Endocrine Abstracts

Adrenal cortical carcinoma is a rare disease with an incidence of 1–2/million per year. Diagnosis is related to autonomy of adrenal steroid synthesis, tumour size above 6 cm, local invasion and tendency to recurrence. Treatment consists in adrenal surgery, non-specific chemotherapy and radiotherapy, and, more specific-Mitotane (Lysodren).

Pathology diagnosis is based on capsular and vascular invasion, histological changes suggestive for malignancy and a high nuclear proliferation index.

We present a series of 7 cases diagnosed with adrenal carcinoma admitted in the last 3 years in the National Institute of Endocrinology, Bucharest.

The patients, 3 M and 4 F, aged 23–73 years, had large adrenal tumours 6–15.8 cm, locally invasive in liver, kidney, spleen, as proved by CT/MR imaging. The pre-surgical endocrine evaluation was available in 3/7, showing both glucocorticoid and androgen autonomous secretion. The rest were admitted after surgery for endocrine/oncological management. All cases were submitted to open surgery. Pathology data confirmed the clinical suspicion; Ki-67 immunostaining was available in 4/7 cases – between 15 and 25%. Two cases were submitted to chemotherapy and other 3 to radiotherapy, none to both. Mitotane became available in Romania in 2011, therefore 5/7 patients started this treatment, targeting the therapeutic levels 14–20 mg/dl. 4/5 reached therapeutic levels; 1 patient with extensive disease died before. Another patient died after surgery for local recurrence during Mitotane treatment, due to a fatal arrhythmia unrelated with the disease.

All the other are without any sign of morphological or biochemical recurrence after 7/11/19 month of Mitotane therapy. Glucocorticoid replacement therapy was associated in all cases; 2/3 developed primary hypothyroidism and L-T₄ substitution was started. Optimal management of adrenal carcinoma requires multidisciplinary approach and evidence of multicentric studies.