Paget disease of the nipple - A multifocal manifestation of higher-risk disease
Paget disease of the nipple - A multifocal manifestation of higher-risk disease
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DOI：
10.1002/cncr.10638
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发表时间：
2002-07-01
期刊：
影响因子：
6.2
通讯作者：
Ryder, K
中科院分区：
文献类型：
--
作者：
Kothari, AS;Beechey-Newman, N;Ryder, K
背景。通过乳房切除术治疗佩吉特病最近受到了挑战，支持乳房保留技术。我们回顾了大量接受乳房切除术的患者，以评估非根治性手术的可行性。本文回顾了70例临床诊断为Paget病的妇女的病例。确定类型，分级，受体和淋巴结状态，以及乳腺恶性肿瘤的乳房x线摄影和病理程度。将侵袭性疾病患者的生存率与未患Paget病的对照组进行比较。在58%的病例中，潜在的恶性肿瘤是侵袭性的。尽管只有三分之一的女性表现为可触及的肿块，但恶性肿瘤通常是广泛的，仅25%的病例局限于乳晕后区域。在43%的病例中，乳房x光检查低估了疾病的真实程度。在导管原位癌患者中，96.5%为高级别癌，100%为高细胞核级别浸润性癌。在83%的病例中检测到c- erbb - b2癌基因过表达。Paget病患者的生存率明显低于匹配的对照组，但如果他们也匹配c- erbb - b2状态，则这种差异被消除。Paget病通常与广泛的潜在恶性肿瘤相关，无论是临床还是乳房x光检查都难以准确评估。因此，乳头锥体切除在75%的病例中会导致不完全切除。潜在的疾病是高级别的，经常是c- erbb - b2阳性，导致预后不良。积极的局部和系统治疗似乎是值得的。(C) 2002年美国癌症协会。
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. (C) 2002 American Cancer Society.