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胰腺尾部发生的类似于造釉细胞瘤样尤文肉瘤:一种罕见实体的病例报告并文献复习。

Adamantinoma-like ewing sarcoma arising in the pancreatic tail: a case report of a rare entity and review of the literature.

机构信息

Department of Pathology, The Eighth Affiliated Hospital, Sun Yat-sen University, Shenzhen, Guangdong, 518033, China.

Department of Pathology, Sun Yat-sen University Cancer Center, No. 651, Dongfeng Road East, Guangzhou, China.

出版信息

Diagn Pathol. 2023 Jul 31;18(1):86. doi: 10.1186/s13000-023-01374-0.

Abstract

ALES is a rare subtype that demonstrates the EWSR1-FLI1 translocation characteristic of ES and demonstrates complex epithelial differentiation including diffuse cytokeratin and p40 expression. It has predominantly recognized in the head and neck and is common in middle-aged population. This case is the first case of ALES reported in the pancreatic tail, sharing some morphological characteristics with ALES in the head and neck, including monotonous cytology, infiltrative growth pattern, and complex epithelioid differentiation, but ALES in the head and neck often has high-grade histological features (e.g., necrosis, high mitotic rate, etc.), and sudden keratinization can also occur, but these features were not reflected in this primary pancreatic tail ALES. Although ALES arising in the pancreatic tail and in the head and neck sites share the immunohistochemical and molecular profile, our case can provide new ideas in differential diagnosis of ALES arising in pancreatic tail and promote increased recognition and understanding of ALES.

摘要

ALES 是一种罕见的亚型,表现出 ES 特征的 EWSR1-FLI1 易位,并表现出复杂的上皮分化,包括弥漫性细胞角蛋白和 p40 表达。它主要发生在头颈部,常见于中年人群。本病例是首例报道的胰腺尾部 ALES,与头颈部的 ALES 具有一些形态学特征,包括单调的细胞学、浸润性生长模式和复杂的上皮样分化,但头颈部的 ALES 通常具有高级别的组织学特征(例如坏死、高有丝分裂率等),并且还可能突然出现角化,但这些特征在这个原发性胰腺尾部 ALES 中并未体现。尽管发生在胰腺尾部和头颈部的 ALES 具有相同的免疫组织化学和分子特征,但本病例可为胰腺尾部 ALES 的鉴别诊断提供新的思路,并促进对 ALES 的认识和理解。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/74ee/10388509/7712b8ae4910/13000_2023_1374_Fig1_HTML.jpg

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