Co-occurrence of pheochromocytoma and paraganglioma of the organ of Zuckerkandl resected simultaneously by laparoscopy: a rare case report and literature review

Abstract

Pheochromocytomas (PHEOs) and paragangliomas are generally grouped as rare chromaffin cell tumors. The co-occurrence of PHEOs and paragangliomas of the organ of Zuckerkandl (POZ) is extremely rare. The most common symptom of pheochromocytoma-paraganglioma (PPGL) is hypertension, and open surgery is still recommended for the treatment of large PPGLs. Herein, we report a case of a successful simultaneous laparoscopic resection of a large PHEO accompanied by POZ in a 40-year-old man with normal blood pressure. DNA analysis revealed a mutation in the succinate dehydrogenase subunit B in both the PHEO and the POZ. To the best of our knowledge, this is the first report of tumors occurring simultaneously in these two locations. We believe that the co-occurrence of PHEO and POZ is extremely rare, and the possibility of PPGL cannot be ruled out in patients with normal blood pressure. The decision to perform laparoscopic surgery remains questionable for patients with a large PHEO and POZ. In addition, a genetic examination should be performed to identify the existence of PPGL-related inherited syndromes.