副神經節瘤為罕見的原發於腎上腺外嗜鉻細胞的腫瘤,約佔所有原發於嗜鉻細胞腫瘤的10~18%;發生率也只有腎上腺嗜鉻細胞瘤的十分之一。本文藉由腹部電腦斷層的發現,我們提出一位罕見的惡性副神經節瘤合併有肺部轉移之病例報告。由於副神經節瘤的臨床表現非常多樣化且缺乏專一性,因此約有十分之一的副神經節瘤是在影像學檢查中意外發現的。因此熟悉副神經節瘤的影像學表現及其可能的臨床特徵,將有利於後腹腔腫瘤的鑑別診斷及腫瘤局部侵犯的評估。若術前有考慮到副神經節瘤的可能性,則應該針對其代謝產物做詳細的生化學檢驗及針對其中可能發生的高血壓危象做預防性的藥物投與。
Paragangliomas are rare tumors arising from extraadrenal chromaffin cell and accounting for 10~48% of all chromaffin tissue-related tumors and one tenth of pheochromocytomas. Herein we present a case of malignant retroperitoneal paraganglioma with lung metastasis and the computed tomography (CT) findings. Approximately 10% of paragangliomas are discovered on imaging study for evaluation of unrelated symptoms. The combination of radiological findings and clinical characteristics may be helpful to diagnose retroperitoneal tumors, including paragangliomas and other neurogenic tumors and, to determine the local extent of tumor. Preoperative suspicion of paraganglioma mandates biochemical screening and prevention for perioperative hypertensive crisis.