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扩展皮肤上皮样肿瘤的鉴别诊断:皮肤上皮样分化的去分化脂肪肉瘤 1 例,并复习文献。

Expanding the differential of cutaneous epithelioid tumors: A case of dedifferentiated liposarcoma with epithelioid features involving the skin, with review of the literature.

机构信息

Geisel School of Medicine at Dartmouth, Hanover, New Hampshire.

Department of Pathology and Laboratory Medicine, Dartmouth-Hitchcock Medical Center, Lebanon, New Hampshire.

出版信息

J Cutan Pathol. 2020 Jun;47(6):554-560. doi: 10.1111/cup.13650. Epub 2020 Feb 11.

Abstract

Liposarcomas are categorized into four distinct histopathological subtypes: atypical lipomatous tumors (ALT)/well-differentiated liposarcoma (WDL), dedifferentiated, myxoid, and pleomorphic. Dedifferentiated liposarcomas account for approximately 18% of all liposarcomas, characteristically arising in the deep soft tissue. They are reported to have lower rates of metastasis compared to other pleomorphic sarcomas. The classic histopathologic appearance is ALT/WDL admixed or juxtaposed with a predominantly nonlipogenic sarcoma. Epithelioid features are rare, appearing in as few as 3% of tumors, and have not previously been reported in a superficial location. Herein, we present a 57-year-old male with intradermal and subcutaneous metastasis of his known deep dedifferentiated liposarcoma with epithelioid features. By H&E the tumor featured cords and sheets of crowded, plump, epithelioid cells with thick nuclear membranes and prominent nucleoli, which raised a broad differential including carcinoma and melanoma. By immunohistochemistry the tumor was diffusely positive for MDM2 and CDK4, on the other hand stains for Sox10, Melan A, MITF, CKAE1/3, desmin, and S100 protein were negative. This case serves as an opportunity to raise awareness of this rare morphological subtype, which can involve the skin and mimic epithelial and melanocytic malignancies. It can be a potential diagnostic pitfall, especially if metastases are the first presentation.

摘要

脂肪肉瘤分为四个不同的组织病理学亚型

非典型性脂肪肉瘤(ALT)/高分化脂肪肉瘤(WDL)、去分化型、黏液样和多形性。去分化型脂肪肉瘤约占所有脂肪肉瘤的 18%,典型发生于深部软组织。与其他多形性肉瘤相比,其转移率较低。经典的组织病理学表现为 ALT/WDL 与主要非脂源性肉瘤混合或并列。上皮样特征很少见,出现在不到 3%的肿瘤中,以前从未在浅表部位报道过。在此,我们报告了一名 57 岁男性,其已知的深部去分化脂肪肉瘤伴上皮样特征发生了真皮内和皮下转移。通过 H&E,肿瘤具有密集、饱满的上皮样细胞条索和片状,核膜厚,核仁明显,广泛的鉴别诊断包括癌和黑色素瘤。通过免疫组织化学,肿瘤弥漫性表达 MDM2 和 CDK4,另一方面,Sox10、Melan A、MITF、CKAE1/3、结蛋白和 S100 蛋白染色均为阴性。该病例为认识这种罕见的形态学亚型提供了机会,这种亚型可累及皮肤并模拟上皮和黑色素细胞恶性肿瘤。如果转移是首发表现,这可能是一个潜在的诊断陷阱。

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