乳头Paget‘s病：附55例报告

Fifty-five female patients with Paget's disease of the nipple treated in our Hospital from 1958 to 1987 are reported. It comprised 1.1% of all the breast carcinomas. The lesions was on the left side in 27 and on the right side in 28. The average age was 48 years. The primary lesion was in breast ducts and then invaded the nipple, areola superficially and deeper breast tissue. Dermal manifestation of Paget's disease is easily confused with chronic dermopathy. In this series, four patients (7.2%) did not have the typical symptoms so pathologic or cytologic examination was relied upon for correct diagnosis. Thirty-one patients were treated by radical mastectomy giving a 3-year disease-free rate of 70.9%. It seems that radical mastectomy is more superior to the other surgical procedures. The 3-year recurrent rate was 38% for patients with Paget's disease of the nipple coexisting with breast carcinoma, but no recurrence was found in patients with simple Paget's disease of the nipple. The 3-year survival rate was 46.4% in patients with axillary lymph node metastasis, 85.2% in patients without, 54.3% in patients with palpable masses in the breast and 85.0% in patients without. Paget's disease of the nipple coexisting with breast carcinoma, axillary lymph node metastasis, palpable breast masses has poor prognosis.     

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.     

乳头Paget病临床病理特征分析

目的 :探讨乳头Paget病临床特点、组织发生伴发病理亚型、性激素受体表达状况等临床病理特征。方法 :回顾分析了 2 1例乳头Paget病患者临床病理资料 ,光镜下重阅分析其病理学特征 ,并采用免疫组化SP法检测ER、PR水平。结果 :2 1例乳头Paget病中 17例临床触及肿块 ,4例未触及肿块 ,全部伴乳腺导管癌 ,前者伴发浸润性导管癌 14例、早期浸润性导管癌 3例 ,后者全部为导管内癌。腋窝淋巴结转移率47 6% ( 10 /2 1)。组织学亚型与淋巴结状态关系密切 ,P <0 0 5。ER阳性率2 3 8% ( 5 /2 1) ,PR阳性率 2 8 6%( 6/2 1) ,ER、PR同时阳性 2 3 8% ( 5 /2 1)。性激素受体水平与淋巴结状态关系密切 ,P <0 0 5 ,而与伴发组织学类型无关 ,P >0 0 5。结论 :本组乳头Paget病均伴发乳腺导管癌 ,伴乳腺肿块者均为浸润性导管癌 ,淋巴结转移率高 ,ER、PR低水平表达。     

Paget’s disease of the nipple in a population based cohort

Background Paget's disease of the nipple is a rare form of breast cancer characterised by the presence of intraepidermal tumour cells. It is often associated with ductal carcinoma in situ (DCIS) and/or invasive cancer in the breast parenchyma. We have studied the presentation and symptoms of Paget's disease, local control and breast cancer corrected survival following breast conserving surgery or mastectomy. Patients and methods The study is based on 223 women with histological verified Paget's disease of the nipple diagnosed between 1976 and 2001 at 13 Swedish hospitals. All women s charts were reviewed. All recurrences and deaths were registered. A comparison was made for differences in breast cancer-corrected survival (BCS) and disease-free survival (DFS) in univariate analyses. Results The median follow-up was 12 (4-28) years. In a vast majority (98%), the main presenting symptom was eczema or ulceration of the nipple. The diagnosis of an underlying breast malignancy was established in 79% of the women before surgery. A cone excision of the nipple-areola complex was performed in 43 women and 169 women had a mastectomy. Eleven elderly women were not operated. One hundred and seventeen women had a non-invasive Paget of which 40 had an underlying DCIS. Invasive cancer was seen in 68 women. In 38 cases the histopathological report did not state if the tumour was invasive or not. Thirty-three women died from breast cancer. In operated women BCS and DFS at 10 years were 87% and 82%, respectively. The 10-year BCS for non-operated patients (n = 11) was 34%. At 10 years, the cumulative local recurrence rate was 9%, 8% among women undergoing mastectomy and 16% among those treated with breast conserving surgery. In univariate analysis the type of surgery, cone excision or mastectomy, had no statistically significant impact on BCS or DFS. Risk factors for breast cancer death and recurrence were having an underlying invasive cancer compared with an in situ carcinoma and having a palpable tumour in the breast. Conclusion The main presenting symptoms were eczema or ulceration of the nipple. Patients with non invasive Pagets disease of the nipple had an excellent cancer outcome. Selected patients with Paget's disease of the nipple were treated with breast conserving surgery with survival rates similar to those achieved with mastectomy.     

