空肠原发性恶性黑色素瘤临床病理观察  

Primary malignant melanoma of jejunum: a clinicophathological analysis

作　　者：张轶华[1] 祁晓莉[1] 张珊珊[1] 乔星[1] 霍艳丽[1] 

机构地区：[1]首都医科大学大兴医院病理科,北京102600

出　　处：《诊断病理学杂志》2014年第8期498-500,共3页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨空肠原发性恶性黑色素瘤的临床病理特征、诊断与鉴别诊断。方法对1例空肠原发性恶性黑色素瘤的病理形态学、免疫组化及荧光原位杂交(FISH)结果进行分析,并复习国内外相关文献。结果患者男性,50岁。间断性左下腹痛1年。腹部彩超及CT示空肠肿物合并肠系膜淋巴结肿大。切除的小肠肿物呈结节状,灰白、灰红色,部分区域出血状,似位于肠壁肌层内,相对应之肠黏膜可见溃疡形成。镜下肿瘤位于肠黏膜下,细胞弥漫分布,细胞大小不一致,核异型性明显,胞质较少,细胞内、外可见少量黑色素。免疫组化示黑色素瘤标记物HMB45和melan-A(+);上皮标记物CKpan及淋巴标记物LCA(-)。FISH显示无EWSR1(22q12)位点断裂。结论原发于消化道空肠部位的恶性黑色素瘤极为罕见,组织形态学、免疫组化及FISH检查对本病的确诊具有重要作用。该瘤预后很差,首选外科手术,对肉眼可见的肿瘤病灶进行根治性切除可延长生存期。Objective To investigate the clinicopathological characteristics, diagnosis and differential diagnosis of a malignant melanoma derived from jejunum, a very rare location. Methods A case of malignant melanoma in the jejunum was studied with morphology, immunohistochemistry and fluorescence in situ hybridization （FISH）, and the literatures were also reviewed. Results The patient was a 50 -year-old male who suffered intermittent pain in his left lower abdomen for about one year. The ultrasound and CT scan showed a big mass in his jejunum with mesenteric lymph node enlargement. Grossly, in the muscularis of a part of jejunum, a nodular tumor was about 4 × 2 × 1 cm in size, pale red to grey,and bleeding in some areas. The ulcer was also been observed in the corresponding mucosa. Microscopically, the tumor cells diffusely distributed in the submucosa with variant size, prominent nuclear atypia and less cytoplasm. A small amount of melanin was detected within the tumor cells and outside. Immunohistochemical staining was positive for HMB45, Melan-A and negative for Ckpan, LCA. The result of FISH showed that the case was without breaking of EWSR1 （22q12） site. Conclusion The primary melanoma of jejunum is seldom seen in jejunum, the features of morphology, IHC and FISH play an very important role in the diagnosis. Primary malignant melanoma of jejunum has a worse prognosis. Surgery is the preferred treatment for this lesion, and radical resection for obvious lesions can prolong the survival of the patient.

关 键 词：空肠 原发性 恶性黑色素瘤 临床病理 

分 类 号：R735.33[医药卫生—肿瘤]