Tosev Georgi, Mendler Michael, Bergmann Frank, Klein Tilman, Pahernik Sascha A, Hadaschik Boris A, Hohenfellner Markus
Department of Urology, University of Heidelberg , Heidelberg, Germany .
Department of Medicine 1 and Clinical Chemistry, University of Heidelberg , Heidelberg, Germany .
J Endourol Case Rep. 2016 Dec 1;2(1):227-231. doi: 10.1089/cren.2016.0119. eCollection 2016.
The present case report describes an extremely rare case of a norepinephrine secreting extraadrenal paraganglioma (PGL) located in the seminal vesicle. A 36-year-old patient had signs of intermittent hypertensive derailments, bradycardia, increased norepinephrine excretion in 24-hour urine, an increased metanephrine plasma concentration, and a positive clonidine suppression test. A suspicious mass was detected in an (18)F-DOPA-PET/CT-scan in the left seminal vesicle. Following adrenergic blockade, a robotically assisted laparoscopic left vesiculectomy with negative soft tissue surgical margins was performed. The patient sustained a couple of few months of voiding difficulties of the lower urinary tract and obstruction of the left upper urinary tract after the surgery, which resolved spontaneously with home medical treatment. Two years after the initial treatment, the patient relapsed, which was confirmed by endocrinologic follow-up tests with increased urine catecholamine, a positive clonidine suppression test, as well as an elevated blood pressure. Staging with (18)F-DOPA-PET/CT-scan confirmed the diagnosis of a recurrent PGL. This was followed by subsequent open surgical removal of the suspicious lesion in the seminal fossa. The patient is still recurrence free 15 months after the second surgery. Complications after the second surgery included an intermittent paresthesia of the left leg lasting 3 to 4 months. No other urologic symptoms such as voiding or erectile dysfunction occurred. DaVinci-assisted laparoscopic vesiculectomy is a viable procedure to treat such cases providing satisfactory results. Relevant for clinical practice are the regular and lifelong follow-up examinations to detect recurrences.
本病例报告描述了一例极为罕见的位于精囊的分泌去甲肾上腺素的肾上腺外副神经节瘤(PGL)。一名36岁患者有间歇性高血压紊乱、心动过缓、24小时尿中去甲肾上腺素排泄增加、间甲肾上腺素血浆浓度升高以及可乐定抑制试验阳性的体征。在(18)F - DOPA - PET/CT扫描中,左侧精囊发现一个可疑肿块。在进行肾上腺素能阻滞剂治疗后,实施了机器人辅助腹腔镜下左侧精囊切除术,手术切缘软组织阴性。术后患者出现了几个月的下尿路排尿困难和左上尿路梗阻,经家庭药物治疗后自行缓解。初始治疗两年后,患者复发,内分泌随访检查发现尿儿茶酚胺增加、可乐定抑制试验阳性以及血压升高,证实了复发。(18)F - DOPA - PET/CT扫描分期确诊为复发性PGL。随后进行了开放性手术切除精囊窝处的可疑病变。第二次手术后15个月,患者仍无复发。第二次手术后的并发症包括左腿间歇性感觉异常,持续3至4个月。未出现其他泌尿系统症状,如排尿或勃起功能障碍。达芬奇辅助腹腔镜下精囊切除术是治疗此类病例的可行方法,效果令人满意。对于临床实践而言,定期和终身随访检查以检测复发情况至关重要。