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皮肤黏液纤维肉瘤:临床病理特征及与皮肤黏液样肿瘤的鉴别诊断

Myxofibrosarcoma presenting in the skin: clinicopathological features and differential diagnosis with cutaneous myxoid neoplasms.

作者信息

Mansoor Atiya, White Clifton R

机构信息

Department of Pathology, Oregon Health Sciences University, Portland, 97201, USA.

出版信息

Am J Dermatopathol. 2003 Aug;25(4):281-6. doi: 10.1097/00000372-200308000-00001.

Abstract

Myxofibrosarcoma (myxoid malignant fibrous histiocytoma) is one of the most common fibroblastic sarcomas in the older patient, where it can sometimes present with anatomically deceptive boundaries. Myxofibrosarcoma is now fully characterized as a distinctive and definable pathologic entity. Clinically there is a tendency for predominantly subcutaneous, multinodular, diffusely infiltrative growth, which may extend to the overlying dermis and present as a cutaneous lesion. Histologically myxofibrosarcoma comprises a spectrum ranging from hypocellular low-grade myxoid to high-grade pleomorphic sarcoma. We report herein 6 cases of myxofibrosarcoma each with dermatological presentation as a cutaneous nodule. The dermal component in each of the lesions was low- to intermediate-grade and predominantly myxoid resulting in confusion with benign myxoid neoplasms in small biopsy specimens. The purpose of this series is to focus the attention of workers in dermatology on a subject rarely discussed in dermatopathology literature: the cutaneous presentation of myxofibrosarcoma and the potential for clinical and histologic misinterpretation, as benign dermal lesions.

摘要

黏液纤维肉瘤(黏液样恶性纤维组织细胞瘤)是老年患者中最常见的纤维母细胞肉瘤之一,有时其边界在解剖学上具有欺骗性。黏液纤维肉瘤现已被完全确认为一种独特且可定义的病理实体。临床上,它倾向于主要在皮下呈多结节、弥漫性浸润性生长,可延伸至上方的真皮并表现为皮肤病变。组织学上,黏液纤维肉瘤包括从细胞稀少的低级别黏液样到高级别多形性肉瘤的一系列表现。我们在此报告6例黏液纤维肉瘤,每例均表现为皮肤结节的皮肤病学表现。每个病变中的真皮成分均为低级别至中级别,且主要为黏液样,这在小活检标本中易与良性黏液样肿瘤混淆。本系列研究的目的是让皮肤科医生关注皮肤病理学文献中很少讨论的一个主题:黏液纤维肉瘤的皮肤表现以及作为良性皮肤病变出现临床和组织学误诊的可能性。

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