Hsieh Frank, Chuang Kai-Ti, Wu You-Ting, Lin Chih-Hung
Department of Plastic & Reconstructive Surgery, Chiayi Chang Gung Memorial Hospital, Chang Gung University, Taiwan.
Department of Laboratory Medicine, Chiayi Chang Gung Memorial Hospital, Chang Gung University, Taiwan.
Plast Reconstr Surg Glob Open. 2018 Aug 8;6(8):e1879. doi: 10.1097/GOX.0000000000001879. eCollection 2018 Aug.
Aggressive angiomyxoma is a very rare benign tumor for male population with fewer than 50 cases reported since the description of this tumor. Most documented cases of aggressive angiomyxomas were found in genital, perineal, and pelvic regions in women of child bearing age. We report a case of a massive (> 20 cm) aggressive angiomyxomas in a man who presented with perineal swellings. Macroscopically the mass was highly vascular and lobulated with high similarity to plexiform neurofibroma. Microscopic examination revealed a hypocellular tumor comprising bland oval and spindle-shaped cells along with vessels of varying calibre. The accompanying stroma was myxocollagenous. Immunohistochemical staining showed CD34 and focal estrogen receptors positivity and negative staining for S100, actin, desmin, and progesterone receptors. The histologic and immunohistochemical features favored the diagnosis of aggressive angiomyxoma. Despite the rarity of such tumor in the male population, aggressive angiomyxoma should be considered in the differential diagnosis when encountering chronic para-perineal lesions.
侵袭性血管黏液瘤是一种非常罕见的良性肿瘤，在男性中发病率极低，自该肿瘤被描述以来，报告的病例少于50例。大多数有记录的侵袭性血管黏液瘤病例发现于育龄期女性的生殖器、会阴和盆腔区域。我们报告一例男性巨大（>20 cm）侵袭性血管黏液瘤病例，该患者表现为会阴部肿胀。大体上，肿块血管丰富，呈分叶状，与丛状神经纤维瘤高度相似。显微镜检查显示肿瘤细胞稀少，由形态温和的椭圆形和梭形细胞以及不同管径的血管组成。伴随的间质为黏液胶原性。免疫组化染色显示CD34和局灶性雌激素受体阳性，而S100、肌动蛋白、结蛋白和孕激素受体染色阴性。组织学和免疫组化特征支持侵袭性血管黏液瘤的诊断。尽管这种肿瘤在男性人群中罕见，但在遇到慢性会阴旁病变时，鉴别诊断中应考虑侵袭性血管黏液瘤。
