Extramammary Paget disease

Prok, Lori; Fitzpatrick, James E.

doi:10.1016/b978-0-7020-6912-3.00081-1

Cited by 2 publications

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“…2 EMPD is a rare adenocarcinoma histopathologically characterised by a predominant intraepithelial growth of neoplastic cells. 1 EMPD is an uncommon malignancy accounting for 6.5% of all cases of Paget disease. It usually develops in older adults, with the mean age at diagnosis being 60-70 years.…”

Section: Discussionmentioning

confidence: 99%

“…Clinically, EMPD classically presents as a relatively well-demarcated flat or slightly elevated, scaly, oozing, erythematous patch or plaque accompanied by pruritus or burning, which often mimics an inflammatory condition leading to significant delay in diagnosis. [1][2][3] The correct diagnosis requires a biopsy with histological examination. Histopathologically, EMPD is typically characterised by single or small aggregates of cells with abundant pale cytoplasm and large pleomorphic nuclei, known as Paget cells, scattered throughout the epidermis.…”

Section: Discussionmentioning

confidence: 99%

“…Immunohistochemically, EMPD typically shows positive staining for cytokeratin 7, cytokeratin CK8/18, epithelial membrane antigen, gross cystic disease fluid protein 15 and carcinoembryonic antigen. [1][2][3]11 The pathogenesis of primary EMPD is uncertain. The most accepted theory is that primary EMPD arises as an intraepidermal neoplasm originating from cells of apocrine gland ducts or pluripotent keratinocyte stem cells.…”

Section: Discussionmentioning

confidence: 99%

“…

Paget disease is a rare disease characterised by the presence of malignant glandular epithelial cells (Paget cells) within the squamous epithelium. 1 This entity is generally classified into mammary Paget disease and extramammary Paget disease (EMPD). Mammary Paget disease affects the skin of the nipple-areolar complex, and EMPD occurs at non-mammary sites, mainly on the apocrine-rich skin of the vulva and perianal areas.

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“…The correct diagnosis requires histopathological examination and is often supported by immunohistochemical analysis.Once diagnosis has been confirmed, an extensive search for an underlying malignancy must be conducted, because of different treatment approach and prognosis for primary and secondary EMPD. [1][2][3] Oral involvement of EMPD is very rare. To the best of our knowledge, there have been only seven reported cases of EMPD involving oral mucosa in the English literature.…”

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Extramammary Paget disease of the buccal mucosa in an elderly patient

et al. 2023

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Objective To report a rare case of oral extramammary Paget disease in an elderly patient. Background Extramammary Paget disease is a rare cutaneous malignancy, and oral mucosa involvement is extremely rare. Material and method The patient was a 72‐year‐old man with a whitish plaque and areas of erosion on the right buccal mucosa. Results An incisional biopsy was performed, and the diagnosis was extramammary Paget disease. Conclusion Both clinicians and pathologists should be aware of this disease to avoid misdiagnoses with other oral benign or malignant lesions.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Extramammary Paget disease of the buccal mucosa in an elderly patient

et al. 2023

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A case of endoscopic submucosal dissection of esophageal acantholytic Paget’s disease with discontinuously spreading and unclear neoplastic extension

Satomi,

Ishiguro,

Murayama

et al. 2024

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Background Paget's disease (PD) is a carcinoma, in which irregular atypical cells with abundant cytoplasm proliferate mainly within the epithelium and is classified into PD occurring in the breast and extramammary Paget's disease (EMPD) occurring outside the breast. Essentially, extramammary PD is reported as a tumor for which it is difficult for surgeons to properly determine the line of resection. Case presentation An 83-year-old male was admitted to our hospital because of roughness of the esophageal epithelium during the follow-up examination for a gastric ulcer. A preoperative biopsy revealed squamous cell carcinoma; therefore, endoscopic submucosal dissection (ESD) was performed. Conclusions The characteristic feature in this patient was the distribution of tumor cells and, accordingly, the difficulty in identifying the neoplastic distribution. In this patient, the odd distribution and growth pattern of the tumor cells made it difficult for the operator to identify the distribution of the lesion preoperatively.

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Extramammary Paget disease

Cited by 2 publications

References 0 publications

Extramammary Paget disease of the buccal mucosa in an elderly patient

Extramammary Paget disease of the buccal mucosa in an elderly patient

A case of endoscopic submucosal dissection of esophageal acantholytic Paget’s disease with discontinuously spreading and unclear neoplastic extension

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