Invasive ductal carcinoma of the nipple: A case report

We report a very rare case of breast carcinoma which possibly arose from the duct of the nipple and formed a nipple mass. A 71-year-old woman presented with an elastically hard and enlarged right nipple, 3.5x 2.8 cm in size. Mammograms and ultrasonograms suggested a benign tumor of the nipple, but histological examination revealed invasive ductal carcinoma. No Paget's cells were found in the epidermis. Modified radical mastectomy was performed. Both estrogen and progesterone receptors were positive. One of the nine axillary nodes dissected had metastatic foci. To our knowledge, this case is only the second reported case of invasive ductal carcinoma originating from the nipple. Differential diagnosis and histogenesis are discussed.     

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.     

Synchronous bilateral male breast cancer: a case report

Synchronous bilateral breast cancer is extremely rare in men and has not, up to date, been reported in Korea. A 54-year-old man presented with a palpable mass in the right breast. The right nipple was retracted and bilateral axillary accessory breasts and nipples were present. On physical examination, a 2 cm-sized mass was palpated directly under the right nipple, and, with squeezing, bloody discharge developed in a single duct of the left nipple. There was no palpable mass in the left breast, and axillary lymph nodes were not palpable. Physical examination of external genitalia revealed a unilateral undescended testis on the left side. Synchronous bilateral breast cancer was diagnosed using mammography, ultrasonography, and core-needle biopsy. Histopathological examination revealed invasive ductal carcinoma in the right breast and ductal carcinoma in situ in the left breast. Bilateral total mastectomy, sentinel lymph node biopsy, and excision of accessory breasts in the axilla were performed.     

乳头湿疹样癌的诊断与治疗(附27例病例分析)

目的 探讨乳头湿疹样癌的临床表现、诊断、治疗及影响预后的因素。方法 回顾性总结1987年10月～2000年12月西安交通大学第一医院收治的27例乳头湿疹样癌病人的临床资料。结果 乳头湿疹样癌主要以乳头、乳晕区湿疹样病变及乳房肿块为首发症状。伴有乳房肿块的5年及10年生存率(53．7％，39．6％)显低于不伴有肿块的病人的5年及10年生存率(100％，88．1％)(P＜0．05)。结论 对于对症处理2周效果不佳的乳头、乳晕区湿疹样变应尽早行病灶刮片或切取活检，以明确诊断。合并有乳腺肿块的乳头湿疹样癌的首选术式为乳腺癌改良根治术或根治术。     

Paget disease of the nipple: a multifocal manifestation of higher-risk disease

BACKGROUND: The treatment of Paget disease by mastectomy has been challenged recently in favor of breast-conserving techniques. A large series of patients treated with mastectomy has been reviewed to assess the feasibility of less radical surgery. METHODS: The cases of 70 women with a clinical diagnosis of Paget disease were reviewed. The type, grade, receptor and node status, and the mammographic and pathologic extent of the underlying breast malignancy were determined. The survival of patients with invasive disease was compared with matched controls without Paget disease. RESULTS: The underlying malignancy was invasive in 58% of cases. Despite the fact that only one third of women presented with a palpable mass, the malignancy was frequently extensive, being confined to the retroareolar region in only 25% of cases. The true extent of the disease was underestimated by mammography in 43% of cases. Of the patients with ductal in situ carcinoma, 96.5% had high-grade carcinomas and 100% had invasive carcinomas of high cytonuclear grade. Overexpression of the c-erb-B2 oncogene was detectable in 83% of cases. Patients with Paget disease had a significantly worse survival than matched controls, but this difference was eliminated if they were also matched for c-erb-B2 status. CONCLUSIONS: Paget disease is often associated with extensive underlying malignancy, which is difficult to assess accurately either clinically or mammographically. As a consequence, cone excision of the nipple would have resulted in incomplete excision in 75% of cases. The underlying disease is of high grade and is frequently c-erb-B2 positive with a resulting poor prognosis. Aggressive local and systemic treatment would seem to be merited.     

Paget's disease of the breast: a study of 43 cases.

Paget's Disease of the breast is caused by spread of duct carcinoma cells along the mammary ducts to the epidermis of the nipple and areola. This is a study of 43 cases of Paget's Disease of the breast. Though only few patients presented with a lump; a carcinoma, either DCIS or IDC or both were found in all cases. The presence of an underlying breast carcinoma in Paget's Disease of the breast suggests that radical mastectomy is the treatment of choice in this condition.     

