Pathogenesis and treatment of Paget's disease of the breast

Fifty cases of Paget's disease of the breast treated surgically at The Johns Hopkins Hospital during the past 30 years were studied. Nineteen patients had Paget's disease confined to the nipple and 31 had an associated palpable tumor. An underlying intraductal or infiltrating duct carcinoma of the breast was present in each case. In six cases, the underlying tumor was 2 cm or more from the nipple with no apparent anatomic connection to the Paget lesion, and one case was encountered in whom intradermal Paget's disease develop in the area of a congenitally absent nipple. These findings support the theory of an intradermal origin for the Paget cell. Survival rates of patients with Paget's disease and a palpable breast mass were similar to those of patients with infiltrating duct carcinoma, the presence of axillary node metastases being the most important prognostic factor. Actuarial five- and ten-year survival rates were 22% and 9.9% for patients with positive nodes. The modified radical mastectomy is recommended as primary therapy for this group of patients. In contrast, none of the patients with Paget's disease of the nipple and no evidence of a palpable breast mass developed recurrent carcinoma. A total mastectomy without an axillary node dissection is the treatment of choice in this type of patient.     

乳腺Paget病61例临床分析

目的探讨乳腺Paget病的早期诊断和治疗方法.方法回顾性分析中国医学科学院肿瘤医院1970年11月～2001年11月收治的61例乳腺Paget病患者的临床资料.结果术前行乳房病变刮片细胞学检查24例,准确率为75.0%(18/24);行切取活检18例,准确率为100.0%(18/18).61例患者行根治性手术43例(根治术15例、改良根治术28例),乳房单纯切除术18例.进行根治性手术的43例患者中,乳房可触及肿块者25例,病理检查有腋下淋巴结转移者16例,转移率为64.0%;而乳房未触及肿块者18例,有腋下淋巴结转移4例,转移率为22.2%.61例患者经过8～240个月随访(中位随访期为62个月),局部复发9例,远处转移12例,5年生存率为79.5%,10年生存率为51.9%.结论尽早行乳房病变细胞学和病理学检查是确诊乳腺Paget病的最佳方法.乳腺肿物是影响乳腺Paget病预后的不良因素,对合并乳腺肿块的患者应该行根治性切除术,未发现肿块的患者可以行乳房单纯切除术.     

Paget's disease of the nipple: A ten year review including clinical,pathological, and immunohistochemical findings

Thirty-five women with biopsy-proven Paget's disease of the nipple were treated over a 10 year period at the Breast Cancer Unit, Guy's Hospital. Twenty-four (69%) patients had Paget's disease without a palpable mass in the breast; eleven (31%) presented with a palpable mass and Paget's disease of the nipple. Definitive treatment consisted of modified radical mastectomy in 32 patients, radiotherapy only in 2, and one patient had no definitive treatment.All 11 patients with Paget's disease and an associated lump proved to have invasive ductal carcinoma; five also had associated positive axillary nodes. Nine of the 23 patients with nipple changes only, treated by mastectomy, also had invasive carcinoma; three of these had positive axillary nodes. The remaining 14 patients with nipple changes only were found to have in situ ductal carcinoma, which was extensive in the majority of cases.In 13 cases, histological sections of the nipple were examined by immunohistochemical staining which showed that the Paget's cells expressed a keratin phenotype that was specifically characteristic of simple epithelial cells as seen in glandular epithelium. This was quite unrelated to the normal keratin phenotype of the surrounding skin keratinocytes.Clinical, pathological, and immunohistochemical data suggest a mammary origin of the abnormal cells in Paget's disease of the nipple. Mastectomy appears to be the treatment of choice.     

乳腺导管内癌20例临床分析

我院自１９７９年１月至１９９３年１２月经手术治疗乳膜癌３５７例，其中导管内癌２０例，占５．６％。首发症状为肿块者占６０％（１２／２０），乳头溢液者占２５％，乳头湿疹样改变者占１０％，肿埠伴溢液者占５％。腋窝淋巴结转移者占１０％。５年生存率９１．７％。分析认为，某些管内癌症状不典型，易漏诊、误诊，应引起重视。对术式的选择以改良根治术为首选。     

Paget's disease of the breast.

