以前列腺占位伴排尿相关症状为特征的前列腺副神经节瘤诊治分析

Clinicopathological characteristics and diagnostic strategies of prostatic paraganglioma presenting with prostatic mass and micturition-associated symptoms

摘要

目的 探讨前列腺副神经节瘤的临床特征、诊断及治疗策略，以期为该罕见病的早期诊断与精准治疗提供循证依据。方法 回顾性分析南京大学医学院附属鼓楼医院收治的1例前列腺副神经节瘤患者的临床资料。同时系统检索PubMed、中国知网及万方等国内外数据库，汇总分析1979-2025年间报道的24例（含本例共计25例）前列腺副神经节瘤患者的临床表现、实验室检查、病理特征、治疗方式及预后数据。结果 流行病学特征：全组25例患者发病年龄为8～76岁，平均（44.6±15.2）岁，以30～50岁中青年男性为主。20.0%（5/25）的患者表现为特征性的排尿相关儿茶酚胺释放症状（如排尿后心悸、一过性高血压等）。实验室检查特征：在进行儿茶酚胺代谢产物检测的19例患者中，14例（73.7%）表现为血或尿去甲肾上腺素及甲氧基肾上腺素类物质显著升高。病理与免疫组化特征：患者神经内分泌标志物嗜铬粒蛋白A、突触素及神经元特异性烯醇化酶阳性率均为100%，前列腺特异性抗原（PSA）与前列腺特异性酸性磷酸酶阴性率均为100%。治疗与预后：96.0%（24/25）的患者接受了外科手术，术式包括根治性前列腺切除术（37.5%）、肿瘤精准切除术（25.0%）及经尿道切除术（12.5%）等。全组89.5%（17/19）的患者预后良好，未见复发。结论 前列腺副神经节瘤临床罕见，对于PSA正常的前列腺占位且有排尿相关心血管症状的患者应高度怀疑该病，儿茶酚胺代谢产物检测是关键筛查手段。机器人辅助手术可实现精准的肿瘤切除与良好的功能保护。鉴于该病的潜在恶性可能及遗传相关性，术后建议终身随访。

Abstract

Objective To evaluate the clinical characteristics, diagnostic reasoning, and therapeutic strategies of prostatic paraganglioma (PPG) to provide an evidence-based foundation for early diagnosis and precision treatment of this rare malignancy. Methods The clinical data of a patient with PPG admitted to our hospital were retrospectively analyzed. Concurrently, a systematic search was conducted across domestic and international databases, including PubMed, CNKI, and Wanfang. Clinical manifestations, laboratory tests, pathological features, therapeutic modalities, and prognostic data of 24 cases reported between 1979 and 2025 were pooled with the institutional case for a comprehensive analysis (n=25). Results Epidemiological features: the age at onset ranged from 8 to 76 years, with a mean age of (44.6±15.2) years, predominantly affecting young and middle-aged adults (30-50 years). Notably, 20.0% (5/25) of the patients exhibited characteristic micturition-associated catecholamine release symptoms (e.g., post-micturition palpitations, transient hypertension). Laboratory findings: among the 19 patients who underwent catecholamine metabolite testing, 14 (73.7%) showed significant elevation in serum or urinary norepinephrine and methoxyadrenaline levels. Pathological and immunohistochemical features: the positivity rate for neuroendocrine markers (chromogranin A, synaptophysin, and neuron-specific enolase) was 100%, while the negativity rate for prostate-specific markers (prostate-specific antigen and prostate-specific acid phosphatase) was also 100%. Treatment and prognosis: surgical intervention was performed in 96.0% (24/25) of the patients, including radical prostatectomy (37.5%), precision tumor excision (25.0%), and transurethral resection (12.5%). Favorable outcomes without recurrence were observed in 89.5% (17/19) of the total cohort. Conclusion PPG is clinically exceedingly rare. The disease should be highly suspected in patients presenting with a prostatic mass, normal prostate-specific antigen level, and micturition-associated cardiovascular symptoms. Testing for catecholamine metabolites is the key screening tool. Robot-assisted surgery facilitates precise tumor excision while ensuring optimal functional preservation. Given the malignant potential and genetic correlations of PPG, lifelong follow-up is recommended.

关键词

前列腺副神经节瘤 / 嗜铬细胞瘤 / 儿茶酚胺 / 排尿后心悸 / 机器人辅助手术

Key words

prostatic paraganglioma / pheochromocytoma / catecholamine / post-micturition palpitations / robot-assisted surgery

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