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罕见侵袭性疾病:膀胱平滑肌肉瘤

Rare and Aggressive Disease: Urinary Bladder Leiomyosarcoma.

作者信息

Venclovas Zilvinas, Simkunaite Kotryna, Pijadin Vaidas, Auskalnis Stasys, Jievaltas Mindaugas, Navickis Tomas, Milonas Daimantas

机构信息

Department of Urology, Lithuanian University of Health Sciences, LT-44307 Kaunas, Lithuania.

Faculty of Medicine, Medical Academy, Lithuanian University of Health Sciences, LT-44307 Kaunas, Lithuania.

出版信息

J Clin Med. 2025 Aug 25;14(17):5999. doi: 10.3390/jcm14175999.

DOI:10.3390/jcm14175999
PMID:40943759
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12429293/
Abstract

: Bladder leiomyosarcoma is an extremely rare non-urothelial malignancy, accounting for less than 0.1% of all bladder tumors. It presents significant diagnostic and therapeutic challenges due to its aggressive nature and the absence of standardized treatment protocols. : We report the case of a 61-year-old woman who presented with hematuria, dysuria, and suprapubic pain. Imaging revealed a large, locally invasive bladder mass, and histopathological examination following transurethral resection confirmed leiomyosarcoma. The patient underwent radical cystectomy with resection of adjacent bowel segments and urinary diversion. Histology showed a high-grade leiomyosarcoma (pT3N0) with extensive necrosis and a high mitotic index. Two months postoperatively, peritoneal dissemination was detected. Systemic chemotherapy with dacarbazine and doxorubicin initially led to the regression of metastases, but disease progression occurred within months, including lung, liver, and bone metastases. Palliative radiotherapy and second-line chemotherapy were initiated. As of now, 16 months have elapsed since surgery. : This case underscores the aggressive clinical course of bladder leiomyosarcoma despite multimodal therapy and the urgent need for individualized management strategies. Given its rarity, this case contributes to the limited literature and highlights the importance of vigilant follow-ups and further studies to establish evidence-based treatment protocols.

摘要

膀胱平滑肌肉瘤是一种极其罕见的非尿路上皮恶性肿瘤,占所有膀胱肿瘤的比例不到0.1%。由于其侵袭性本质以及缺乏标准化治疗方案,它带来了重大的诊断和治疗挑战。我们报告了一例61岁女性病例,该患者出现血尿、排尿困难和耻骨上疼痛。影像学检查发现一个巨大的、局部浸润性膀胱肿块,经尿道切除术后的组织病理学检查确诊为平滑肌肉瘤。患者接受了根治性膀胱切除术,切除了相邻肠段并进行了尿流改道。组织学显示为高级别平滑肌肉瘤(pT3N0),伴有广泛坏死和高有丝分裂指数。术后两个月,检测到腹膜播散。使用达卡巴嗪和阿霉素进行全身化疗最初使转移灶消退,但数月内疾病进展,包括出现肺、肝和骨转移。于是开始进行姑息性放疗和二线化疗。截至目前,手术已过去16个月。该病例强调了尽管采取了多模式治疗,膀胱平滑肌肉瘤的临床病程仍具有侵袭性,且迫切需要个体化管理策略。鉴于其罕见性,该病例丰富了有限的文献资料,并凸显了进行密切随访以及开展进一步研究以建立循证治疗方案的重要性。

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本文引用的文献

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Urinary incontinence as the first clinical symptom of urinary bladder leiomyosarcoma.尿失禁作为膀胱平滑肌肉瘤的首发临床症状。
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