侵袭性血管黏液瘤是一种少见的间叶性肿瘤,为探讨其临床病理学特征、诊治及预后,本研究收集北京大学国际医院4例盆腹腔软组织侵袭性血管黏液瘤的临床影像资料,分析其病理学形态、免疫表型及治疗随访情况,并回顾相关文献。本组4例均为女性,年龄27~49岁,平均年龄33岁,无诱因腹胀或无明显不适经体检发现,3例原发、1例复发。3例CT及磁共振扫描可见分层/漩涡征。肿瘤主体均位于盆腔,1例同时累及腹腔。肿瘤最大径5~22 cm,切面灰白、灰黄色,实性,质软,灶性水肿或囊性改变。显微镜下瘤细胞短梭形,异型不明显,间质富疏松水肿黏液或胶原,有丰富的薄壁及厚壁血管,部分血管壁玻璃样变性。肿瘤可浸润周围脂肪、神经组织。免疫组织化学染色提示4例肿瘤均雌激素受体、孕激素受体、结蛋白、平滑肌肌动蛋白弥漫阳性,CD34仅血管表达,S-100蛋白、CD117、Dog1均阴性。手术完整切除肿瘤,术后随访有1例二次复发。侵袭性血管黏液瘤确诊以病理形态学为基础,辅以免疫组织化学进一步诊断及鉴别诊断;手术切除后可多次复发。

Aggressive angiomyxoma is a rare mesenchymal tumor. To discuss the clinicopathological characteristics, treatment and prognosis of aggressive angiomyxoma, four cases of aggressive angiomyxoma of soft tissue in abdominopelvic cavity were collected from January 2015 to August 2017 in Peking University International Hospital. The clinical data, imaging examination, histopathological features, immunophenotype, therapy and prognosis were analysed. The related literatures were reviewed. All of the patients were adult females, age range from 27 to 49 years and mean 33 years. The clinical complaint was abdominal distention with no definite predisposing factor, or occasional physical-exam finding with no obvious discomfort. Three cases were primary and one case was recurrent. Typical layered or swirled structural sign was presented by CT and MRI scanning of three cases. All tumors located in the pelvic cavity, and attached to the uterus, vagina, rectum, bladder or ureter. One case was involved in the abdominal cavity simultaneously,adhesive to the spine, inferior vena cava and spleen. The gross appearance of tumors was from 5 to 22 cm in maximum diameter. The sectioned surfaces were soft, solid, white or yellow-gray, focally accompanied by edema, mucoid degeneration or cystic change. Microscopic observation showed that tumor cells were short spindle shaped and little atypical, the stroma was loose like ede-matous mucus or collagen, and the vessels were rich in thin and thick-wall. Partially the vessel wall expressed hyaline degeneration. Also tumors might infiltrate surrounding tissue, such as fat or nerve. The immunohistochemistry results of all cases were estrogen receptor and progesterone receptor diffusely mo-derate positive, Desmin and smooth muscle actin mostly positive, whereas CD34 expressed only in vessel and S-100 protein, CD117 and Dog1 all negative. All the tumors were complete surgical excision. During follow-up, one case recurred the second time. Our conclusions are the diagnosis of aggressive angiomy-xoma is based on pathological morphology supplemented by immunohistochemistry, and the tumor may relapse after surgical resection.