目的:分析食管原发性恶性黑色素瘤(primary malignant melanoma of the esophagus,PMME)的病理诊断特点、鉴别诊断、生物学行为及预后.方法:收集安阳市肿瘤医院5例PMME,对其进行光镜观察、免疫组织化学染色、临床病理分析并进行随访.结果:PMME多见于中老年人,常发生于食管中段,大体呈蕈伞型.光镜下瘤细胞异型性明显,呈梭形、卵圆形或不规则形,以梭形细胞为主.肿瘤细胞胞核体积大,核仁清晰、染色质丰富.瘤细胞形态多样且无色素易误诊为分化差的鳞状细胞癌、肉瘤样癌、淋巴瘤等.免疫组织化学结果肿瘤细胞显示vimentin,S-100,HMB-45和Melan-A阳性;不表达神经特异性烯醇化酶(neuron-specific enolase,NSE)、白细胞共同抗原(leukocyte common antigen,LCA)、细胞角蛋白(cytokeratin,CK)、嗜铬素蛋白-A(chromogranin proteins A,CgA)和突触素(syn).随瘤细胞浸润不同组织层次可伴有淋巴结转移.结论:PMME是一种罕见肿瘤,恶性度高、预后差.由于其症状与食管其它肿瘤相似,在活检或手术前很难确诊,对发生于中老年人的食管中段蕈伞型肿物应考虑原发性恶性黑色素瘤的可能.临床病理分析肿瘤大小、浸润层次、淋巴结转移及治疗方式可能影响预后.%Objective:To analyze the pathological characteristics,differential diagnosis,biological behavior and prognosis of primary malignant melanoma of the esophagus (PMME).Methods:Five cases of PMME in Anyang Tumor Hospital were collected and analyzed by microscopy,immunohistochemical staining,clinical pathology analysis and followed up.Results:PMME is more common in the elderly,it often occurs in the middle of esophagus,generally mushroom umbrella.Under microscope the tumor cell showed atypia,spindle-shaped,oval or irregular,with shuttle shaped cells.Bulky tumor cell nuclei,prominent nucleoli,abundant chromatin.Various forms of tumor cells and non-pigmented was misdiagnosed as poorly differentiated squamous cell carcinoma,sarcomatoid carcinoma,lymphoma and so on.Immunohistochemistry of tumor cells showed positive in vimentin,S-100,HMB-45 and Melan-A;do not express neuron-specific enolase (NSE),leukocyte common antigen (LCA),cytokeratin (CK),chromogranin proteins A (CgA) and synaptophysin (syn).Infiltration of tumor cells with different organizational levels maybe associated with lymph node metastasis.Conclusion:PMME is a rare tumor,with high malignancy and poor prognosis.Due to its symptoms similar to other esophagus cancer,it's difficult to be diagnosed before biopsy or surgery,to those esophageal fungating tumors occurred in the elderly,the primary malignant melanoma should be considered.The clinical pathological analysis of tumor size,invasive level,lymph node metastasis and treatment may influence prognosis.
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