小汗腺血管瘤样错构瘤临床病理观察

Eccrine angiomatous hamartoma：a clinicalopathologic analysis of 4 cases

目的：探讨小汗腺血管瘤样错构瘤的临床及病理组织学特征、诊断和鉴别诊断。方法应用光镜及免疫组织化学染色对4例小汗腺血管瘤样错构瘤进行临床、组织病理学和免疫表型观察，同时复习相关文献。结果4例中男性3例，女性1例。年龄分别为4个月、3.5岁、5.5岁和14.0岁。左小指、右示指、腰背部和膝部各1例。临床上病变主要表现单发的丘疹、结节或斑块，皮色或紫红色，最大径0.4～6.0 cm。3例皮损随年龄增长而增大，局部多汗1例；组织学上，病变位于真皮中下部及皮下组织上部，主要由分化成熟的小汗腺及血管成分增生组成；免疫组织化学显示：血管成分CD31、CD34和第八因子相关抗原呈阳性表达，小汗腺腺体S-100蛋白、癌胚蛋白、上皮细胞膜蛋白、CAM5.2和GCDFP15呈阳性表达。4例均行外科单纯切除术，术后随访0.5～4.5年，均无复发。结论小汗腺血管瘤样错构瘤是一种罕见的良性错构瘤性病变，临床表现、组织形态学及免疫组织化学相结合有助于诊断与鉴别诊断。

Objective To investigate the clinical and histopathologic features , diagnosis and differential diagnosis of eccrine angiomatous hamartoma （ EAH ）.Methods Four cases of eccrine angiomatous hamartoma were studied by light microscopy and immunohistochemical staining along with review of the literature.Results There were 3 male and 1 female patients at diagnosis in age of 4 months, 3.5 years, 5.5 years and 14.0 years, respectively.Tumor sites included the left little finger （1 case）, right index finger （1 case）, lower back （1 case） and knee （1 case）.Clinically, most cases presented as a solitary, flesh or reddish papule , plaque or nodule with size of 0.4-6.0 cm in diameter.The skin lesions in 3 cases enlarged commensurate with the growth of the patients , and local hyperhidrosis in one case.Histologically, EAH was characterized by proliferation of well-differentiated eccrine secretory and ductal elements closely associated with thin-walled angiomatous channels in the middle or deep dermis and subcutaneous tissue.By immunohistochemistry , the vascular components were positive for CD 31, CD34 and factorⅧrelated antigen while the eccrine glands were positive for S-100 protein, CEA, EMA, CAM5.2 and GCDFP15.Local surgical resection was performed in 4 cases and follow-up data （0.5 to 4.5 years） showed no recurrence.Conclusion EAH is a rare, benign cutaneous hamartoma.Combination of clinical manifestations , histological changes and immunohistochemical findings is useful for the diagnosis and differential diagnosis .