Imaging and Clinical Findings of Primary Malignant Fibrous Histiocytoma of the Urinary Bladder: A Case Report.

Journal of the Korean Society of Radiology Pub Date : 2024-05-01 Epub Date: 2024-01-15 DOI:10.3348/jksr.2023.0083
Yoon Jung Lee, Eun Ji Lee, Jae Heon Kim, So Young Jin, Seong Sook Hong, Jiyoung Hwang, Yun-Woo Chang
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Abstract

Primary malignant fibrous histiocytoma (MFH) is a malignant tumor of mesenchymal origin that rarely occurs in the urinary tract, particularly in the urinary bladder. Unlike urothelial carcinoma, which accounts for most bladder cancers, it occurs in the submucosal portion of the bladder wall and consists of the lamina propria, muscularis propria, and adventitia. It is presumed to originate from poorly differentiated pluripotent mesenchymal cells in which fibroblasts and histiocytes are partially differentiated. Radiologically, it is known as the "non-papillary tumor" and is commonly diagnosed as a large mass without necrosis, which shows invasion beyond the muscularis propia. Although the prognosis of this rare malignancy depends on pathological parameters, it generally has a poor prognosis with high local tumor recurrence. Here, we present a case of primary MFH in the urinary bladder with clinical symptoms of lower abdominal pain without gross hematuria that recurred rapidly and showed an aggressive disease course.

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膀胱原发性恶性纤维组织细胞瘤的影像学和临床发现:病例报告。
原发性恶性纤维组织细胞瘤(MFH)是一种间质来源的恶性肿瘤,很少发生在泌尿道,尤其是膀胱。与大多数膀胱癌中的尿路上皮癌不同,它发生在膀胱壁的粘膜下部分,由固有层、固有肌和固有膜组成。据推测,它起源于分化不良的多能间充质细胞,其中成纤维细胞和组织细胞已部分分化。在放射学上,它被称为 "非乳头状肿瘤",通常被诊断为无坏死的大肿块,其侵袭范围超过固有肌。虽然这种罕见恶性肿瘤的预后取决于病理参数,但一般预后较差,局部肿瘤复发率较高。在此,我们介绍了一例原发性膀胱MFH患者,其临床症状为下腹痛,无大血尿,复发迅速,病程凶险。
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