With the early management of medullary thyroid carcinoma, multiple endocrine neoplasia type 2 has become an example of chronic disease with prolonged follow-up to look for pheochromocytoma diagnosis and appropriate management. In any genetic disease where pheochromocytoma can happen bilaterally, the surgery has long been based on bilateral adrenalectomy (even in the presence of a single pheochromocytoma), and as a consequence on a life long treatment for adrenal insufficiency. Progress has then been made by choosing unilateral adrenalectomy in case of unilateral pheochromocytoma, which now makes sense. Adrenal sparing surgery is probably another step in trying to improve the patients overall quality of life. It is a valuable option well described in patients with pheochromocytoma and Von Hippel Lindaus disease. Determining the interest of such a therapeutic approach implies to take into account the risk of recurrence, in comparison with the gain in terms of adrenal function. Moreover, assuming this risk of recurrence implies to characterize the potential of malignancy of hereditary pheochromocytoma, considering that already medullar hyperplastic tissue will remain after surgery. Data on MEN2 patients are scarce in the literature, usually based on a low number of patients, or a short-term follow-up, and this might explain why this approach is still not considered as the gold standard (when technically do-able).
Based on a large international consortium collecting data on 1210 patients with multiple endocrine neoplasia type 2, including more than 500 with pheochromocytoma, we determined the outcome of patients with adrenal sparing surgery in comparison with classical adrenalectomy. Our results emphasized the low rate of adrenal insufficiency, and a relatively low risk of recurrence after a median follow-up of 10 years after adrenal sparing surgery. Interestingly, the rate of recurrence was comparable in patients with classical adrenalectomy, whereas, as expected, the rate of adrenal insufficiency was highly superior with the classical approach. Adrenal sparing surgery is thus clearly a valuable therapeutic option, provided a prolonged and regular follow-up can be maintained (Castinetti et al., Lancet Oncology, 2014).
16 - 20 May 2015
European Society of Endocrinology