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目的 研究皮肤及眼球外原发性恶性黑色素瘤的临床与病理特征 ,以提高临床早期诊断率。方法 对 18例皮肤及眼球外原发性恶性黑色素瘤进行临床资料分析 ,病理组织学、免疫组化及电镜研究。结果 本组患者平均年龄 5 1.6岁 ,以女性为主。病变部位 :肛管直肠 5例 ,鼻腔及鼻窦 4例 ,口腔粘膜 2例 ,睑结膜、膀胱、阴道、小阴唇、宫颈、骨及乳腺各l例。肿瘤组织学形态多样 ,既象癌又象肉瘤 ,其中 16例可见黑色素颗粒 ,2例为无色素性。免疫组化标记 :肿瘤细胞S-10 0 蛋白 ,HMB 4 5及Vimentin抗体阳性表达。电镜检查 6例均可见不同时期的黑色素小体。结论 恶性黑色素瘤不仅源于体表皮肤的色素细胞 ,还可起自粘膜及间叶组织中残留的神经嵴细胞。
Objective To study the clinical and pathological features of primary malignant melanoma outside the eye and skin to improve the early diagnosis rate. Methods The clinical data of 18 cases with primary malignant melanoma outside the eye and skin were analyzed by histopathology, immunohistochemistry and electron microscopy. Results The average age of patients in this group was 5 1.6 years old, mainly female. Lesions: 5 cases of anorectal, nasal and sinus in 4 cases, 2 cases of oral mucosa, conjunctiva, bladder, vagina, labia minora, cervix, bone and breast in l cases. Tumor histology varied, both as cancer and sarcoma, of which 16 cases of melanin particles, 2 cases of non-pigmented. Immunohistochemistry: tumor cells S-10 0 protein, HMB 4 5 and Vimentin antibody positive expression. Electron microscopy of 6 cases were seen at different stages of melanosomes. Conclusion Malignant melanoma not only originates from pigment cells on the surface of the skin, but also acts as residual neural crest cells in mucosa and mesenchyme.