Endocrine Abstracts

Introduction: Bilateral pheochromocytomas (PHEOs) most often occur as components of hereditary syndromes: MEN2A, VHL disease or neurofibromatosis. The best surgical technique in such cases is yet disputed between cortical sparing or total adrenalectomy. Two important complications are related to each surgical technique: adrenal insufficiency (AI) or recurrence-which one is more harmful for the patient?

Aim: To evaluate the complications (recurrence, AI) rate reported to surgical technique and to genetic status.

Material and Methods: We retrospectively retrieved data of 103 patients diagnosed with PHEOs in a tertiary centre from Romania and identified patients with bilateral disease.

Results: We identified 11 (10.6 %) patients with bilateral disease; 8 synchronous, 3 metachronous. Mean age at diagnosis was 32±24 y.o; 3 (27.3%) patients had pediatric age at diagnosis; 6 women, 5 men. Eight patients presented with benign PHEO, 3 with malignant disease at diagnosis. Two had adrenergic pattern, 7 noradrenergic, 2 no info. Mean tumor diameter was 47.8±3 mm. Seven patients had hereditary disease - MEN2A (5), VHL (2), 4 no info. Median follow-up duration was 9 (1-41) years. The patients’ outcome reported to surgical technique: 5 had cortical sparing-(3 developed AI and 1 recurrence); 3 patients had total adrenalectomy-(both had AI, 1 recurrence); 1 patient had total unilateral adrenalectomy-contralateral tumor impossible to operate (1 recurrence); 2 no info (1 exitus, 1 lost to follow-up). The outcome reported to genetic status: from 5 patients with MEN2A, 1 had recurrence after total adrenalectomy. In patients with VHL we found 2 recurrences at 7 (bilateral) and 24 years (unilateral), at the same patient after subtotal adrenalectomy. The rest four patients without documented mutations had no recurrence. Overall, in our cohort we found a 27.2 % recurrence rate (2 patients-3 recurrences, 1 MEN2A, 1 VHL) after 1 total adrenalectomy, 1 cortical sparing. 50 % of patients with cortical sparing had AI and 100% of patients with total adrenalectomy had AI.

Conclusion: Cortical sparing does not always assure a normal cortical function. There are some factors that should be evaluated before choosing the proper surgery technique including tumor size, the ability to secure vascular supply to the remaining adrenal cortex and the likelihood of a predisposing mutation leading to high risk of recurrence/malignancy in the future. Patients with RET mutation have a higher risk of recurrence and multiple tumors in the same adrenal, therefore, total adrenalectomy is the best option for them.