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To date, pagetoid spread-the proliferation of pagetoid cells in intraepidermal lesions, as observed in secondary extramammary Paget's disease-has not been reported in squamous epithelium derived from the extension of head and neck carcinomas. Herein, we report a case of pagetoid squamous cell proliferation associated with a primary intraosseous carcinoma (PIOC) arising in the periapical lesion of the maxilla, a finding not reported previously. A 60-year-old man presented with prostate adenocarcinoma and bilateral pubic bone, ilium bone, and sacral bone metastases.

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Clinical and Pathological Analysis of Perianal Paget's Disease: A Case Report and Review of 89 Cases.

Cancer Diagn Progn

January 2025

Department of Gastroenterological Surgery, Osaka International Medical and Science Center, Osaka Keisatsu Hospital, Osaka, Japan.

Background/aim: Perianal Paget's disease (PPD) is an intraepithelial invasion of the perianal skin that is frequently associated with anorectal carcinoma. Rectal canal carcinoma with Pagetoid spread (PS) is a relatively rare disease, and few reports on its outcomes are available. The relatively rare nature of this disease makes the development of treatment recommendations difficult.

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Objectives: Urothelial carcinoma in situ (CIS) with early papillary formation is terminology sometimes used to suggest incipient high-grade papillary urothelial carcinoma (PUC) but may lead to confusion between true CIS and lateral flat spread of PUC.

Methods: It remains unclear how pathologists and urologists interpret this scenario, so a survey was circulated to 68 pathologists (group 1 = 28 academic genitourinary pathologists; group 2 = 17 pathologists with a self-reported genitourinary focus; group 3 = 23 pathologists self-reported as not genitourinary specialists) and 32 urologists.

Results: Regarding atypical urothelial lesions that appear mainly flat but contain possible papillae, group 3 was more likely to label this as CIS compared with groups 1 and 2 (35% for group 3 vs 13% for groups 1 and 2), while groups 1 and 2 more often adopted another descriptive diagnosis, such as "CIS with early papillary features" (38% for groups 1 and 2 vs 13% for group 3).

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Perianal Paget's disease (PPD) is a rare skin adenocarcinoma that arises in the apocrine glands of the perianal region. It is often misdiagnosed as eczema, leukoplakia, squamous cell carcinoma, Bowen's disease, lichen planus, or condylomata acuminata. We report a case of a 63-year-old male who presented to the emergency room (ER) for evaluation of an anal mass that had persisted over 6 months.

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Article Synopsis
  • Secondary extramammary Paget's disease is a rare condition that occurs when tumor cells spread from nearby tissues, often linked to underlying cancers of the skin, genitourinary, or gastrointestinal tracts.
  • An 80-year-old woman with a 7-year history of urothelial carcinoma developed a lesion on her labia majora, diagnosed as pagetoid spread of her carcinoma, but she chose not to have it surgically removed.
  • After receiving several treatments, including platinum-based chemotherapy and pembrolizumab, her condition improved significantly with enfortumab vedotin, leading to a successful complete resection of the tumor.
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