Thway Khin, Fisher Cyril
Department of Histopathology, Sarcoma Unit, Royal Marsden NHS Foundation Trust, 203 Fulham Road, London SW3 6JJ, UK.
Department of Histopathology, Sarcoma Unit, Royal Marsden NHS Foundation Trust, 203 Fulham Road, London SW3 6JJ, UK.
Ann Diagn Pathol. 2014 Apr;18(2):109-16. doi: 10.1016/j.anndiagpath.2013.10.007. Epub 2013 Nov 23.
Malignant peripheral nerve sheath tumors are soft tissue neoplasms that show differentiation toward cells of the nerve sheath. They often arise from peripheral nerves or preexisting benign nerve sheath tumors and are generally high-grade neoplasms, which behave aggressively with high incidence of distant metastases. Malignant peripheral nerve sheath tumor can be histologically diverse and is difficult to diagnose because of its morphological overlap with a variety of other sarcomas and its lack of specific immunohistochemical markers or genetic profile. We review the pathology of malignant peripheral nerve sheath tumor, with reference to etiology, molecular genetics, and clinical factors.
恶性外周神经鞘瘤是一种软组织肿瘤，表现出向神经鞘细胞分化的特征。它们通常起源于外周神经或先前存在的良性神经鞘瘤，一般为高级别肿瘤，具有侵袭性，远处转移发生率高。恶性外周神经鞘瘤在组织学上具有多样性，由于其与多种其他肉瘤在形态上有重叠，且缺乏特异性免疫组化标志物或基因特征，因此难以诊断。我们参考病因、分子遗传学和临床因素，对恶性外周神经鞘瘤的病理学进行综述。
