Verma Anuj, Rekhi Bharat
Department of Surgical Pathology, Tata Memorial Hospital, Parel, Mumbai, India.
Histol Histopathol. 2017 Sep;32(9):861-877. doi: 10.14670/HH-11-879. Epub 2017 Feb 1.
Myoepithelial tumor (MET) of soft tissue and bone is an unusual tumor of uncertain differentiation and histogenesis, but lately has been recognized as a distinct tumor entity. This tumor forms a morphologic continuum with a mixed tumor and a parachordoma, but is different from an extra-axial chordoma or chordoma periphericium. METs display a range of histopathologic features, including architectural arrangements/growth patterns, cell types and intervening stroma, leading to their several differential diagnoses. Presently, moderate nuclear atypia is the acceptable criterion to differentiate a myoepithelial carcinoma from a myoepithelioma. Immunohistochemical (IHC) stains, including epithelial antibody markers, along with S100 protein and GFAP are necessary in confirming a diagnosis of a MET. Lately, certain specific "molecular signatures" been described underlying METs, identification of which that can further aid in their accurate diagnosis and in differentiating these tumors from their diagnostic mimics. Complete surgical resection forms the treatment mainstay, irrespective of a myoepithelioma or a myoepithelial carcinoma. This review will focus upon clinicopathologic, immunohistochemical and molecular features of METs of soft tissue and bone, along with their differential diagnoses and diagnostic implications.
软组织和骨的肌上皮肿瘤(MET)是一种分化和组织发生不确定的罕见肿瘤,但最近已被确认为一种独特的肿瘤实体。该肿瘤与混合瘤和副脊索瘤形成形态学连续谱,但不同于轴外脊索瘤或外周脊索瘤。MET表现出一系列组织病理学特征,包括结构排列/生长模式、细胞类型和间质,这导致了它们的多种鉴别诊断。目前,中度核异型性是区分肌上皮癌和肌上皮瘤的可接受标准。免疫组织化学(IHC)染色,包括上皮抗体标志物以及S100蛋白和GFAP,对于确诊MET是必要的。最近,已经描述了METs潜在的某些特定“分子特征”,识别这些特征可以进一步帮助准确诊断这些肿瘤,并将它们与诊断上的相似肿瘤区分开来。无论肿瘤是肌上皮瘤还是肌上皮癌,完整的手术切除都是主要的治疗方法。本综述将重点关注软组织和骨METs的临床病理、免疫组织化学和分子特征,以及它们的鉴别诊断和诊断意义。