Adrenal-sparing surgery for phaeochromocytoma

Background: Adrenalectomy is the current treatment for phaeochromocytoma. C onsequently, patients with bilateral adrenal phaeochromocytoma become stero id dependent. An adrenal-sparing surgical technique was introduced in 1985. The results of this treatment have been reviewed. Methods: Since 1985, 39 patients with adrenal phaeochromocytoma (16 men and 23 women, aged 10-76 years) have been treated. Thirty-three patients had u nilateral and six had bilateral phaeochromocytomas. Seven of the former 33 had a contralateral adrenal tumour resected previously. All 39 patients wer e re-evaluated biochemically and clinically for ipsilateral recurrence. Results: Adrenal-sparing surgery was performed successfully in 37 of the 39 patients. In one, adrenal-sparing resection was impossible anatomically an d total adrenalectomy was necessary. Another patient with bilateral tumours had retroperitoneal haemorrhage and became steroid dependent. None of the remaining 12 patients who had bilateral adrenal surgery required steroid re placement. Adrenocortical function was normal in eight and mildly impaired in two of the ten patients who had evaluation by adrenocorticotrophic hormo ne stimulation. After a mean follow-up of 73 months, one patient with von H ippel-Lindau disease developed a recurrence in the ipsilateral adrenal glan d. Genetic testing revealed that 26 of the 39 patients, including half of t hose with a unilateral tumour, had hereditary phaeochromocptoma. Conclusion: Adrenal-sparing surgery is safe and effective, and may become t he treatment of choice in patients with hereditary phaeochromocytoma.