Endocrine Abstracts (2008) 16 P328

Cushing syndrome with an atypical evolution

Pierre Lecomte1, Peggy Pierre1, Marie Castric1, Christine Chabrolle1, Loïc de Calan2 & Serge Guyétant3

1Endocrine Unit, University Hospital, Université F. Rabelais, 37044 Tours Cedex 9, France; 2Endocrine Surgery, Universitary Hospital, Université F. Rabelais, 37044 Tours Cedex 9, France; 3Pathology Unit, University Hospital, Université F. Rabelais, 37044 Tours Cedex 9, France.

A 72-year-old depressed woman complained about fatigue, weight gain (20 kilos/2 years), severe and recent hypertension, easy bruises, and venous insufficiency. Whereas she was treated with six antihypertensive drugs, she had hypokaliemia (2.6 mM). Cardiac ultrasounds showed myocardial hypertrophy. A Cushing syndrome was suspected. UFC was increased (400 mM/d), cortisol cycle disrupted and a standard DXM test showed no cortisol suppression (775 nM/l). ACTH levels were consistently low. A 4 cm round mass with calcifications, discovered by CT scan in the left adrenal gland, was removed by laparoscopy. Pathological summary was 5 cm benign adrenal tumour (Weiss score 2). The patient was treated with 15 mg hydrocortisone. Nine months later, she had gained 3 more kilos with biological signs favouring osteoporosis. UFC was 1280 nM/d without DXM suppression (cortisol 604 nM/l) and kaliemia 3.2. A new CT scan visualized a polylobular mass in the same surgical area and several ‘nodules’ near pancreas cauda, spleen and around spleen vein. After a ketoconazole short treatment, about twenty small nodules from 1 mm to 2.5 cm were surgically removed in the epiploon, near the pancreas cauda and the spleen, similar to the main tumour (extemporaneous examination), apparently.

Pathological results indicated that nodules limited by fibrosis were mostly agglomerates of large cells with 9 mitoses/50 large power fields and necrosis. Some foci were different with apoptosis, necrosis and oncocytes: 8 mitoses/10 hpf (Ki 67 score 10–15%). There was no vascular invasion. Pathological diagnosis was adrenocortical carcinoma. The previous report was reviewed and noticed a 3 Weiss score (tumour of uncertain malignancy). The patient is treated with mitotane, hydrocortisone and fludrocortisone. Her condition is currently stable with some side effects of mitotane.

This observation underlines the remaining difficulties of pathological exam of endocrine tumours even when they are performed by skilled pathologists.

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