食管原发性恶性黑色素瘤的临床病理观察

The clinicopathological observation of primary malignant melanoma of the esophagus

目的:探讨食管原发性恶性黑色素瘤的诊断、鉴别诊断及生物学行为。方法:报道1例食管原发性恶性黑色素瘤,对其进行光镜观察、免疫组织化学及特殊染色检测,并复习文献。结果:食管原发性恶性黑色素瘤多见于中老年人,常发生于食管中、下段1/3处,大体呈息肉状;光镜下瘤细胞异型性明显,呈梭形、圆形或不规则型,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,部分细胞胞浆内有棕黄色颗粒;免疫组化结果肿瘤细胞显示波形蛋白(Vimentin)、S-100蛋白、HMB45和Melanoma-pan阳性;不表达神经特异性烯醇化酶(neuron-speciific 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 A,CgA)、人绒毛膜促性腺激素(human chorionic gonadotropin,HCG)、上皮膜抗原(epithelial membtane antigen,EMA)、高分子量CK、CK18、小细胞肺癌(small cell lung cancer,SCLC)和突触素(synaptophysin)等。结论:食管原发性恶性黑色素瘤是一种罕见肿瘤,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、下段息肉样肿物应考虑原发性恶性黑色素瘤的可能。

Objective： To investigate the diagnosis, differential diagnosis and biologic behaviors of primary malignant melanoma of the esophagus. Methods： A case of primary malignant melanoma of the esophagus was reported with microscopical observation, immunohistochemistry and reyiew of literature. Results： Most of primary malignant melanoma of the esophagus occured in middle and old age persons. The tumors were usually located in the distal third of the thoracic esophagus. Glossily, the tumor tended to be polypoid. Microscopically, the tumor cells were multi - pleomorphic, and they showed be round, spindle shaped and irregular. The tumor nucleus were large, prominent nucleoli and abundant cytoplasm. Some tumor ceils were with melanin deposition of melanin pigment. Immunohistochemically, the tumor ceils were positive for HMB - 45, Melanoma - pan, S - 100 protein and Vimentin. Conclu- sion： Primary malignant melanoma of the esophagus was a rare neoplasm. It was difficult to diagose before biopsy or operation because its clinical symptom was similar to other neoplasms of the esophgus. It should be considered as primary malignant melanoma of the esophagus when a polypoid tumor in the lower third of esophagus was observed in the middle or old aged person.