Surgical treatment of pheochromocytoma in MEN 2

Abstract

Multiple endocrine neoplasia type 2 (MEN 2) is a rare autosomal dominant cancer syndrome. Forty to fifty percent of patients with MEN 2A develops pheochromocytoma. Surgeons treating these patients with pheochromocytoma have always been faced with question of whether to perform mono-or bilateral adrenalectomy and the timing of surgical intervention. Over the past 20 years, thanks to the development of ever more sophisticated techniques of diagnostic imaging (TC, MRI, Scintigraphy, PET), which make it possible to identify small lesions, and to ever more rapid laboratory tests, there has been a change in the surgical management of this condition. Surgeons moved from bilateral open adrenalectomy (6- 9) to laparoscopic partial adrenalectomy and cortical sparing (10-13). After partial adrenalectomy one third of the patients require replacement therapy because the function of the residual parenchyma was compromised by excessive devascularization during surgery. In patients with bilateral pheochromocytoma it is advisable to perform only partial adrenalectomy of at least one gland, i.e. to completely remove the gland with the larger lesion and remove part of the gland with the smaller lesion to reduce the risk of recurrence. The authors report 4 cases of MEN 2, including 2 first-degree relatives, which illustrate the progress made in surgical treatment for pheochromocytoma.