Third Hospital of Shanxi Medical University, Shanxi Bethune Hospital, Shanxi Academy of Medical Sciences, Tongji Shanxi Hospital, Taiyuan, China.
J Int Med Res. 2023 Mar;51(3):3000605231161211. doi: 10.1177/03000605231161211.
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.
嗜铬细胞瘤(PHEO)和副神经节瘤通常被归类为罕见的嗜铬细胞肿瘤。Zuckerkandl 器官的 PHEO 和副神经节瘤(POZ)同时发生的情况极为罕见。嗜铬细胞瘤-副神经节瘤(PPGL)最常见的症状是高血压,对于大型 PPGL,仍建议采用开放手术治疗。在此,我们报告了 1 例 40 岁男性血压正常的大型 PHEO 合并 POZ 的成功腹腔镜切除病例。DNA 分析显示 PHEO 和 POZ 中琥珀酸脱氢酶亚基 B 均发生突变。据我们所知,这是这两个部位同时发生肿瘤的首例报道。我们认为 PHEO 和 POZ 同时发生的情况极为罕见,对于血压正常的患者不能排除发生 PPGL 的可能性。对于大型 PHEO 和 POZ 的患者,是否进行腹腔镜手术的决定仍存在疑问。此外,应进行遗传检查以确定是否存在与 PPGL 相关的遗传性综合征。