SAT-383 Aldosterone-Producing Adrenocortical Carcinoma With Poor Prognostic Factors Successfully Treated With Multimodal Therapy

Abstract J. Llumiquinga: None. P. Frudit: None. F.Q. Aratani: None. S. Beeby: None. V. Srougi, MD: None. J. Chambo, MD: None. F. Ledesma, MD: None. M. Polo: None. C.R. Victor: None. A. Hoff: None. A. Latronico: None. B.B. Mendonca: None. M.Q. Almeida: None. M.C. Fragoso: None. Background: Adrenocortical carcinomas (ACCs) are rare malignancies with an incidence of one to two per million per year. Aldosterone-producing ACCs (APACs) are extremely rare with an incidence of less than 1%. We describe a rare case of APAC, presenting with proximal upper and lower limb weakness and persistent hypokalemia with aldosterone and cortisol co-secretion. Clinical Case: A 60-year-old male Brazilian diagnosed with hypertension at age 40 years old, without significant family history of neoplasia, presented progressive weakness and paresis in the upper and lower limbs associated with weight loss (5 kg in one month). He had neither clinical feature suggestive of Cushing’s syndrome, or pheochromocytoma (PCC) or resistant hypertension (RH). Biochemical test revealed hypokalemia 1.3 (3.5 a 5.1 mEq/L), which persisted despite serial corrections, primary aldosteronism - aldosterone levels 73.5 ng/dL (NR 39.2 ng/dL), renin < 4 uUI/mL (up to 39.9 uUI/mL) - and autonomous cortisol secretion - cortisol (08:00 am) 11.8 µg/dL (NR: 6.7 a 22.6 µg/dL), urinary cortisol levels 632.9 µg/24Hs (NR: 15 a 122 µg/24Hs), midnight salivary cortisol levels 0.742 μg/dl (NR: <0.274 μg/dl) overnight DST with 1 mg of dexamethasone 15.1 µg/dl (NR: < 1.8µg/dl). Plasma ACTH levels and normetanephrine were normal. Contrast-enhanced abdominal CT (CECT) revealed a heterogeneous mass measuring 7.1 x 5.2 x 8.2 cm with lobulated margins in the left adrenal, with marked glycolytic metabolism. The patient was submitted to video laparoscopic left adrenalectomy (R0 status) and pathology revealed ACC with a modified Weiss score of 6/7, Ki67 41%, GRAS score 3/4, Helsinki Score 49. The tumor was classified as ENSAT III, T3 N0 M0. He underwent four cycles of adjuvant chemotherapy with EDP plus mitotane. High-dose hydrocortisone plus fludrocortisone were also prescribed. Following 3 years of diagnosis, subsequent PET-CT showed no areas of glycolytic hypermetabolism suspicious for recurrence. Adrenal insufficiency, hypogonadism and hypothyroidism developed due to side effects of mitotane. He received hormonal replacement therapy. Currently the patient presents with preserved functional status - ECOG 0, Karnofsky 90% with global and free survival of 3 years. Discussion: In our case, despite the poor prognostic factors, there was no evidence of local recurrent or metastatic lesions during the follow-up period although it is imperative to remember that there is a high chance of recurrence or metastases with recurrence, demanding close imaging and clinical follow-up. Saturday, June 1, 2024