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.     

MRI accurately depicts underlying DCIS in a patient with Paget's disease of the breast without palpable mass and mammography findings

Breast-conserving therapy must be carefully indicated amongpatients with Paget's disease of the breast, because the diseaseis often associated with an underlying in situ or invasive carcinoma,even when there are no palpable mass or mammography findings.We report a 52-year-old woman who complained of skin color changeof her right nipple for 11 months. No mass was palpable in herbreasts, and mammography did not show any density or calcification.Nipple biopsy revealed Paget's disease of the breast with ductalcarcinoma in situ (DCIS) in the breast epithelium just beneaththe nipple. Magnetic resonance imaging (MRI) of the breast demonstrateddiffuse segmental enhancement in two different quadrants. Accordingto the pattern of enhancement, the lesions depicted by MRI werediagnosed as an extensively spreading type of DCIS. Based oninformed consent, the patient received a total mastectomy. Thehistopathological examination demonstrated non-invasive ductalcarcinoma with comedo-necrosis. The histological mapping withsubserial sectioning demonstrated an extent of the lesions thatcorresponded accurately to the lesions defined by MRI. We concludethat MRI may play an important role in selecting candidatesfor breast-conserving therapy out of those patients with mammaryPaget's disease with no clinical evidence of an underlying breastcarcinoma.     

Paget disease of the breast: analysis of 41 patients

The treatment for the patients with Paget disease of the breast is controversial. This review of its natural history, treatment approach, and clinical outcome will help to formulate treatment. Forty-one patients with a diagnosis of Paget disease of the breast were retrospectively reviewed at Providence Hospital & Medical Centers from 1980 to 1999. Ninety-eight percent of patients had underlying carcinoma (ductal carcinoma in situ and/or invasive ductal cancer). Patients with a palpable mass have a much higher incidence of invasive ductal cancer, positive lymph node, and a worse survival rate. The median length of follow-up was 42 months (range: 6--200 months). Twenty-seven percent of patients (11/41) had conservative operations, including 1 patient with a palpable mass; 10 patients with no palpable mass; and 3 patients with recurrence after conservative operation. Thirty-seven percent of patients received adjuvant therapy. Paget disease of the breast has very high incidence of underlying carcinoma (100% in a palpable mass, 96% in nonpalpable mass). Patients with a palpable mass have a worse survival than do patients with nonpalpable mass. Conservative operation should cautiously be selected even for patients with no palpable mass because of a higher recurrence rate.     

Occult breast carcinoma: a report of four cases and review of literature

We report four occult carcinoma breast cases in which extensive axillary node metastases was the first manifestation. Upper outer quadrentectomy with axillary dissection was done in three patients while modified radical mastectomy was done in one. Primary tumor could be found in three patients, one had squamous cell carcinoma (SCC) & two had infiltrating duct carcinoma (IDC). However primary tumor was not detected in breast tissue of the fourth patient. Extensive lymph node metastases were found in three out of 14,15 out of 15(SCC), 24 out of 24 and 1 out of three axillary nodes respectively. Results of immunohistochemical staining for estrogen and progesterone receptors on three cases were negative. All patient received postoperative radiotherapy and chemotherapy. We have reviewed the literature and discussed the approach to diagnosis and management in female patients presenting with metastatic carcinoma in the axillary nodes with emphasis on the appropriate pre-treatment evaluation.     

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.     

Paget's disease of the nipple without detectable breast tumor: conservative management with radiation therapy

Between 1960 and 1984, 20 selected patients with Paget's disease of the breast confined to the nipple were treated conservatively with radiotherapy alone (17/20 pts) or limited surgery and radiotherapy (3/20 pts). Median follow-up was 7.5 years. No patients died of breast disease. Three patients had recurrence in the treated breast, and were treated by mastectomy. All recurrences were located in the nipple or areola and were all Paget's disease, without associated intraductal or invasive carcinoma. No axillary node recurrences occurred. The actuarial 7-year probability of living free of disease with breast preserved was 81%. Among the 15 patients who had a minimum follow-up of 3 years, without recurrence, 12 (80%) had a good cosmetic result. These results suggest that radiation therapy could be an effective alternative to radical surgery in the treatment of patients with Paget's disease of the nipple without concomitant breast tumor.     

Paget disease of the breast—an easily overlooked disease?

