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膀胱副神经节瘤的临床病理特征分析:一项回顾性研究。

Clinicopathological features analysis of Paraganglioma of urinary bladder: A retrospective study.

作者信息

Li Wenhua, Wei Wei, Yuan Lijun, Zhang Ying

机构信息

Department of Pathology, Liuzhou Worker's Hospital, Liuzhou 545005, China.

Department of Pathology, Liuzhou People's Hospital, Liuzhou 545006, China.

出版信息

Ann Diagn Pathol. 2025 Aug;77:152477. doi: 10.1016/j.anndiagpath.2025.152477. Epub 2025 Mar 27.

DOI:10.1016/j.anndiagpath.2025.152477
PMID:40158236
Abstract

Paraganglioma of urinary bladder (PUB) is a rare neuroendocrine neoplasm. This study is a retrospective analysis of clinicopathological features in 11 cases of PUB. The studied cohort included seven male and four female patients with a median age of 64 years (range 37-73 years). The maximum tumor diameter ranged from 1 to 4 cm (median: 2.5 cm). Macroscopically, most lesions appeared as smooth, polypoid intraluminal protrusions; one case exhibited a nodular mass extending into the outer bladder wall. Microscopic evaluation demonstrated tumor infiltration into the muscularis propria (6 cases) or both lamina propria and muscularis propria (5 cases). Tumor cells were arranged in nested (Zellballen) or organoid patterns. Tumor cells uniformly expressed CD56, synaptophysin, and chromogranin. The Ki-67 proliferation index was ≤8 % in 10 cases; one case with a 4 cm tumor demonstrated a higher Ki-67 index (20 %), correlating with infiltrative growth and increased mitotic activity. Among the 10 cases that were evaluated, 2 (20 %) showed a loss of SDHB expression; Eight (80 %) of 10 cases were GATA3-positive, and all cases were negative for OCT3/4. Nine (81.8 %) underwent transurethral resection of bladder tumor, and 2 (18.2 %) underwent partial cystectomy. Intraoperative blood pressure fluctuations were observed in 2 patients (18.2 %). The median follow-up time was 26 months (range 4-73 months); one patient experienced a recurrence of endometrial cancer 4 years later and was lost to follow-up at 73 months; the remaining 10 patients survived without recurrence or metastasis. Improved preoperative recognition of PUBs relies on integrating clinical, biochemical, and imaging findings. Standardized immunohistochemical panels may enhance diagnostic accuracy.

摘要

膀胱副神经节瘤(PUB)是一种罕见的神经内分泌肿瘤。本研究对11例PUB患者的临床病理特征进行了回顾性分析。研究队列包括7例男性和4例女性患者,中位年龄为64岁(范围37 - 73岁)。肿瘤最大直径为1至4厘米(中位值:2.5厘米)。宏观上,大多数病变表现为光滑的息肉样腔内突起;1例表现为结节状肿块延伸至膀胱外壁。显微镜评估显示肿瘤浸润至固有肌层(6例)或固有层和固有肌层(5例)。肿瘤细胞呈巢状(Zellballen)或类器官模式排列。肿瘤细胞均表达CD56、突触素和嗜铬粒蛋白。10例患者的Ki-67增殖指数≤8%;1例肿瘤直径为4厘米的患者Ki-67指数较高(20%),与浸润性生长和有丝分裂活性增加相关。在评估的10例病例中,2例(20%)显示SDHB表达缺失;10例中的8例(80%)GATA3呈阳性,所有病例OCT3/4均为阴性。9例(81.8%)接受了经尿道膀胱肿瘤切除术,2例(18.2%)接受了部分膀胱切除术。2例患者(18.2%)术中出现血压波动。中位随访时间为26个月(范围4 - 73个月);1例患者4年后子宫内膜癌复发,73个月时失访;其余10例患者无复发或转移存活。提高PUB的术前识别依赖于整合临床、生化和影像学检查结果。标准化免疫组化检测组合可能提高诊断准确性。

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引用本文的文献

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An Unusual Entity in Urology: Urinary Bladder Paraganglioma.泌尿外科中的一种罕见实体:膀胱副神经节瘤。
J Med Cases. 2025 Aug 22;16(8):287-292. doi: 10.14740/jmc5145. eCollection 2025 Aug.
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Case report: Synchronous bladder and retroperitoneal paragangliomas: an extremely rare entity.病例报告:同步性膀胱和腹膜后副神经节瘤:一种极其罕见的疾病。
Front Oncol. 2025 Aug 13;15:1593934. doi: 10.3389/fonc.2025.1593934. eCollection 2025.