摘要
目的:探讨胆管葡萄状肉瘤,临床病理学特点和鉴别诊断,不同分化程度的胆管葡萄状肉瘤与预后的关系。方法:收集1990年4月～2004年10月间共3例胆管葡萄状肉瘤,复习临床资料,收集每例组织蜡块进行切片,HE染色、特殊组织化学染色及免疫组化染色观察分析。结果:本组3例发病年龄为14个月、5岁4个月、4岁。男2例、女1例。1例由胆总管蔓延至胆囊颈部,1例蔓延至肝门。瘤细胞及其排列具有特征性结构,在正常或伴有增生的粘膜下为未分化型肌母细胞即:"形成层"状,内部为粘液瘤样区域。细胞学特点是各种形态肿瘤细胞均有不同程度嗜酸胞浆。免疫组化:vimentin(+)、desmin(+)、actin(+)、myoglobin(+)。结论:胆管葡萄状肉瘤罕见,几乎均见于幼儿。高分化胆管葡萄状肉瘤易误诊为一些良性病变,延误治疗,需引起重视;低分化葡萄状肉瘤,则易误诊为恶性淋巴瘤等。本病具有侵袭性及远处转移,预后差。但目前手术、放、化疗效果满意,生存期较大。
Objective： To study the clinicopathological features and differential diagnosis of botryold rhabdomyosarcoma in the bile duct and the relationship between different clinical pathological classifications and the prognosis. Methods： A retrospective analysis was performed using data from 3 patients with botryoid rhabdomyosarcoma of the bile duct from 1990 to 2004. The paraffin-embedded specimens were analysed after H＆E staining, special histochemistry and immunohistochemistry. Resuits： The ages of the 3 cases ranged from 14 months to 4 years. Two were male and 1 was female. The pathological changes were mainly located in the trunk of the bile duct. In 1 case it was extended to the neck of gallbladder and the top of the gallbladder itself and in 1 case it spread to the liver. The tumor cells displayed a characteristic structure upon histological examination. There were undifferentiated muscle cells, namely the cambium layer below the natural or hyperplastic mucous membrane. The tumor cells were positive for H＆E staining. Immunohistoehemical staining showed that tumor cells were positive for vimentin, desmin, actin and myogolobin. Conclusions： Botryoid rhabdomyosarcoma of the bile duct is rare and mainly appears in infants. Botryoidal sarcoma of bile duct is easily diagnosed based on pathological changes, but an error in pathology can result in an incorrect clinical diagnosis. The poorly-differentiated botryoid rhabdomyosarcoma was incorrectly diagnosed as malignant lymphoma. It was more invasive and had a poor prognosis. The very young patients with botryoid rhabdomyosarcoma have a good prognosis and a long survival time alter surgery followed with radiation and chemotherapy.
出处
《中国肿瘤临床》
CAS
CSCD
北大核心
2007年第9期521-523,共3页
Chinese Journal of Clinical Oncology
关键词
胆管肿瘤
葡萄状肉瘤
临床病理
免疫组织化学
Tumor of bile duct Botryoid rhabdomyosarcoma Clinicopathological Immunohistochemistry