BACKGROUND AND OBJECTIVES: Paget disease of the breast has long been recognized as a distinct clinical disease. The clinical manifestations and outcomes of Paget disease of the breast were reviewed to understand its earlier diagnostic clues and achieve an optimal treatment plan. Patients and Methods Thirty-one patients with Paget disease of the breast were included in this study. The postoperative outcomes and possible related prognostic factors were reviewed and analyzed. RESULTS: The 5-year overall survival was 69%. Nineteen patients (61%, Group A) did not have a palpable breast mass and 12 patients (39%, Group B) had a palpable breast mass. There was significant difference (P < 0.01) in the 5-year overall survival between Group A (94%) and Group B (19%). Group A patients had significantly higher incidences of underlying noninvasive breast carcinoma (73% vs. 8%, P < 0.01) and n0 lymph nodes status (84%vs. 50%, P = 0.043) than those of Group B. CONCLUSIONS: Paget disease of the breast without a palpable breast mass carries a more favorable prognosis. Patients with any nipple complaints deserve a detailed evaluation even in the absence of a palpable breast mass in order not to overlook a favorable disease.     

Recurrence of breast carcinoma after breast conserving treatment

Clinicopathologic data were analysed of 15 patients with recurrence of carcinoma after breast conserving treatment and who underwent salvage mastectomy with curative intent. Twelve recurred in the same site as the original tumor after an average interval of 29 months; the remaining three arose elsewhere in the breast after an average, 83 months. Ten patients presented with palpable lumps, one with Paget's disease of the nipple and four with mammographical microcalcifications. Thirteen patients are alive without evidence of disease (mean follow-up 20 months), one patient died of chest wall recurrence 10 months after mastectomy and one is alive with metastatic disease 9 months after mastectomy. Extensive ductal carcinoma in situ, which was found in 53% of the initial excisions in this series, may be associated with a high risk of local recurrence.     

Conservative management of Paget disease of the breast with radiotherapy: 10- and 15-year results

BACKGROUND: At 5-year follow-up, patients with Paget disease of the breast who were treated with breast-conserving surgery (BCS) and radiotherapy (RT) had excellent results. The current report provides 10- and 15-year rates of tumor control in the breast, as well as disease-free and overall survival rates following BCS and RT in a cohort of patients with Paget disease presenting without a palpable mass or mammographic density. METHODS: Through a collaborative review of patients treated with BCS and RT from seven institutions, 38 cases of Paget disease of the breast presenting without a palpable mass or mammographic density were identified. All patients had pathologic confirmation of typical Paget cells at time of diagnosis. Thirty-six of 38 patients had a minimum follow-up greater than 12 months and constitute the study cohort. Ninety-four percent of patients underwent complete or partial excision of the nipple-areola complex and all patients received a median external beam irradiation dose of 50 Gy (range, 45-54 Gy) to the whole breast. Ninety-seven percent of patients also received a boost to the remaining nipple or tumor bed, a median total dose of 61.5 Gy (range, 50.4-70 Gy). RESULTS: With median follow-up of 113 months (range, 18-257 months), 4 of 36 patients (11%) developed a first recurrence of disease in the treated breast only. Two of the four recurrences in the breast were ductal carcinoma in situ (DCIS) only and two were invasive with DCIS. Two additional patients had a recurrence in the breast as a component of first failure. Actuarial local control rates for the breast as the only site of first recurrence were 91% at 5 years (95% confidence interval [CI], 80-100%) and 87% (95% CI, 75-99%) at both 10 and 15 years. Actuarial local control rates for breast recurrence, as a component of first failure, were 91% (95% CI, 80-100%), 83% (95% CI, 69-97%), and 76% (95% CI, 58-94%) at 5, 10, and 15 years, respectively. No clinical factors were identified as significant predictors for breast recurrence. Five-, 10- and 15-year actuarial rates for survival without disease of 97% (95% CI, 90-100%) and 5-, 10-, and 15-year actuarial rates of overall survival of 93% (95% CI, 84-100%) at 5 years and 90% (95% CI, 78-100%) at 10 and 15 years were reported. CONCLUSIONS: These data confirm excellent rates of local control, disease-free survival, and overall survival at 10 and 15 years following BCS and RT for Paget disease of the breast. This study continues to support the recommendation of local excision and definitive breast irradiation as an alternative to mastectomy in the treatment of patients with Paget disease presenting without a palpable mass or mammographic density.