Nielsen G P, Rosenberg A E, Young R H, Dickersin G R, Clement P B, Scully R E
James Homer Wright Pathology Laboratories, Massachusetts General Hospital, Harvard Medical School, Boston, USA.
Mod Pathol. 1996 Mar;9(3):284-91.
Nine vulvar and three vaginal angiomyofibroblastomas from patients 23 to 71 years of age (mean, 46 yr) were analyzed. The tumors were well circumscribed and ranged from 0.9 to 11 cm (average, 4.7 cm) in maximal dimension. On microscopic examination, they had hypercellular and hypocellular areas. The neoplastic cells were spindle-shaped, plasmacytoid, or epithelioid; a variable number were binucleated or multinucleated cells. A focal storiform pattern was present in one tumor, and, in one tumor, the neoplastic cells formed a collar around a central area of dense collagen. There was no significant nuclear atypia, and there was less than one mitotic figure per 10 high-power fields. The tumors contained small- to medium-sized blood vessels, which were characteristically thin walled and, occasionally, ectatic and branching. The stroma was edematous, separated collagen fibers and contained a variable number of inflammatory cells, especially lymphocytes and mast cells. Three vulvar tumors contained a variable amount of fat. Ultrastructural study of three tumors showed intracytoplasmic, dilated, rough endoplasmic reticulum, moderate numbers of pinocytotic vesicles, and numerous filaments without dense bodies; rare intercellular rudimentary junctions were identified. Eleven of 11 tumors were immunoreactive for vimentin, 11 of 12 for desmin, three of 11 for muscle actin, one of 12 for smooth muscle actin, and four of 12 for CD34. There was no staining for factor XIIIa, keratin, S100 protein, Leu-7, glial fibrillary acidic protein, or CD68. Follow-up revealed no recurrences or metastases. Angiomyofibroblastoma is a distinctive benign tumor that arises most commonly in the vulva and vagina and has a diverse histologic and immunohistochemical profile.
对9例来自23至71岁（平均46岁）患者的外阴血管肌纤维母细胞瘤和3例阴道血管肌纤维母细胞瘤进行了分析。肿瘤边界清楚，最大直径为0.9至11厘米（平均4.7厘米）。显微镜检查显示，肿瘤有细胞丰富区和细胞稀少区。肿瘤细胞呈梭形、浆细胞样或上皮样；有数量不等的双核或多核细胞。1例肿瘤出现局灶性车辐状结构，1例肿瘤的肿瘤细胞围绕中央致密胶原区域形成环状结构。无明显核异型性，每10个高倍视野中核分裂象少于1个。肿瘤内含有中小血管，其特征为壁薄，偶尔扩张并分支。间质水肿，胶原纤维分离，含有数量不等的炎症细胞，尤其是淋巴细胞和肥大细胞。3例外阴肿瘤含有数量不等的脂肪。对3例肿瘤进行超微结构研究显示，胞质内有扩张的粗面内质网、中等数量的吞饮小泡和大量无致密体的细丝；可见罕见的细胞间原始连接。11例肿瘤中有11例波形蛋白免疫反应阳性，12例中有11例结蛋白免疫反应阳性，11例中有3例肌动蛋白免疫反应阳性，12例中有1例平滑肌肌动蛋白免疫反应阳性，12例中有4例CD34免疫反应阳性。因子ⅩⅢa、角蛋白、S100蛋白、Leu-7、胶质纤维酸性蛋白或CD68均无染色。随访未发现复发或转移。血管肌纤维母细胞瘤是一种独特的良性肿瘤，最常见于外阴和阴道，具有多样的组织学和免疫组化特征。
