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膀胱黏液样恶性纤维组织细胞瘤

Myxoid malignant fibrous histiocytoma of the bladder.

作者信息

Oesterling J E, Epstein J I, Brendler C B

机构信息

Department of Urology, Johns Hospkins University School of Medicine, Baltimore, Maryland.

出版信息

Cancer. 1990 Oct 15;66(8):1836-42. doi: 10.1002/1097-0142(19901015)66:8<1836::aid-cncr2820660832>3.0.co;2-y.

Abstract

Although the most common soft tissue sarcoma of adulthood, malignant fibrous histiocytoma (MFH) is an extremely rare tumor of the urinary bladder. Only three well-documented cases have been reported in the world literature. The patient presented in this report represents the first case of the myxoid variant to develop in the urinary bladder. Whereas all previous patients with MFH of the bladder had intermittent hematuria, this patient's chief complaint was bladder outlet obstruction due to extension of the tumor into the prostate. He was managed with radical cystoprostatectomy, postoperative radiation therapy to the tumor bed, and adjuvant chemotherapy using doxorubicin. The patient tolerated the therapy well and was disease-free at the 3-year follow-up visit. The histogenesis, clinical features, pathologic characteristics, and treatment considerations of this rare bladder tumor are discussed in detail.

摘要

尽管恶性纤维组织细胞瘤(MFH)是成人最常见的软组织肉瘤,但却是一种极为罕见的膀胱肿瘤。世界文献中仅报道过3例有充分记录的病例。本报告中的患者是膀胱发生黏液样变型的首例病例。既往所有膀胱MFH患者均有间歇性血尿,而该患者的主要症状是肿瘤延伸至前列腺导致膀胱出口梗阻。他接受了根治性膀胱前列腺切除术、术后对肿瘤床进行放射治疗以及使用阿霉素的辅助化疗。患者对治疗耐受性良好,在3年随访时无疾病复发。本文详细讨论了这种罕见膀胱肿瘤的组织发生、临床特征、病理特点及治疗要点。

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