乳房Paget病22例临床与组织病理分析  

Clinical and pathological analysis of 22 cases of mammary Paget's disease

作　　者：张韡[1] 姜祎群[1] 温斯健[1] 杨莹[1] 王小坡[1] 曾学思[1] 孙建方[1] 

机构地区：[1]中国医学科学院皮肤病研究所,南京210042

出　　处：《实用皮肤病学杂志》2015年第6期428-431,共4页Journal of Practical Dermatology

基　　金："协和青年基金资助"和"中央高校基本科研业务专项基金"(编号:333201357)

摘　　要：目的探讨22例乳房Paget病患者的临床表现和组织病理改变。方法所有标本均来自2010-2013年医院门诊就诊者，分析其临床资料、苏木素．伊红（HE）及免疫组化染色结果、随访信息。结果22例乳房Paget病患者均为女性，平均年龄55岁（35～83岁），平均病程3．4年（0．5～15年），均为单侧发病，14例（64％）累及乳头。组织病理改变显示，15例患者表现为大细胞型，5例表现为小细胞型，2例表现为大细胞型并小细胞型。14例呈全层分布模式，6例呈经典分布模式，腺泡样分布模式和腺泡样并棘层松解分布模式各1例。22例患者在乳腺外科均确诊为乳腺癌。第1年随访中失访9例，2例发生全身广泛转移而死亡，11例行广泛切除术后随访至今未见复发。结论乳房Paget病与乳腺癌关系密切，一经诊断需及时至乳腺外科就诊，高度警惕转变成乳腺癌。临床上大部分患者乳头受累，组织病理学上可见到不同的细胞类型及分布模式，其临床意义尚不明确。Objective To investigate the clinical and histopathologieal features of 22 cases of mammary Paget＇s disease. Methods Specimens were collected from 22 outpatients with mammary Paget＇s disease evaluated in our hospital during the period from 2010 to 2013. Clincal data, findings in the examination with H＆E staining and immunohistochemical staining as well as follow-up data were retrospectively evaluated for these cases. Results All patients enrolled were female with an average age of 55 years old （range 35-83）, and the average course of disease were 3 years （range 0.5-15）. All the patients were unilaterally involved and 14 patients （64%） had the involvement of the nipple. Histopathologieally, the typical Paget cells （PC） were presented in 15 patients （68%）, while small cells were presented in 5 cases （23%） and 2 cases （9%） presented with a combination. Sheet-like pattern was showed in 14 patients （64%）, while prototypical pattern was showed in 6 cases （27%）, glandular pattern was showed in 1 case （5%） and 1 cases （5%） presented with a combination of glandular and acantholysis-like pattern. All the patients were confirmed with breast cancer by further investigations. During the 1 year follow-up, 9 patients were dropped and 2 cases died of extensive metastasis; 11 cases were alive without recurrence after extensive resection till to now. Conclusion Mammary Paget＇s disease is significantly associated with breast cancer, and it often involves the nipple, which should be paid attention to in the clinical practice for underlying breast cancer. There are different types of PC and distribution patterns while their clinical implications need further investigation.

关 键 词：Paget病 乳房 临床表现 组织病理学 随访 

分 类 号：R737.9[医药卫生—肿瘤] R730-26[医药卫生—临床医学]