起源于节细胞神经瘤的恶性外周神经鞘膜瘤临床病理观察

Malignant peripheral nerve sheath tumor arising from ganglioneuroma: a clinicopathological analysis and review of the literature

目的观察起源于节细胞神经瘤(GN)的恶性外周神经鞘膜瘤组织形态学特点,并探讨诊断与鉴别诊断。方法结合1例胸椎内起源于节细胞神经瘤的恶性外周神经鞘膜瘤(MPNST)的临床及影像学资料,观察镜下形态学特征及免疫组化结果,并进行相关文献复习。结果起源于节细胞神经瘤的恶性外周神经鞘膜瘤镜下可见典型的节细胞神经瘤成分,并逐渐过渡到恶性外周神经鞘膜瘤的形态特征,两者没有截然分界。免疫组化:肿瘤细胞vimentin、S-100和CD56(+),SMA、GFAP、EMA、CK、NF、CD34、desmin、Syn、Myo D1和myogenin(-)。结论节细胞神经瘤可以恶变为恶性外周神经鞘膜瘤。起源于节细胞神经瘤的恶性外周神经鞘膜瘤罕见,需仔细观察光镜下特殊的组织结构形态并结合免疫组化结果进行诊断,还需要与其他软组织恶性肿瘤进行鉴别诊断。

Object This report observed the microscopical histology of a malignant peripheral nerve sheath tumor arising from a ganglioneuroma（ MPNST ex ganglioneuroma）,and to explore the diagnosis and differential diagnosis with review the literature. Methods Combined with the clinical and imaging data of a malignant peripheral nerve sheath tumor arising from a ganglioneuroma（ MPNST ex ganglioneuroma） in thoracic cavity, we studied the microscopical histology,immunohistochemical staining,and reviewed the previously literatures. Results Microscopically,the tumor had typical components of the ganglioneuroma,and was seen transforming into MPNST. Immunohistochemically,the tumor components were positive for vimentin,S-100 and CD56,negative for SMA,GFAP,EMA,CK,NF,CD34,Desmin,Syn,Myo D1,and myogenin. Conclusion Despite of rarity,the MPNST can arise from the ganglioneuroma. We should observe the microscopical histology and Immunohistochemical staining for the diagnosis and differential diagnosis.