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富含黏蛋白性结缔组织的皮肤血管肌脂肪瘤1例

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患者男,27岁,右臀部肿物缓慢增大不伴痛痒5年余.皮肤科情况:右臀部可见一约3 cm × 3 cm大小暗红色结节,表面光滑,境界清楚,质地较硬,轻度压痛.皮损组织病理示:肿瘤团块位于真皮下部,为边界清晰的圆形结节,周围有纤维性假包膜,肿瘤主要由丰富的厚壁血管、成熟脂肪组织及梭形平滑肌细胞组成,平滑肌细胞排列可呈束状并可相互交织,并可见结缔组织,包括丰富的黏蛋白、胶原、血管、星状细胞及成纤维细胞,未见异型性及核分裂象.阿辛蓝染色阳性.免疫组织化学染色示:肿瘤团块内血管CD31(+)、CD34(+);梭形平滑肌细胞SMA(+)、Desmin(+)、S100(-)、HMB45(-);结缔组织成分中 Fcator ⅩⅢa(+);脂肪细胞S100(+).诊断:富含黏蛋白性结缔组织的皮肤血管肌脂肪瘤.
Cutaneous Angiomyolipoma of Mucin-Rich Connective Tissue:A Case Report
A 27-year-old man presented with a mass on the right hip for more than five years without pain or itch.Dermatological examination showed a dark red nodule,3 cm × 3 cm in size,on the right hip with a smooth surface,clear boundary,hard texture,and mild tenderness.The histopathology of the skin lesion showed that the mass was located in the lower dermis,and was a well-defined circular nodule surrounded by a fibrous pseudoenvelope.The tumor was mainly composed of abundant thick-walled blood vessels,mature adipocytes,and fusiform smooth muscle cells,which were arranged in bundles and interwoven,and no atypia or mitosis was observed,while connective tissue containing abundant mucin,collagen,blood vessels,stellate cells and fibroblasts could be seen.Alcian blue staining was positive for mucin.Immunohistochemical staining results showed that CD31 and CD34 were positive in the blood vessels in the mass,and smooth muscle antibody(SMA)in spindle-shaped smooth muscle cells and Desmin were positive,while S100 and HMB45 was negative.Fcator ⅩⅢa was positive in the fibroblasts in connective tissue.A diagnosis of cutaneous angiomyolipoma of mucin-rich connective tissue was made.

CutaneousAngiomyolipomaMucinConnective tissue

贾红侠、冉立伟

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首都医科大学附属北京朝阳医院皮肤科,北京 100043

皮肤 血管肌脂肪瘤 黏蛋白 结缔组织

2025

中国皮肤性病学杂志
西安交通大学

中国皮肤性病学杂志

北大核心
影响因子:0.66
ISSN:1001-7089
年,卷(期):2025.39(1)