Rare Recurrent ‘Aldosterone Secreting Adrenocortical Carcinoma (APACs)’ Requiring Adjuvant Therapy

Presentation Number: SAT 384
Date of Presentation: April 1st, 2017

Itivrita Goyal* and Chi Tang
University at Buffalo, Buffalo, NY


Background: Adrenocortical carcinoma (ACC) is extremely rare with an incidence of 1-2 cases per 1 million population and account for 0.05%-0.2% of all malignancies. In addition, aldosterone-producing adrenocortical carcinoma (APAC) accounts for less than 1% of cases of primary hyperaldosteronism. It is a common practice to give mitotane as adjuvant therapy for post-surgical ACC patients with high risk of recurrence1. However, there is no randomized clinical trial on the selection of patients for mitotane adjuvant therapy due to the rarity of ACC. Based on small retrospective studies, an international panel of ACC specialists proposed that mitotane therapy should not be mandatory for stage I or II disease (based on the European Network for the Study of Adrenal Tumors staging system) with negative resection margin and Ki-67<10%2. Here we report a case of aldosterone-producing ACC that fulfilled the criteria above, and therefore did not receive adjuvant mitotane therapy. Despite fitting the above criteria, he developed recurrence of the tumor in one year after left adrenalectomy.

Clinical case: A 57-year-old man was referred to our endocrinology clinic for persistent hypertension and hypokalemia. Laboratory tests showed serum sodium levels varied between high normal and high with persistently low serum potassium (in the absence of potassium depleting diuretics), plasma renin activity (PRA) of 0.28 ng/ml/hour, plasma aldosterone concentration (PAC) of 36.6 ng/dl and markedly elevated PAC/PRA ratio of 130.71 ng/dl per ng/ml/hr. CT abdomen/pelvis showed a 4.3 cm mass in the left adrenal gland. Repeated CT scans showed marked interval enlargement, suggestive of an adrenal carcinoma. The patient underwent left adrenalectomy. Pathology showed a 7 cm organ-confined, necrotic adrenocortical carcinoma with clear margin, large vessel (muscular venous) vascular invasion and Ki-67 of <10%. No evidence of regional lymph node or distant metastasis was identified. Plasma aldosterone became undetectable after the operation. Adjuvant therapy was not started as it was categorized as stage II, Ki-67 was < 10% and resection margin was negative. However, a year following surgery the patient developed a nodule in the bed of the resected left adrenal gland. Another resection was performed and pathology showed recurrent adrenocortical carcinoma partly evident within a distended muscular vein. Mitotane was started since then.

Conclusion: Therefore, we propose that vascular invasion and necrotic state of the tumor might be associated with higher risk of recurrence and should therefore be considered in determining the need for adjuvant chemotherapy, in addition to the criteria based on the international consensus. This case provides evidence to support Weiss criteria, which takes into account mitotic count, tumor necrosis and vascular invasion status in predicting recurrence.


Nothing to Disclose: IG, CT