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目的探讨血管肌纤维母细胞瘤(AMF)的临床病理学特点及诊断和鉴别诊断要点。方法采用HE及免疫组化方法,分析5例发生于外阴及其他部位的血管肌纤维母细胞瘤。结果 5例AMF中2例位于外阴,1例位于阴道,1例位于足底,1例位于腹股沟。镜下见肿瘤境界清楚,在大片黏液背景中见丰富薄壁的海绵样血管和梭形上皮样肿瘤细胞。瘤细胞分布有明显的疏密区,密集区瘤细胞聚集于血管周围,成束或链状排列;疏松区弥散于黏液背景中,且常与胶原纤维伴行。免疫组化:肿瘤细胞vimentin和desmin(+),CD34、actin、CD117、S-100、CD31和CK(-);发生于外阴和阴道的3例ER和PR(+)。术后随访3例,均无复发。结论血管肌纤维母细胞瘤是一种好发于女性外阴部的良性软组织肿瘤,局部切除可治愈,但也有少数复发病例报道,组织学上需与侵袭性血管黏液瘤、富于细胞的血管纤维瘤、纤维上皮性间质息肉、上皮样平滑肌瘤、栅栏状肌纤维母细胞瘤等鉴别。
Objective To investigate the clinicopathological characteristics of vascular myofibroblastic tumor (AMF) and its diagnostic and differential diagnosis. Methods HE and immunohistochemical methods were used to analyze 5 cases of vascular myofibroblastoma in the vulva and other parts. Results In 5 AMF cases, 2 cases were located in the vulva, 1 case was located in the vagina, 1 case was located in the plantar, and 1 case was located in the groin. Microscopically, the tumor was clear, with rich, thin-walled cavernous vasculature and spindle-like epithelioid tumor cells in the bulk of mucus. Tumor cells have obvious density distribution, dense area of tumor cells gathered in the perivascular, bundles or chains arranged; loose area dispersed in the mucus background, and often accompanied by collagen fibers. Immunohistochemistry: The tumor cells vimentin and desmin (+), CD34, actin, CD117, S-100, CD31 and CK (-); 3 cases of ER and PR (+) occurred in the vulva and vagina. Three patients were followed up, no recurrence. Conclusions Vascular myofibroblastoma is a benign soft tissue tumor appearing in the vulva of the female. Local excision can be cured, but there are also a few cases of recurrence. The histology is associated with aggressive vascular myxoma, rich cell angiofibroma , Fibrous epithelial mesenchymal polyps, epithelioid leiomyomata, palisade myofibroblastic tumor identification.