侵袭性血管黏液瘤7例临床病理分析

Aggressive anginomyxoma:7 cases report and review of literature

目的 探讨侵袭性血管黏液瘤（aggressive anginomyxoma，AAM）的临床病理特征与鉴别诊断。方法收集7例AAM，并与2例血管肌纤维母细胞瘤（AMFb）、1例浅表性血管黏液瘤作对照观察。免疫组织化学（采用SP法）所用抗体为vimentin、desmin、SMA、MSA、CD34、ER／PR、S-100蛋白、CK和CD68。结果 7例AAM患者均为女性，23～54岁，中位年龄43．5岁。病变部位位于外阴部3例，累及阴道1例，位于盆腔及髂窝部2例，会阴累及坐骨结节1例。肿瘤直径3．5～17cm，平均7．5cm。位于皮下或较深部，呈浸润性生长，切面均质灰白或灰黄褐色，黏液胶冻样或肉质样。镜下瘤细胞幼稚，呈星形、梭形，弥漫或结节样分布，基质黏液样变性并见胶原纤维及为数众多的大小厚薄不一的血管分布其中，厚壁血管常透明变性，其周围常有增生肌纤维束，切面呈“袖套状”，间质可见肥大细胞和红细胞外渗，1例可见多核巨细胞，肿瘤边缘浸润等特点。组织化学奥辛蓝染色阳性，免疫组化：7例AAM vimentin均阳性，5例SMA和desmin阳性，4例MSA阳性，3例CD34阳性，4例中有3例ER／PR阳性，而S-100蛋白、CK、CD68均阴性，多核巨细胞CD68阳性。5例有随访，术后1—2年有3例复发，2例未见复发和转移，健在。结论 侵袭性血管黏液瘤较为少见，好发于成年女性盆腔及会阴的软组织，侵袭性和复发性是其重要的临床特征。病理诊断易误诊或漏诊。免疫组化desmin、SMA、MSA、CD34等联合检测对确定AAM病变有所帮助，但与AMFb、浅表性血管黏液瘤在鉴别诊断上意义不大。

Purpose To study the clinicopathologic features and differential diagnosis of aggressive anginomyxoma （AAM）. Methods Seven cases of AAM were collected, and other two cases of angiomyofiblastoma（AMFb） and one superficial angiomyxoma（SA） were also studied as control. All the tumors were examined by microscopy and immunohistochemistry. Results Seven patients with AAM were females, aged 23 to 54 years, with median age of 43.5 years. 3 cases occurred in the vulva, 1 case in vagina, 2 cases in pelvic and perineal and 1 case in sciatic region. AAM tended to be large, ranging in size from 3.5cm to 17 cm（median 7. 5 cm）. AAM located in subcutaneous tissues or deeply soft tissues, with invasive growth pattern. The cut surface was gray or tan, with mucoid and gelatinous. Microscopically ： the tumor cells of AAM were infantile, spindle or stellate, diffuse and nodular arrangement. A distinctive histologic feature of AAM was its vaseularity. Non-arborizing, thin-wall, ectastic capillaries or, more commonly, small thick-wall vessels were dispersed throughout the tumor. The latter feature was due either to thick smooth muscle in the walls of the vessels or to their investiture by periadventitial condensation of eosinophilic fibrillar material. Mast cells and extravasated red blood cells were frequently found in the stroma, and multinuclear cells appeared in 1 case. Histochemical staining （Alcain blue） was positive. Immunohistochemical study showed that 7 cases were positive for vimentin, 5 for SMA and desmin, 4 for MSA, and 3 for CD34. 3 of 4 cases were positive for ER/PR and negative for S-100, CK or CD68, but multinuclear cell was positive for CD68. 5 cases were followed up, and the results showed that 3 cases were local recurrence, and 2 cases without recurrence and metastases. Conclusions AAM is a rare soft tumor. The major sites of involvement are vulva andpelvis floor. Misdiagnosis has happened frequently. Immunohistochemical staining of desmin, SMA, MSA and CD34 are helpful to diagnosis for AAM, but no significance to distinguish it from AMFb and SA.