文章：

非典型梭形细胞/多形性脂肪瘤性肿瘤：综述与更新

Atypical Spindle Cell/Pleomorphic Lipomatous Tumor: A Review and Update

原文发布日期：13 September 2024

DOI: 10.3390/cancers16183146

类型: Article

开放获取: 是

英文摘要：

Atypical spindle cell/pleomorphic lipomatous tumor (ASCPLT) is a rare and recently described adipocytic neoplasm that primarily occurs in the subcutis of the limbs and limb girdles, particularly of middle-aged adults. It has locally recurrent potential if incompletely excised but no risk for distant metastasis. ASCPLT is histologically similar to spindle cell/pleomorphic lipoma and atypical lipomatous tumor and shows a mixture of atypical spindle cells, adipocytes, lipoblasts, floret-like multinucleated giant cells, and/or pleomorphic cells. It has been recently recognized that ASCPLT can undergo sarcomatous transformation. However, the biological significance of morphological sarcomatous transformation in ASCPLT remains uncertain. Immunohistochemically, the tumor cells show variable expression of CD34, S-100 protein, and desmin. Loss of nuclear Rb expression is observed in the majority of cases. ASCPLT lacksMDM2gene amplification but can showRB1gene deletion in a significant subset of cases. Complete surgical excision is the treatment of choice. This review provides an overview of the current knowledge on the clinicoradiological features, pathogenesis, histopathology, and treatment of ASCPLT. In addition, we will discuss the differential diagnosis of this new entity.

摘要翻译： 

非典型梭形细胞/多形性脂肪瘤性肿瘤（ASCPLT）是一种罕见且新近描述的脂肪细胞性肿瘤，主要发生于四肢及肢带皮下组织，尤其多见于中年人群。若切除不彻底，该肿瘤具有局部复发潜力，但无远处转移风险。组织学上，ASCPLT与梭形细胞/多形性脂肪瘤及非典型脂肪瘤性肿瘤相似，表现为非典型梭形细胞、脂肪细胞、脂肪母细胞、花环状多核巨细胞和/或多形性细胞的混合存在。近期研究证实ASCPLT可发生肉瘤样转化，但其形态学肉瘤样转化的生物学意义尚不明确。免疫组化显示肿瘤细胞对CD34、S-100蛋白和结蛋白表达不一，多数病例可见核Rb表达缺失。ASCPLT不伴有MDM2基因扩增，但在相当比例病例中可检测到RB1基因缺失。完全手术切除是首选治疗方案。本文综述了当前对ASCPLT临床影像学特征、发病机制、组织病理学及治疗的认识，并将对这一新病种的鉴别诊断进行探讨。

原文链接：

Atypical Spindle Cell/Pleomorphic Lipomatous Tumor: A Review and Update