Mammary Paget's disease and radiotherapy: a systematic literature review.

OBJECTIVE: Paget disease of the breast (PDB) is a rare form of cutaneous breast cancer. Up to date, no randomized studies evaluated the different management strategies. This systematic review investigates the role of radiotherapy and its best technical profile in the treatment of this disease, with great attention to doses and fractionation regimens. MATERIALS AND METHODS: A systematic search was performed on PubMed, Embase and Scopus in order to detect case reports, case series and prospective as well as retrospective clinical studies describing histologically proven PDB and providing information about pertinent radiation treatments. Searching strategy followed PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analysis) guidelines. RESULTS: Inclusion criteria were met by six papers, dealing with radiation treatments performed postoperatively and in exclusive settings. No evaluations were performed on preoperative radiotherapy. CONCLUSIONS: Actually, the standard treatment of PDB reflects oncological principles of breast carcinoma therapy, including the role of breast-preserving surgery. The traditional radiotherapic dose is 50 Gy, with daily fractionation of 2 Gy. Adjuvant radiotherapy following breast preserving surgery represents the current standard of care; prospective studies could be of help in defining the role of exclusive radiotherapy, hypofractionated schemes and smaller target volumes.

Modern Trends in the Surgical Management of Paget's Disease.

PURPOSE: We examined the incidence and modern national trends in the management of Paget's disease (PD), including the use of breast-conserving surgery (BCS), mastectomy, axillary surgery, and receipt of radiotherapy. METHODS: Using surveillance, epidemiology and end results (SEER) data, we identified 2631 patients diagnosed with PD during 2000-2011. Of these patients, 185 (7%) had PD of the nipple only, 953 (36.2%) had PD with ductal carcinoma in situ (PD-DCIS), and 1493 (56.7%) had PD with invasive ductal carcinoma (PD-IDC). Trends in age-adjusted incidence, primary surgery, sentinel lymph node biopsy (SLNB), and axillary lymph node dissection were examined. Multivariable logistic regression was used to evaluate factors associated with receipt of BCS and radiotherapy. RESULTS: A decrease in the age-adjusted incidence of PD occurred from 2000 to 2011 (-4.3% per year, p < 0.05). The overall rates of mastectomy in the PD only, PD-DCIS, and PD-IDC groups were 47, 69, and 88.9%, respectively. Only in the PD-IDC group did the proportion of patients undergoing BCS increase significantly, from 8.5% in 2000 to 15.7% in 2011 (p = 0.01). Of those who underwent axillary surgery, the proportion of patients undergoing SLNB increased from 2000 to 2011. In adjusted analyses, Paget's subgroup, older age, central tumor location, low/intermediate grade, tumor size <2.0 cm, SEER region, and year of diagnosis after 2006 were significantly associated with receipt of BCS. CONCLUSIONS: The incidence of Paget's disease has decreased over time while modern trends in local therapy suggest that BCS, SLNB, and adjuvant radiotherapy remain underutilized.

Papel de la radioterapia en el tratamiento del carcinoma in situ de mama / Role of radiotherapy for the treatment of breast in situ carcinoma

Como consecuencia de los programas de cribado o despistaje mamográficos, la incidencia de tumoresde mama no invasivos ha aumentado de forma sorprendente. Sin embargo, cual debe ser el tratamientode elección para este tipo de tumores no ha sido bien establecido y ha dejado la controversia dela mejor opción terapéutica a realizar, la mastectomía o el tratamiento conservador. Se ha realizadouna revisión de la literatura, así como, una discusión de las alternativas de tratamiento posibles en elabordaje terapéutico del carcinoma in situ de mama, en especial del papel de la radioterapia

As a result of mammographic screening programs, the incidence of diagnosed non-invasive breastcarcinomas has increased markedly. Nevertheless, the elective treatment for these kind of tumours hasnot yet been established, leading to controversies to decide about which is the best therapeutic option,whether mastectomy or conserving therapy. We make a review of the literature, and discuss thedifferent treatment options for breast carcinoma in situ, especially the role of radiation therapy

Breast conservation surgery using nipple-areolar resection for central breast cancers.

