文章：

角蛋白阳性巨细胞丰富型肿瘤：综述与更新

Keratin-Positive Giant Cell-Rich Tumor: A Review and Update

原文发布日期：20 May 2024

DOI: 10.3390/cancers16101940

类型: Article

开放获取: 是

英文摘要：

Keratin-positive giant cell-rich tumor (KPGCT) is an extremely rare and recently described mesenchymal neoplasm that occurs in both soft tissue and bone, frequently found in young women. It has locally recurrent potential if incompletely excised but low risk for metastasis. KPGCT is histologically similar to conventional giant cell tumors of soft tissue but shows the presence of keratin-positive mononuclear cells. Interestingly, KPGCT also shares some morphological features with xanthogranulomatous epithelial tumors. These two tumors have recently been shown to harbor anHMGA2–NCOR2fusion, arguing in favor of a single entity. Surgery is the treatment of choice for localized KPGCT. Therapeutic options for advanced or metastatic disease are unknown. This review provides an overview of the current knowledge on the clinical presentation, pathogenesis, histopathology, and treatment of KPGCT. In addition, we will discuss the differential diagnosis of this emerging entity.

摘要翻译： 

角蛋白阳性富于巨细胞肿瘤（KPGCT）是一种极为罕见且新近描述的间叶源性肿瘤，可发生于软组织和骨骼，常见于年轻女性。若切除不彻底，该肿瘤具有局部复发倾向，但转移风险较低。组织学上，KPGCT与传统的软组织巨细胞瘤相似，但存在角蛋白阳性的单核细胞。值得注意的是，KPGCT亦与黄色肉芽肿性上皮肿瘤共享部分形态学特征。近期研究表明这两种肿瘤均携带HMGA2-NCOR2基因融合，支持其为同一疾病实体。手术是局限性KPGCT的首选治疗方案，晚期或转移性疾病的治疗策略尚不明确。本综述概述了目前对KPGCT临床表现、发病机制、组织病理学及治疗的认识，并将对这一新兴病种的鉴别诊断进行探讨。

原文链接：

Keratin-Positive Giant Cell-Rich Tumor: A Review and Update