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成人多形性肥大细胞瘤。

Pleomorphic mastocytoma in an adult.

作者信息

Exposito-Serrano Vicente, Agut-Busquet Eugenia, Leal Canosa Lorena, Herrerías Moreno Jose, Saez Amparo, Luelmo Jesús

机构信息

Department of Dermatology, Hospital Universitari Parc Taulí, Sabadell, Spain.

Department of Pathology, Hospital Universitari Parc Taulí, Sabadell, Spain.

出版信息

J Cutan Pathol. 2018 Feb;45(2):176-179. doi: 10.1111/cup.13080. Epub 2017 Dec 11.

Abstract

Cutaneous mastocytoma (CM) is a localized variant of mastocytosis, characterized by an over-accumulation of mast cells in the skin, without extra-cutaneous organ involvement. It is defined as the presence of up to 3 isolated mast-cell skin lesions and commonly develops in newborns and children. We report the case of a 35-year-old healthy Caucasian woman presenting with a 4-year history of a pruritic brown plaque on her left breast. Hematoxylin-eosin staining showed a dense dermal infiltrate of atypical mast cells extending to the subcutis. The cells presented a marked nuclear pleomorphism with bilobed and multilobed nuclei. Immunohistochemical studies revealed strongly expressed KIT (CD117) and CD25 proteins. Serum tryptase levels and bone marrow biopsy were normal. The diagnosis was a solitary cutaneous pleomorphic mastocytoma. This case can be added to 17 other cases of adult mastocytoma documented in the literature, although, unlike other reported cases, and as far as we are aware, this is the first case of pleomorphic mastocytoma in an adult.

摘要

皮肤肥大细胞瘤(CM)是肥大细胞增多症的一种局限性变体,其特征是皮肤中肥大细胞过度积聚,不累及皮肤外器官。它被定义为存在多达3个孤立的肥大细胞皮肤病变,常见于新生儿和儿童。我们报告了一例35岁健康的白种女性病例,其左乳出现瘙痒性棕色斑块已有4年病史。苏木精-伊红染色显示非典型肥大细胞密集浸润真皮并延伸至皮下组织。细胞呈现明显的核多形性,有双叶和多叶核。免疫组织化学研究显示KIT(CD117)和CD25蛋白强烈表达。血清类胰蛋白酶水平和骨髓活检正常。诊断为孤立性皮肤多形性肥大细胞瘤。该病例可补充到文献记载的其他17例成人肥大细胞瘤病例中,不过,与其他报道的病例不同,据我们所知,这是成人多形性肥大细胞瘤的首例。

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