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目的探讨伴有多向分化的恶性外周神经鞘瘤(MPNST)的临床病理特征及诊断依据。方法对1例79岁男性患者的左大腿软组织肿瘤进行组织形态学观察及免疫组化检测,结合文献对其病理组织学特征进行分析。结果本例MPNST肿瘤的组织形态复杂多样,在梭形细胞背景上见明确的腺样结构、神经内分泌分化伴有菊形团形成、纤维组织细胞样、软骨、横纹肌母细胞和脂肪肉瘤样分化及色素细胞和神经节细胞分化。结论单纯用其他肿瘤来命名不能囊括如此复杂多样的组织形态,因此最后诊断为MPNST伴多向分化。此外,在同一MPNST中同时见到如此多的形态文献尚未见报道。
Objective To investigate the clinicopathological features and diagnosis of malignant peripheral nerve sheath tumors (MPNST) with multidirectional differentiation. Methods The histomorphology and immunohistochemistry of left thigh soft tissue tumor in a 79-year-old male patient were analyzed. The histopathological features were analyzed based on the literature. Results The morphological changes of MPNST tumors in this case were complex and diverse, with clear adenoid structures on the spindle cells background, neuroendocrine differentiation accompanied by the formation of chrysoid, fibrous tissue-like cells, cartilage, rhabdomyoblast and liposarcoma Pigment cells and ganglion cells differentiate. Conclusions The naming of other tumors can not encompass such complex and diverse forms of tissue, so MPNST with multidirectional differentiation was finally diagnosed. In addition, there have been no reports of so many morphological studies in the same MPNST.