HYPOTHESIS: Resection of the nipple-areolar complex (NAC) for central breast cancers that involve the nipple or areola, with postoperative radiation therapy, adheres to the oncologic principles established for breast conservation surgery of other breast cancers. Good or excellent cosmetic results can be achieved. The rate of ipsilateral breast recurrence will be similar to that seen with peripheral breast cancers. The indications for breast conservation surgery can be safely extended to include patients with breast cancers that involve the NAC. DESIGN: Retrospective medical record review; follow-up patient questionnaire. SETTING: Community teaching hospital. PATIENTS: Fifteen patients, aged 46 to 88 years, whose central breast cancers involved the NAC precluding preservation of the NAC. INTERVENTIONS: Nipple-areolar complex resection, postoperative radiation therapy. MAIN OUTCOME MEASURES: Ipsilateral breast recurrence, survival, cosmesis. RESULTS: Ten patients had subareolar cancers that directly involved the nipple or areola; 5 patients had Paget disease of the nipple. Average tumor size was 1.6 cm (range, 0.2-3.5 cm). With a mean follow-up of 32 months (range, 4-109 months), there has been only 1 recurrence (7%), which was treated successfully by modified radical mastectomy. All 15 patients are alive and free of disease. Cosmetic results are satisfactory to excellent, as judged by both the patients and the surgeons. CONCLUSIONS: Nipple-areolar complex resection for central subareolar cancers that directly involve the NAC, as well as for Paget disease of the nipple, extends the indications for breast conservation in other areas of the breast, and with acceptable cosmesis.

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.

Enfermedad de paget de mama en un varón asociada a un carcinoma intraductal subyacente / Paget's disease of the male breast associated with intraductal carcinoma

Varón de 79 años que presenta, en la areola mamaria izquierda, lesiones eccematosas. El estudio histopatológico de una biopsia revela hallazgos diagnósticos de enfermedad de Paget y carcinoma intraductral subyacente. Se realiza mastectomía radical modificada y terapia adyuvante con tamoxifeno y radioterapia. (AU)

Surgical treatment in Paget's disease of the breast.

The extent of the operation in Paget's disease of the breast depends largely on the degree of clinical advancement of neoplasm as well as counter-indications for breast conserving therapy. Despite the increasing use of breast conserving therapy, rather few such operations of this kind have been performed in our department. We have carried out 29 mastectomies and only 6 breast conserving operations. No matter what type of therapy was applied the percentages of 5-year survivals for the patients with non-invasive component and for the ones with invasive component were respectively 89.76% and 49.32%.

Enfermedad de Paget mamaria / Paget's disease of breast

Presentamos un caso de enfermedad de Paget mamario con estudio de los aspectos clínicos, histopatológicos, radiológicos y el tratamiento quirúrgico con terapia sistémica adyuvante. Se presenta esta patología poco frecuente para revisar su diagnóstico diferencial y el procedimiento adecuado para enfrentarlo y mejorar la calidad de vida del paciente.

The conservative management of Paget's disease of the breast with radiotherapy.

