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Endocrine Abstracts (2017) 49 EP140 | DOI: 10.1530/endoabs.49.EP140

1Department of Endocrinology, Diabetes and Metabolism of Centro Hospitalar de São João, Porto, Portugal; 2Faculty of Medicine, Porto, Portugal; 3Department of General Surgery of Centro Hospitalar de São João, Porto, Portugal; 4Department of Pathological Anatomy of Centro Hospitalar de São João, Porto, Portugal; 5Department of Oncology of Centro Hospitalar de São João, Porto, Portugal; 6Endocrine and Cervical Pathology Multidisciplinary Group of Centro Hospitalar de São João, Porto, Portugal.


Introduction: Adrenocortical carcinoma (ACC) is a rare and aggressive endocrine malignancy with a poor prognosis. Hormone-secreting ACC appear with manifestations of virilization, feminization or Cushing syndrome.

Case Report: A 63-year-old woman with no pathological history of relief was referred to Endocrinology evaluation with an history of deepening of the voice since 1-year ago, as well as hair loss and facial and abdominal hirsutism with 6-month evolution. The analytical study revealed the following results: total testosterone 6.03 (Normal Range (NR) 0.06–0.82) ng/ml, delta-4 androstenedione >10.00 (NR 0.30–2.99) ng/ml, DHEA-S 545.8 (NR 18.9–205) μg/dl, 17-OH-Progesterone 6.32 ng/ml, SHBG 71.4 (NR 14.1–68.9) nmol/l, FSH 7.96 ng/ml, LH 5.96 ng/ml Estradiol 123.8 pg/ml; normal urinary catecholamines and metanephrines; normal renin and aldosterone values; ACTH 2.1 ng/l (NR <63.3), urinary free cortisol 353.6 (NR 36–137) μg/day, late-night salivary cortisol 0.499 (NR <0.32) μg/dl and plasma cortisol after 1-mg overnight dexamethasone suppression test 13.3 μg/dl (NR<1.8). She performed an abdominal MRI with documentation of a massive solid tumor lesion with heterogeneous contrast uptake, well delimited and measuring about 13.4 cm in diameter, which was admitted to be dependent on the left adrenal gland. Were identified several hepatic and lung nodularities suggestive of secondary lesions. In a multidisciplinary evaluation, it was decided to perform left adrenalectomy by laparotomy. No ascites or signs of peritoneal carcinomatosis were present. The anatomopathological examination revealed an ACC ENSAT stage IV, pT2NxMx, with a modified Weiss score of five and a classic Weiss score of seven. The patient is now proposed for adjuvant chemotherapy with mitotane.

Conclusions: We report a case of an ACC combining hyperandrogenism with Cushing’s syndrome. Hormonal hypersecretion, particularly hypercortisolism, is associated with a worst outcome. Complete tumor removal remains the only potentially curative treatment.

Volume 49

19th European Congress of Endocrinology

Lisbon, Portugal
20 May 2017 - 23 May 2017

European Society of Endocrinology 

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