Objective: Bilateral pheochromocytomas are rare diseases. The purpose of this retrospective study was to elucidate the clinical characteristics of patients with bilateral pheochromocytomas.
Methods: We analyze the clinical data of 25 patients with bilateral pheochromocytomas who were treated at Peking Union Medical College Hospital between 1952 and 2004.
Results: The average age at diagnosis was (32 +/- 14) years. 19 cases (76%) were familiar type, and among the 19 cases, 13 cases were multiple endocrine neoplasia (MEN), 5 cases von Hippel-Lindau (vHL) disease and 1 case of isolated familial pheochromocytomas. In the 25 patients, bilateral pheochromocytomas were discovered at the same time in 88%, and multiple tumors existed in at least one side of the adrenal gland in 56%. 50% of cases recurred after resection of pheochromocytomas.
Conclusions: Hereditary syndromes should be screened when pheochromocytoma is bilateral, and the patients' family members also should be screened for hereditary syndromes. During operation for bilateral adrenal pheochromocytoma, multiple tumors in one side should be considered. Long-term follow-up is necessary because recurrence may develop many years after operation.