Extramammary Paget’s Disease: Summary of Current Knowledge

Lam, Christina; Funaro, Deana

doi:10.1016/j.det.2010.08.002

Cited by 175 publications

(240 citation statements)

References 119 publications

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“…3 Epidemiological studies have reported that the majority of extramammary Paget's disease patients are older adults, ranging in age from 50 to 80 years old and having an average age of 65 years. 4 Caucasian female patients are the most frequently reported population; however, in Asian populations, there is a male predominance for this disease. 4,5 Although some familial extramammary Paget's disease cases have been reported, 6 the epigenetic and genetic abnormalities that contribute to the pathogenesis of this rare disease remain poorly understood.…”

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confidence: 99%

Correlation of DLC1 gene methylation with oncogenic PIK3CA mutations in extramammary Paget's disease

Kang

Zhang

et al. 2012

Modern Pathology

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Extramammary Paget's disease is a rare cutaneous malignant neoplasm. The genetic and epigenetic mechanisms underlying its pathology remain unknown. In this study, we investigated the expression levels, and mutation and methylation status of a common tumor suppressor gene, deleted in liver cancer 1 (DLC1), and an oncogene, PIK3CA, in tumor (n ¼ 132) and normal tissues (n ¼ 20) from unrelated patients. The presence of epigenetic and genetic lesions was then correlated to the patient pathology data to determine the potential role of these genes in extramammary Paget's disease etiology and progression. The DLC1 gene was found to be downregulated in 43 (33%) tumors, as compared with immunohistochemistry results from normal tissues. Methylation-sensitive, high-resolution melting analysis indicated that the DLC1 promoter was hypermethylated in 51 (39%) extramammary Paget's disease tumors. This hypermethylation was associated with significantly decreased DLC1 levels (P ¼ 0.011), and had a strong positive correlation with advanced age (P ¼ 0.002). PIK3CA mutations were detected by direct sequencing in 32 (24%) tumors, the majority of which were invasive. Furthermore, PIK3CA mutations significantly correlated with DLC1 hypermethylation. Thus, aberrant DLC1 methylation and PIK3CA mutations may have important roles in extramammary Paget's disease pathogenesis, and may represent potential molecular targets for therapy.

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confidence: 99%

Correlation of DLC1 gene methylation with oncogenic PIK3CA mutations in extramammary Paget's disease

Kang

Zhang

et al. 2012

Modern Pathology

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“…It is considered to have a poor prognosis if there is an underlying carcinoma, which is the case in most of the patients [7,8]. Extramammary Paget's disease is most frequently observed at the genital and perianal region [9]. Paget's disease is recognized by observation of malignant cells at the otherwise nonneoplastic epidermis or mucosal epithelium, frequently at the basal layer and sometimes spreading into the superficial layers either as single cells or clusters, sometimes forming glandular structures [7][8][9].…”

Section: Discussionmentioning

confidence: 99%

“…Extramammary Paget's disease is most frequently observed at the genital and perianal region [9]. Paget's disease is recognized by observation of malignant cells at the otherwise nonneoplastic epidermis or mucosal epithelium, frequently at the basal layer and sometimes spreading into the superficial layers either as single cells or clusters, sometimes forming glandular structures [7][8][9]. Immunohistochemistry is helpful as the malignant cells present different expression of antigens from the non-neoplastic epithelial cells [9].…”

Section: Discussionmentioning

confidence: 99%

“…Paget's disease is recognized by observation of malignant cells at the otherwise nonneoplastic epidermis or mucosal epithelium, frequently at the basal layer and sometimes spreading into the superficial layers either as single cells or clusters, sometimes forming glandular structures [7][8][9]. Immunohistochemistry is helpful as the malignant cells present different expression of antigens from the non-neoplastic epithelial cells [9]. ''Pagetoid spread'' is used to define intraepithelial spread of cancer cells, when a massive carcinoma is identified beneath the basal membrane [3][4][5][6][7].…”

Section: Discussionmentioning

confidence: 99%

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Undifferentiated Laryngeal Carcinoma with Pagetoid Spread

Sarıoğlu

Doğan

Şahi̇n

et al. 2015

Head and Neck Pathol

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Pagetoid spread, is used to define intraepithelial spread of cancer cells, when a massive carcinoma is identified beneath the basal membrane. There are only few reports of pagetoid spread at the head and neck region. Herein a 74 year old male patient with bilateral transglottic laryngeal high grade malignant epithelial tumor with pagetoid spread is presented. The tumor was located at the submucosa and there was spread of the CK7 and CK19 positive tumor cells into the non neoplastic mucosa, which was CK5/6 positive, sparing the basement membrane, creating a typical pagetoid pattern. Radiographic and positron emission tomography scan examination of the patient was unremarkable at presentation other than the laryngeal and neck lesions; but extensive systemic metastasis developed at 6 months following operation. To the best of our knowledge no epithelial malignancy with pagetoid spread was described at the larynx. Pagetoid spread may be a hallmark of very aggressive behavior in laryngeal carcinoma.There are only a few reports of pagetoid spread at the head and neck region. Herein a 74-year old male patient with bilateral undifferentiated transglottic laryngeal carcinoma with pagetoid spread is presented. The patient developed extensive systemic metastasis at 6 months following operation. Pagetoid spread may be an overlooked hallmark of very aggressive behavior in laryngeal carcinoma.

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“…Cutaneous Paget's disease is a rare intraepithelial neoplastic condition [1,2]. The breast Paget's disease can be associated with invasive and in situ breast cancer and is a well-defined entity.…”

Section: Introductionmentioning

confidence: 99%