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姓 名: | ××× | 性别: | 男 | 年龄: | 39 |
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简要病史: | 右胸壁肿物一年余,大小:3X2CM。 | ||||
肉眼检查: | 灰白灰褐色不整形软组织数块,大小:2X2X1.3CM |
不管是从免疫组化结果还是形态学上,都考虑为低度恶性隆皮纤肉瘤。
对于DFSP的起源有学者认为起源于表达CD34的皮肤树突状细胞或者其亚型,或者DFSP可能是某种特殊类型的表达CD34的神经鞘瘤(May be a peculiar type of nerve sheath tumor since CD34 positive, or may derive from a subset of CD34 positive dermal dendritic cells )。本例CD34(+++),GFAP(+),或许更支持这种说法。
而MPNST(恶性外周神经鞘膜瘤),多发于头颈部及前臂,多数生长于大神经周围体积一般较大可见显著的出血和坏死等,发生于体表皮下者相对少见(至于伴有神经纤维瘤病恶变的病例另论)。本例免疫组化S-100(-)也不支持此诊断。
Bulky deep-seated tumor usually arising from major nerves in neck, forearm, lower leg, buttock。
Gross: large mass producing a fusiform enlargement of a major nerve (often sciatic)Micro: monomorphic serpentine cells, palisading, large gaping vascular spaces, perivascular plump tumor cells, geographic necrosis with tumor palisading at the edges (resembles glioblastoma multiforme),frequent mitotic figures; may have bizarre cells; 15% have metaplastic cartilage, bone, muscle。
Positive stains: S100 (62%), Leu7/CD57 (in neurofibroma-like areas), p53, CD57 (55%), collagen IV, CD99/O13 (86%), protein gene product 9.5,{but PGP9.5 not specific)
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