在具有不同组织发生的各种良性和恶性疾病中观察到皮肤的透明细胞肿瘤,共享具有丰富的透明细胞质的细胞的存在。在这里,我们报告了一名健康的年轻患者的临床病理特征,喷发和传播,从婴儿早期开始的良性透明细胞真皮肿瘤。家族史或基因检测和咨询都没有提供进一步的有用信息。病变多局限在面部和左下肢,伴有粉红色毛细血管扩张丘疹和小结节。在4年的时间里,共对16个不同的皮肤病变进行了活检,并进行了组织病理学和免疫组织化学研究;还切除了另一个病变进行电子显微镜检查.组织病理学证实纺锤体至椭圆形和圆形细胞与整个真皮中的透明/颗粒细胞混合在一起的多个血管周围生长。有明显的血管增生和许多毛细血管样血管,局灶性血管外皮细胞瘤样特征。免疫组织化学肿瘤细胞对h-caldesmon和局灶性平滑肌α-肌动蛋白和CD13均呈阳性,表明肌样分化,而对溶酶体抗原的弥漫性细胞质染色一致,例如CD68PG-M1和NKI/C3以及超微结构的发现支持了溶酶体介导的凋亡过程的观点。讨论了与其他透明细胞皮肤肿瘤的鉴别诊断。
Clear cell tumors of the skin are observed in a wide variety of benign and malignant conditions with different histogenesis, sharing the presence of cells with abundant clear cytoplasm. Herein, we report the clinicopathologic features of a healthy young patient affected by asymptomatic, eruptive and disseminated, benign clear cell dermal tumors since early infancy. Neither family history nor genetic testing and counseling provided further useful information. The lesions were mostly confined to the face and lower left extremity with pink teleangiectatic papules and small nodules. Over a 4-year period, a total of 16 different cutaneous lesions were biopsied and histopathologic and immunohistochemical studies carried out; an additional lesion was also removed for electron microscopy examination. Histopathology evidenced multiple perivascular growths of spindle to oval and round cells intermingled with clear/granular cells throughout the dermis, with prominent desmoplasia and numerous capillary-like vessels with focal hemangiopericytoma-like features. Immunohistochemical neoplastic cells were uniformly positive for h-caldesmon and focally smooth muscle α-actin and CD13 indicating myoid differentiation whereas the consistent diffuse cytoplasmic staining for lysosome antigen, such as CD68PG-M1 and NKI/C3 along with the ultrastructural findings supported the view of a lysosome-mediated apoptotic process. The differential diagnosis with other clear cell cutaneous neoplasms is discussed.