BACKGROUND: The purpose of this study was to evaluate the feasibility of breast-conserving therapy involving limited surgery and definitive radiotherapy as a treatment for Paget's disease, and to determine the disease free and overall survival associated with this approach. METHODS: The authors retrospectively reviewed the charts of all patients treated during the period 1980-1994 for Paget's disease of the breast who did not present with a palpable mass or mammographic density. Through a collaborative review, 30 cases were identified. A biopsy confirming the presence of typical Paget's cells was performed on all patients. All patients received external beam radiotherapy to the breast, with a median dose of 50 gray (Gy). Ninety-seven percent received a boost to the remaining nipple or tumor bed, with a median dose to the tumor bed of 61.5 Gy. RESULTS: The median follow-up for surviving patients was 62 months. Three patients (10%) developed a recurrence in the breast as the only site of first failure, and 2 additional patients (7%) experienced failure in the breast as a component of first failure. The median time to local failure was 69 months. The 5- and 8-year actuarial estimates of local failure as the only site of first failure were 9% (95% confidence interval [CI], 0-20%) and 16% (95% CI, 0-31%), respectively. Of the 5 patients with local failures, 3 were among 22 patients (14%) who underwent complete resection of the nipple or nipple-areola complex, compared with 2 failures among 6 patients (33%) after partial resection (P = 0.29). There were no failures among 2 patients who had a biopsy only. Four of 5 local failures were salvaged by mastectomy, and 3 of these patients were free of disease after a median follow-up of 52 months. The 5- and 8-year estimates of disease free survival for the overall series were both 95% (95% CI, 87-100%); cause specific overall survival was 100% at 8 years. CONCLUSIONS: Breast-conserving therapy involving complete resection of the nipple-areola complex followed by definitive radiotherapy is a viable alternative to mastectomy in the treatment of Paget's disease. High rates of disease free and cause specific survival, in addition to adequate local control, justify consideration of a conservative approach.

The Edinburgh randomized trial of axillary sampling or clearance after mastectomy.

Between January 1980 and October 1983, 417 patients were randomized for mastectomy followed by axillary node sampling or full axillary clearance. The aim of the study was to determine whether a standard 'four-node' axillary sample, followed by careful dissection of removed tissue, could accurately indicate the extent of local treatment required. Axillary radiotherapy was given only to patients with histological involvement of sampled nodes and not to any having axillary clearance. The incidence of involved nodes was similar for both groups, as were distant relapse and survival rates. Currently 62.6 percent are alive after clearance and 65.0 percent after sampling. A non-significant increase in the rate of locoregional relapse was observed for those treated by axillary node clearance, this being due mainly to increased relapse on the unirradiated chest wall (clearance 21 percent versus sampling 12 percent in patients with node-positive disease). There was only a minor difference in axillary relapse, favouring axillary clearance (3.0 versus 5.4 percent). In patients with operable breast cancer, mastectomy with axillary node sampling gives equal control to mastectomy with axillary node clearance but, as morbidity is greater, surgical clearance of the axilla is the preferred option.

The place for conservative treatment in the management of Paget's disease of the nipple.

Four cases of Paget's disease confined to the nipple are presented. Three of them were treated conservatively by local radiotherapy and the fourth was treated by mastectomy. Histopathological examination of the breast in the last case failed to show any evidence of in situ or invasive carcinoma. The first three patients are well and without evidence of recurrence 3 to 5.5 years after treatment. Could conservative treatment in the form of local excision or radiotherapy offer an alternative to mastectomy? A discussion on the place of conservative treatment is presented.

A case report of Paget's disease of the breast treated with radiotherapy alone.

Paget's disease of the breast is a rare form of breast cancer. Patients with nipple-areolar disease alone and no palpable mass have an excellent prognosis. Although there is some argument about the treatment of this type of Paget's disease, only two reports have been published on the role of conservative treatment. This report discusses a case of limited Paget's disease of the nipple and areola of the breast which was treated by radiation alone without limited surgery.

Breast conserving treatment of Paget's disease.

Between 1971 and 1984, 13 patients with histologically proven Paget's disease were treated conservatively with radiotherapy only. The disease was clinically confined to the nipple or surrounding skin, without signs of an underlying tumor. With a mean follow-up of 58.6 months (ranging between 15 and 118 months), and a median follow up of 52 months, no recurrences locally or at distance were seen. Therefore in these selected cases a mastectomy could be avoided. The results with this breast conserving management suggest a place for radiotherapy in the treatment of Paget's disease limited to the nipple.

Radiotherapy for Paget's disease of the nipple: a conservative alternative.

The case records of 28 patients with Paget's disease of the nipple treated by radio-therapy alone were reviewed retrospectively. 16 of 19 patients who had no palpable underlying tumour and who were mammographically normal at the time of original treatment remain free of disease with a median follow-up of 5 years 3 months. In this selected group, radical radiotherapy with small fields localised to the involved skin is an effective alternative to mastectomy.