Endocrine Abstracts

Introduction: Adrenal tumors are common tumors with a prevalence of around 3% in a population over the age of 50 years. In contrast, adrenocortical carcinoma (ACC) is a rare malignancy (incidence 1–2 per 1 million population) with a heterogeneous presentation and a variable but generally bad prognosis. Patients present with evidence of adrenal steroid hormone excess in approximately 60% of cases.

Case report: A 49-year-old woman with symptoms of facial swelling, weight gain; predominantly abdominal, appearance of hair on face and neck, high blood pressure, bruising and insomnia during the last four months. In addition, polymenorrhoea in the last two menstrual cycles. No medical history of interest except the start of antihypertensive treatment in the last year. The patient exhibited clinical features associated with excessive steroid hormone or catecholamine levels. Thus, blood tests with an adrenal hormone profile were requested:

These clinical symptoms and analytical alterations led to the discovery of a 8x8x11 cm heterogeneous hypoechoic left adrenal mass on an abdominal magnetic resonance. After surgery adrenal cortical carcinoma with areas of necorsis and hemorrhage is confirmed. It exceeded adrenal gland and reached peripancreatic tissues. It presented perineural invasion but no vascular invasion. Negative immunostaining for steroidogenic factor 1, melanA markers and chromogranin A was confirmed. The surgery was completed with right suprarenalectomy, tail and body pancreatic resection and nodulectomy of segment three of the liver (it confirmed intraoperative metastasis). The treatment regime usually depends on cancer stage. Two major staging systems are used: the American Joint Committee on Cancer (AJCC) TNM staging system and the ENSAT staging system(European Network for the Study of Adrenal Tumors). The ENSAT staging system is essentially the same as the AJCC system, but reserves stage IV only for tumors with distant metastasis. In our patient, we are in stage IV, pTN3NxM1 and following the criteria of ENSAT he had started mitotane-EDP (doxirubicin-cisplatin-etoposide). Moreover, she is taking hidroaltesona 20-10-10 mg daily because there is an increase in cortisol metabolism secondary to mitotane.

Conclusion: In advanced ACC, mitotane is still the standard of care. However, most patients will experience progress and will require rescue therapies due to the delayed diagnosis and aggressiveness of the ACC. Thus, new treatment concepts are urgently needed.