BILATERAL LAPAROSCOPIC ADRENALECTOMY WITH LEFT ADRENAL SPARING IN MEN IIA. CASE REPORT AND VIDEO PRESENTATION

Backround: The Multiple Endocrine Neoplasia type IIA (MEN IIA) syndrom is caused by mutations of the RET proto-oncogene on chromosome 10 and is transmitted to the offspring in an autosomal dominant fashion. All patients develop medullary thyroid carcinoma and the risk for pheochromocytoma and primary hyperparathyroidism is clearly associated with the presence of the RET mutation at a specific position.

Aims: We present our technique in adrenal- sparing laparoscopic resection on a patient with bilateral adrenal pheochromocytomas

Material: A 34 year-old man, who at the age of 15 underwent total thyroidectomy due to medullary thyroid carcinoma and five years later lymph node dissection of the neck. In 2006, hypertensive crisis led him to hospital. The 24 hour urine biochemical analysis demonstrated high levels of catecholamines and abdominal CT showed tumor in both adrenal glands. The MIBG scan confirmed the diagnosis of bilateral pheochromocytoma.

Results: A right laparoscopic adrenalectomy was performed and one and a half month later it was followed by a left laparoscopic adrenal-sparing resection of the tumor. His postoperative course was uneventful and his histopathological examination of both resected adrenal glands revealed pheochromocytoma. The size ofthe right specimen was 1 0.5x7x2.5 cm whereas the left was 5.5×3.6×3 cm. Two years after the procedure there is no evidence of recurrence and the patient does not receive corticosteroids

Conclusions: Adrenal-sparing resection for bilateral benign pheochromocytomas is a safe procedure that can maintain the function ofthe adrenal gland cortex and thus offer quality of life to the patient.