Pheochromocytomas (PHEOs) are rare catecholamine-secreting adrenal tumors. Approximately
60-90% of bilateral PHEOs are hereditary. We retrospectively analyzed the clinical
characteristics of patients with bilateral PHEOs and the morbidity rate (malignancy,
tumor recurrence and adrenal insufficiency rate) related to surgery technique and
genetic status of the patients.Fourteen patients (12.5%, 9 women, 5 men) had synchronous
or metachronous bilateral PHEOs (out of 112 PHEO patients who underwent surgery between
1976-2021). Mean age at diagnosis was 35 ± 18.5 years (3 were children). Nine patients
(64.2%) presented synchronous bilateral tumors, 5 (35.7%) contralateral metachronous
tumors, 2-12 years after the first surgical intervention; 3 (21.4%) were metastatic.
Median follow-up: 5 years (1-41), IQR 19 months. 78.5% had a germline mutation (8
RET gene with MEN2A syndrome, 3 VHL syndrome, 3 not tested). Post surgery recurrence
was noted in 16.6% of patients (1 with MEN2A syndrome and metastatic PHEOs, 1 with
VHL syndrome), with similar rates after total adrenalectomy or cortical-sparing adrenal
surgery. Adrenal insufficiency was avoided in 40% after cortical-sparing surgery.Bilateral
PHEOs are usually associated with genetic syndromes. The surgical technique for patients
with hereditary bilateral PHEOs should be chosen based on a personalized approach,
as they are at higher risk for developing new adrenal tumors requiring additional
surgeries.
