Choi Hye Ryeon, Yap Zeng, Choi Soon Min, Choi Sun Hyung, Kim Jin Kyong, Lee Cho Rok, Lee Jandee, Jeong Jong Ju, Nam Kee-Hyun, Chung Woong Youn, Kang Sang-Wook
Department of Surgery, Yonsei University College of Medicine, Seoul, Korea.
Ann Surg Treat Res. 2020 Dec;99(6):315-319. doi: 10.4174/astr.2020.99.6.315. Epub 2020 Nov 26.
Paragangliomas (PGL) are rare neuroendocrine tumors derived from chromaffin cells of the autonomic nervous system. We aim to describe our experience and the long-term outcome of abdominal PGL over the last decade.
A retrospective review of patients diagnosed with PGL in our hospital between November 2005 and June 2017 was conducted. All nonabdominal PGL were excluded and the clinicopathological features and long-term outcomes of the patients were analyzed.
A total of 46 patients were diagnosed with abdominal PGL. The average age of diagnosis was 55.4 years and there was no sex predilection. The average tumor size was 5.85 cm and they were predominantly located in the infrarenal position (50%). The mean follow-up period was 42 months (range, 1.8-252 months). All patients with metastases had Pheochromocytoma of the Adrenal Gland Scaled Score (PASS) of ≥4. One patient presented with synchronous metastases while 2 developed local recurrence and distant metastases. One presented with only local recurrence. One patient died 5 years after diagnosis.
Abdominal PGL is a rare tumor with excellent long-term prognosis. Recurrence although uncommon, can occur decades after initial diagnosis. Long-term follow-up is therefore recommended for all patients with PGL, especially in patients with PASS of ≥4.
副神经节瘤（PGL）是源自自主神经系统嗜铬细胞的罕见神经内分泌肿瘤。我们旨在描述过去十年中腹部PGL的诊疗经验及长期预后情况。
对2005年11月至2017年6月期间在我院诊断为PGL的患者进行回顾性研究。排除所有非腹部PGL病例，并分析患者的临床病理特征和长期预后情况。
共46例患者被诊断为腹部PGL。诊断时的平均年龄为55.4岁，无性别差异。肿瘤平均大小为5.85 cm，主要位于肾下位置（50%）。平均随访时间为42个月（范围1.8 - 252个月）。所有发生转移的患者肾上腺嗜铬细胞瘤评分（PASS）≥4。1例患者出现同步转移，2例发生局部复发和远处转移。1例仅出现局部复发。1例患者在诊断后5年死亡。
腹部PGL是一种罕见肿瘤，长期预后良好。复发虽不常见，但可在初始诊断数十年后发生。因此，建议对所有PGL患者进行长期随访，尤其是PASS≥4的患者。
