Endocrine Abstracts

JOINT1957

Introduction: Adrenocortical carcinomas are epithelial malignant tumors arising in the cortical zone of the adrenal gland. They are extremely aggressive and very rare, with an incidence of 1 to 2 cases / year / million population and a prevalence of 4 to 12 cases per million population, in contrast to benign adrenal tumors, which account for 3% of the general population over the age of 50. These tumors have been divided into non-functional and functional tumors. We report a rare case of adrenocortical carcinoma with co-secretion of aldosterone and cortisol.

Case report: A 46-year-old patient referred to our department for etiological assessment of malignant hypertension for 8 years and severe hypokalemia. He presented with edema of the lower limbs and acanthosis nigricans. The aldosterone/renin ratio (ARR) was 381 pmol/mUI, indicating aldosterone excess. Dexamethasone minute braking was negative at 9.7mg/dl. Abdominal CT scan revealed a large, relatively well-limited, calcified left adrenal mass with heterogeneous enhancement, measuring approximately 63 x 86 x90 mm, with no other abdominal lesions. The patient underwent unilateral adrenalectomy, which resulted in a large, ovoid, firm, whitish-grey mass. Pathological examination with immunohistochemical complement was compatible with adrenocortical carcinoma, a Weiss score of 3. Postoperatively, the patient’s blood pressure returned to normal and hypokalemia did not recur.

Discussion: Adrenocortical carcinoma is a rare malignancy. The clinical presentation is variable. Functional or secretory adrenocortical carcinomas cause an endocrine syndrome. In order of frequency, this may be isolated hypercorticism or hypercorticism associated with virilization or feminization, or primary hyperaldosteronism, as in the case of our patient. The literature confirms the rarity of exclusive aldosterone production by adrenocortical carcinoma. The diagnosis is made in the presence of signs of hormonal hypersecretion dependent on the hormone synthesis pathway developed by the tumoral endocrine tissue, or in the presence of a tumoral syndrome. Adrenal CT is the first-line examination for adrenal exploration in cases of hormonal hypersecretion syndrome. Surgery is the first-line treatment, whatever the type of secretion. In cases of recurrence where excision has been incomplete or rejected, the second-line treatment is mitotane alone or combined with chemotherapy. Adjuvant radiotherapy is not systematic, and is mainly used to treat secondary bone or brain damage.

Conclusion: This case illustrates the importance of a comprehensive hormonal evaluation of patients presenting with a picture of primary hyperaldosteronism with or without functional signs of hypercorticism, to diagnose these likely underestimated co-secretions.