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一名表现为福尼尔坏疽的患者阴囊多形性恶性纤维组织细胞瘤/未分化高级别多形性肉瘤:病例报告

Pleomorphic malignant fibrous histiocytoma/undifferentiated high-grade pleomorphic sarcoma of the scrotum in a patient presenting as fournier gangrene: a case report.

作者信息

Guo Juan, Zhou Shengmei, Rao Nagesh P, Pez Gholam H

机构信息

Department of Pathology, Keck School of Medicine, LAC and USC Medical Center, University of Southern California, CA, USA.

出版信息

Appl Immunohistochem Mol Morphol. 2010 Oct;18(5):473-8. doi: 10.1097/PAI.0b013e3181de57f6.

Abstract

Pleomorphic malignant fibrous histiocytoma (MFH), also known as undifferentiated high-grade pleomorphic sarcoma according to the latest World Health Organization classification, is a diagnosis of exclusion and extremely rare in adult scrotal/paratesticular region. Clinical presentation of scrotal/paratesticular pleomorphic MFH is usually a painless and gradual scrotal swelling. We report a case of scrotal MFH in a 63-year-old man who presented as Fournier gangrene after 10-month painful scrotal swelling and multiple procedures. The specimen of emergent debridement was submitted for pathologic and bacteriologic examination. Microscopically, the lesion had marked architectural and cytologic pleomorphism. The neoplastic cells were positive for vimentin, but negative for all lineage-specific markers. Fluorescence in-situ hybridization showed an aneuploid karyotype and negative results for lipomatous tumor abnormalities. Bacterial cultures of the specimen showed extensive growth of virulent polymicrobes. The diagnosis of scrotal/paratesticular pleomorphic MFH with concurrent Fournier gangrene was made. Thoracic computed tomography scan showed bilateral multiple pulmonary nodules. The patient died 1 month later.

摘要

多形性恶性纤维组织细胞瘤(MFH),根据世界卫生组织最新分类也被称为未分化高级别多形性肉瘤,是一种排除性诊断,在成人阴囊/睾丸旁区域极为罕见。阴囊/睾丸旁多形性MFH的临床表现通常是无痛性且逐渐加重的阴囊肿胀。我们报告一例63岁男性阴囊MFH病例,该患者在经历10个月的阴囊疼痛肿胀及多次手术后出现福尼尔坏疽。紧急清创标本送病理和细菌学检查。显微镜下,病变具有明显的结构和细胞多形性。肿瘤细胞波形蛋白阳性,但所有谱系特异性标志物均为阴性。荧光原位杂交显示非整倍体核型,脂肪瘤异常结果为阴性。标本细菌培养显示有大量毒力强的多种微生物生长。诊断为阴囊/睾丸旁多形性MFH并发福尼尔坏疽。胸部计算机断层扫描显示双侧多发肺结节。患者1个月后死亡。

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