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手指炎性肌纤维母细胞瘤的罕见表现。

Rare presentation of inflammatory myofibroblastic tumor on a digit.

机构信息

ProPath, Dallas, Texas, USA.

Department of Dermatology, University of North Texas Health Science Center, Ft. Worth, Texas, USA.

出版信息

J Cutan Pathol. 2021 Jul;48(7):965-968. doi: 10.1111/cup.14016. Epub 2021 Apr 17.

Abstract

Inflammatory myofibroblastic tumors (IMTs) are rare soft tissue neoplasms consisting of a mixture of spindle-shaped myofibroblasts or fibroblasts and a variable inflammatory infiltrate composed of eosinophils, plasma cells, and lymphocytes. Associations with trauma and infectious agents have been proposed, but the etiology is unknown. While IMT predominantly develops in the lungs of pediatric patients or young adults, extrapulmonary IMT is well documented and may occur anywhere. Cutaneous IMT is rare and few have been reported on the hand in the English language. The mean age of onset is 10 years, with a slight female predilection. IMT demonstrates intermediate malignant potential, with a tendency to recur locally. Metastases are rare. According to a recent review, anaplastic lymphoma kinase (ALK) positivity on immunohistochemistry is related to local recurrence, but not distant metastases. We report an unusual case of a 36-year-old male, with a lesion on the right second digit, displaying classic histopathologic and immunohistochemical features of IMT, including ALK staining, and confirmatory fluorescence in situ hybridization-proven ALK gene rearrangement.

摘要

炎性肌纤维母细胞瘤(IMTs)是一种罕见的软组织肿瘤,由梭形的肌纤维母细胞或成纤维细胞与不同程度的炎症浸润混合组成,其中包括嗜酸性粒细胞、浆细胞和淋巴细胞。有人提出其与创伤和感染因子有关,但病因尚不清楚。虽然 IMT 主要发生在儿童或年轻成人的肺部,但也有记录表明其也可发生在肺外,且可发生于任何部位。皮肤 IMT 罕见,英文文献中报道的手部 IMT 更是寥寥无几。该病的发病平均年龄为 10 岁,女性略多见。IMT 具有中等恶性潜能,倾向于局部复发,转移少见。根据最近的一项综述,免疫组织化学ALK 阳性与局部复发相关,而与远处转移无关。我们报告了 1 例不常见的病例,患者为 36 岁男性,右手第 2 指有一病变,具有 IMT 的典型组织病理学和免疫组织化学特征,包括 ALK 染色,荧光原位杂交证实存在 ALK 基因重排。

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