an active life as of this writing.
Adult adrenocortical carcinoma are aggressive tumors with a very poor prognosis. The prognosis depends on the stage. Unfortunately, 60 to 70% of patients have stage III or IV disease at the time of diagnosis. After a recurrence of adrenal cancer, virtually all patients die within five years.
This unexpectedly favorable prognosis, spanning 6 years, in a patient with ENSAT stage IV ACC with mitotic index less than 20/50HPF adds to the case for including histological grade in prognostication and therapeutic decision making of adrenocortical carcinoma.
The Aga Khan University
Dr. Aisha Sheikh is FCPS (Medicine), she did her Fellowship training in Diabetes, Endocrinology and Metabolism from The Aga Khan University Hospital, Karachi, Pakistan and later did her Post-graduate diplomas in Diabetes and Endocrinology from UK. She is Consultant Endocrinologist and is affiliated as faculty member with The Aga Khan University, Medicell Institute of Diabetes, Endocrinology & Metabolism and University of South Wales, UK. She is actively involved in medical education and research and has published several research papers and a book chapter on "Hyperthyroidism and pregnancy" and "Diabetes and Ramadan" . She holds the position of Vice President Sindh of Pakistan Endocrine Society. She has keen interest in antenatal Diabetes & Endocrinology and has played a key role in development of Gestational Diabetes Mellitus recommendations and action plan from the platform of South Asian Federation of Endocrine Societies (SAEFS).
Department of Family Medicine, Aga Khan University Hospital
Karachi, Sindh, Pakistan
Dr Imran Hassan did his MBBS from Services Institute of Medical Sciences in Lahore, Pakistan. He is currently a resident in the department of Family Medicine at the Aga Khan University Hospital in Karachi, Pakistan. He has a keen interest in endocrinology and plans to incorporate it in his practice as a special interest after graduation.