摘要
目的 提高对外阴海绵状淋巴管畸形（CLM）的认识。方法 分析1例双侧小阴唇CLM的临床表现、组织病理及免疫组化，并进行讨论。结果 17岁未婚女性双侧小阴唇缓慢增大的皮下肿物2年。体检见双侧小阴唇肾形皮下肿物，质地较软。肿物切面黄白色，其内见米粒大小散在囊腔，充满无色清亮液体；组织病理显示,真皮弥漫性分布大小不一的扩张淋巴管，管腔内衬单层扁平上皮，腔之间为较厚的纤维性间隔。淋巴管内皮细胞D2？40、CD31阳性，CD34阴性；间质成纤维细胞孕激素受体阳性，雌激素受体阴性。诊断：双侧小阴唇海绵状淋巴管畸形。手术切除双侧小阴唇肿物，随访6个月未见复发。结论 双侧小阴唇组织病理及免疫组化对疾病的诊断及鉴别诊断有重要作用。
Objective To improve the understanding of cavernous lymphatic malformation（CLM）of the vulva. Methods The clinical manifestations, histopathological and immunohistochemical features of a case of CLM involving bilateral labia minora were analyzed, and relevant literatures were reviewed. Results A 17-year-old unmarried female patient presented with gradually enlarged subcutaneous masses on the bilateral labia minora for 2 years. Physical examination showed that soft kidney-shaped subcutaneous masses were observed on the bilateral labia minora. After surgical resection, a yellow-white solid cut surface was observed, and there were scattered rice-sized cyst cavities filled with clear colorless liquid. Histopathologically, a large number of dilated lymphatic vessels with different sizes of lumens were scatteredly distributed in the dermis. The lumens were lined by a single layer of flattened endothelial cells, and there were thick fibrous septa between the lumens. Endothelial cells in the lymphatic vessels were stained positive for D2-40 and CD31, but negative for CD34. Interstitial fibroblasts were stained positive for progesterone receptors, but negative for estrogen receptors. Finally, the patient was diagnosed with CLM of bilateral labia minora. After surgical resection of the masses on the bilateral labia minora, no relapse was observed during the follow-up of 6 months. Conclusion Histopathological and immunohistochemical features facilitate the diagnosis and differential diagnosis of CLM.
出处
《中华皮肤科杂志》
CAS
CSCD
北大核心
2018年第2期136-137,共2页
Chinese Journal of Dermatology
关键词
淋巴管畸形
淋巴管瘤
外阴
Lymphatic abnormalities
Lymphangioma
Vulva
