ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Endocrine Abstracts (2019) 63 P16 | DOI: 10.1530/endoabs.63.P16

Cortex protecting surgery in MEN 2A

Hatice Sebile Dokmetas1, Fatih Kilicli1, Yasar Ozdenkaya2, Gunes Cavusuglu3, Meric Dokmetas3, Bugra Erol3 & Kubra Karaipek3

1İstanbul Medipol University, Department of Endocrinology, İstanbul, Turkey; 2İstanbul Medipol University, Department of General Surgery, İstanbul, Turkey; 3İstanbul Medipol University, Department of Internal Medicine, İstanbul, Turkey.

Cortex protecting surgery for bilateral pheochromocytoma protects the patient from adrenal insufficiency. Risk of relapse is low. 35 years old woman had paroxysmal seizures and hypertension crises for the last year which became more frequent in the last 10 days. She had thyroidectomy and under the use of levothyroxine the patient. Because of a nodule on ultrasonography and paternal history of MEN 2A which led to a check of calcitonin level that resulted 8 times higher than the upper limit, at the age of 23 she had had a thyroid surgery. Patology: 0.4 and 0.9 mm medullary thyroid carcinoma. Her father had a surgery for MEN 2A years ago. Abdominal MRI: Right adrenal gland had a mass of pheochromocytoma at the size of 42×41 mm, and the left one had a mass at the size of 331×32 mm. Urine metanephrin: 3093 (N:<298 ug/24 hours), normetanephrin: 6837 (N:<354 ug/ml), PTH:244 (N:15–65 pg/ml), Neck ultrasonography showed no enlarged parathyroid gland. RET protooncogen: Exon 11 and Codon 634 mutation. Surgery was scheduled for bilateral cortex protecting adrenalectomy and 3.5 parathyroidectomy. Pathology report: Parathyroid hyperplasia and pheochromocytoma. Postopetative follow up revealed normal calcium levels, cortisol: 11 mcg/dl, no hypertension. Because of the normal serum cortisol levels without any replacement, we recommend cortex protecting adrenalectomy for patients with bilateral pheochromocytoma on the condition of an experienced surgeon.

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