Complete surgical resection and neoadjuvant mitotane therapy led to normalization of estradiol levels and no further recurrence in a male with estrogen-producing ACC after five years.
This case highlights the importance of including estradiol in the evaluation of male hypogonadism and gynecomastia to facilitate early recognition of rare feminizing adrenocortical tumors.
Absolute Event Rate: 0% vs 0%
Abstract Introduction Feminizing adrenocortical tumors FATs are an extremely rare subtype of adrenocortical carcinoma ACC, representing about 1–2% of cases. They typically manifest with gynecomastia, hypogonadism, and weight loss, and carry a poor prognosis compared to other ACC variants. Clinical Case A 49-year-old male with a history of hyperlipidemia and peptic ulcer disease presented with decreased libido and bilateral gynecomastia. Laboratory workup showed suppressed testosterone and gonadotropins, elevated prolactin, and low IGF-1. Pituitary MRI was unremarkable. In 2018, abdominal imaging revealed a large retroperitoneal heterogenic mass 14×15×16 cm adjacent to the left kidney. Hormonal workup demonstrated markedly elevated estradiol levels, while DST 1mg and urinary catecholamines were within normal limits. ACC was suspected and the patient was initiated on neoadjuvant mitotane therapy, followed by en bloc adrenalectomy with nephrectomy. Histopathological analysis confirmed ACC with a WEISS score of 4, stage II T2N0M0, and Ki-67 index of 15%. The patient was maintained on adjuvant mitotane therapy with steroid replacement. Outcome and Follow-up: Two years later, surveillance CT revealed a 1 cm pulmonary nodule in the left upper lobe. Surgical resection confirmed metastatic ACC. Following metastasectomy, estradiol levels normalized. The patient has remained on mitotane therapy with no evidence of further recurrence during five years of follow-up. Conclusion Estrogen-producing ACCs are exceptionally rare and often diagnosed late. Complete surgical resection is the cornerstone of treatment. Mitotane is recommended in high-risk cases, although its optimal duration remains controversial. This case highlights the importance of including estradiol in the evaluation of male hypogonadism and gynecomastia, as early recognition of FATs can improve outcomes.
Nassar et al. (Thu,) reported a other. Complete surgical resection and neoadjuvant mitotane therapy led to normalization of estradiol levels and no further recurrence in a male with estrogen-producing ACC after five years.