Twenty-nine histologically verified cases of Paget's disease of the breast treated at the Hadassah University Hospital in the years 1949-1972 were followed up and analyzed. Dividing this material into two groups according to the presence or absence of a palpable breast tumor revealed significant difference in behavior and survival. Patients with a breast mass (34%) had a 50% axillary lymph node involvement and behaved as with any other ordinary breast cancer, with a 5-year survival rate of 40% and a 10-year survival rate of 33%. Patients with no palpable breast mass (66%) had only a 10.5% lymph node involvement, the 5-year survival rate being 94% and the 10-year survival rate being 91%. Delay in diagnosis seems to play no significant factor in survival rates and outcome. We believe radical mastectomy to be the treatment of choice in all cases of Paget's disease of the breast.     

乳腺癌合并派杰氏病34例分析

目的　探讨乳腺癌 (BC)合并派杰氏病 (PD)的流行病学、临床、病理特点、诊治和预后。方法　统计 195 8～ 1997年经手术和病理证实的乳腺癌合并派杰氏病 34例 ,从发生率、发病年龄、发病趋势、临床表现、分期和病理、诊断、治疗和预后等方面加以分析。结果　乳腺癌合并派杰氏病在乳腺癌中的发生率为 0 .9% ,可分为乳头病变组和乳腺肿块组 ,高发年龄 35～ 5 5岁 ,近年来就诊人数下降 ,治疗采取以手术为主的综合治疗。乳头病变组经病理证实 ,腋窝淋巴结阳性率、转移率均为 2 6 .7% ;5 ,10 ,2 0年生存率分别为 93.1%、78.1%和 6 7.0 %。乳腺肿块组经病理证实 ,腋窝淋巴结阳性率 5 2 .6 % ,远处转移率 42 .1% ;5 ,10 ,2 0年生存率分别为 6 0 .5 %、5 3.8%和 5 3.8%。结论　乳腺派杰氏病作为乳腺癌的一种特殊类型可与乳腺癌同时存在 ,病变局限于乳头的患者的预后优于合并乳腺包块者。     

湿疹样乳腺癌85例临床分析

目的探讨湿疹样乳腺癌的临床特点、诊治方法及预后因素,提高对湿疹样乳腺癌的认识。方法回顾性分析作者在韩国首尔峨山医院进修进时收集的85例湿疹样乳腺癌患者的临床资料。结果共入组85例患者,女性83例,男性2例。乳腺有湿疹样表现79例（92．9％）,伴乳腺肿块52例（61．2％）,同侧腋窝淋巴结转移27例（占本组85例的31．8％及伴乳腺肿块病例的51．9％）,均为伴有乳腺肿块的病例。52例行乳癌改良根治术,30例行乳腺单纯切除术,3例行局部切除术。单纯乳头及乳晕湿疹样乳腺癌11例（12．9％）,均为无乳腺肿块的病例,其余74例（87．1％）均合并其他类型的乳腺癌。本组5年生存率为82．1％,其中无乳腺肿块者5年生存率为93．9％,合并肿块者5年生存率为75．0％（P〈0．05）,无同侧腋窝淋巴结转移者5年率为96．6％,有转移者5年生存率为51．9％（P〈0．01）。结论湿疹样乳腺癌有特殊的临床表现,但易误诊,病灶细胞学和病理学检查可以确诊,伴乳腺肿块者应选择乳癌改良根治术,无乳腺肿块者可选择乳腺单纯切除术,预后与乳腺肿块及同侧腋窝淋巴结转移密切相关。     

男性乳腺癌26例临床疗效探讨

目的：总结分析男性乳腺癌的临床病理特点、诊断、治疗措施及预后因素。方法：回顾性分析了在中国医学科学院肿瘤医院行手术治疗的26例男性乳腺癌患者的临床病理资料。结果：26例患者均经细胞学检查或切除活检确诊。患者接受乳腺癌根治术3例,改良根治术22例,乳腺区段切除术1例。病理为浸润性导管癌者25例,其中1例合并乳头Paget’s病;粘液腺癌1例。25例行腋窝淋巴结切除的患者中,腋窝淋巴结转移12例。20例患者行激素受体检测,ER阳性者17例（85.0%）,PR阳性者14例（70.0%）,Her-2阳性者6例（30.0%）。随访期内,死亡5例,肺转移2例,骨转移1例。结论：以外科治疗为主的综合治疗是目前对于男性乳腺癌的治疗模式,男性乳腺癌的预后与临床分期有一定关系。     

Paget＇s Disease of the Breast： Clinical Analvsis of 45 Patients

Objective Paget’s disease is an uncommon breast malignancy and often misdiagnosed. If the patient is treated at an early stage, the prognosis is satisfactory. Our study analyzed the clinical characteristics of the disease and the factors influencing the prognosis. Methods Fourty-five patients with Paget’s disease who were admitted to our hospital were analyzed retrospectively. Results The most common symptoms of the disease were erosion and a bleeding-like eczematoid change at the nipple/areola. Of the 40 patients with an eczematoid change, 11 patients had verified Paget’s disease with a palpable mass on physical examination, and 29 patients had verified Paget’s disease with a nonpalpable mass. Only 5 patients manifested a mass with no eczematiod change. Thirteen patients had ipsilateral axillary lymphadenopathy. In this study, 25 cases were treated by radical mastectomy and 20 cases were treated by modified radical mastectomy. Five and 10-year survival rates for the patients with nonpalpable masses and for those with palpable masses were 95.5%, 78.6%, and 53.8%, 36.4% respectively. The former were significantly higher than the latter (P <0.01 and <0.05 respectively). Five and 10-year survival rates for the patients without underlying carcinoma (DCIS/IDC) and for the patients with underlying carcinoma were 100%, 88.9% and 69.6%, 43.8% respectively. The former were significantly higher than the latter ( P<0.05) Five and 10-year survival rates for the patients with negative lymph nodes and for the patients with positive lymph nodes were 92.0%, 76.5% and 50.0%, 25.0% respectively. The former were also significantly higher than the latter (P<0.05). Conclusion Treatment at an early stage is very important and influences the prognosis directly for Paget’s disease of the breast. The survival rates of patients with nonpalpable masses without underlying carcinoma and without lymphadenopathy, were significantly higher than patients with palpable masses with underlying carcinoma and with lymphadenopathy respectively. There was significant statistical difference between each of the 2 groups.     

乳腺派杰氏病45例临床分析

目的:研究分析乳腺派杰氏病的临床特点及影响其预后的因素.方法:对我院收治的45例经病理诊断的乳腺派杰氏病例资料进行回顾性分析.结果:45例中有乳头和/或乳晕区糜烂、出血等湿疹样表现者40例(其中伴乳头下方肿块11例);单纯表现为乳头下肿块5例;伴同侧腋窝淋巴结肿大13例.本组25例作根治术,20例作改良根治术.派杰氏病不伴深部肿块者的5年和10年生存率分别为95.5%和78.6%,而伴有深部肿块者的5年和10年生存率分别为53.8%和36.4%,两者的5年和10年的生存率比较均有统计学意义(其P值分别为＜0.01和＜0.05);无乳头下方浸润者的5年和10年生存率分别为100%和88.9%,而有乳头下方浸润者的5年和10年生存率分别为69.6%和43.8%,前者亦优于后者(P＜0.05);腋窝淋巴结阴性者的5年和10年生存率分别为92.0%和76.5%,而阳性者则分别为50.0%和25.0%,前者同样优于后者(P＜0.05).结论:乳腺派杰氏病多数有典型的临床表现,凡有湿疹样表现者均要考虑本病的可能性;本病预后的关键在于早期治疗.不伴深部肿块、没有深部浸润和腋淋巴结阴性者的手术治疗有很好效果,伴有肿块、有深部浸润和腋淋巴结阳性者的预后差,且均有统计学意义.