Invasive acantholytic anaplastic extramammary Paget disease: A previously unreported neoplasm in the vulva and review of the literature
This report describes the first documented invasive acantholytic anaplastic extramammary Paget disease (AAEMPD) of the vulva. An 87-year-old female presented with a recurrent vulvar lesion refractory to topical imiquimod and treated with multiple wide local excisions (WLE). Microscopic examination o...
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Elsevier
2024-12-01
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| Series: | Gynecologic Oncology Reports |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2352578924001930 |
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| author | Flora Mae G. Sta. Ines Julia R. Salinaro Mary Marchese Cara A. Mathews M. Ruhul Quddus |
| author_facet | Flora Mae G. Sta. Ines Julia R. Salinaro Mary Marchese Cara A. Mathews M. Ruhul Quddus |
| author_sort | Flora Mae G. Sta. Ines |
| collection | DOAJ |
| description | This report describes the first documented invasive acantholytic anaplastic extramammary Paget disease (AAEMPD) of the vulva. An 87-year-old female presented with a recurrent vulvar lesion refractory to topical imiquimod and treated with multiple wide local excisions (WLE). Microscopic examination of the final WLE specimen revealed unique histologic features, primarily supra-basal intraepidermal acantholysis with epidermal papillomatosis and hyperkeratosis. The epidermis, composed of two distinct cell populations, exhibited full-thickness atypia. Paget cells with high mitotic activity were present in the basal and parabasal layers surrounding benign squamous cells in the mid-squamous mucosa. The histologic features were suspicious of the EMPD involving a warty lesion and/or invasive squamous cell carcinoma. In addition to the intraepidermal component, dermal invasion was also present with lymphovascular space invasion. Immunohistochemical studies (KRT7, HER2, and GATA3 reactivity in Paget cells, p63 negativity, and rare mucin in Paget cells) supported the diagnosis of acantholytic anaplastic EMPD. AAEMPD, a rare variant of EMPD, shares similar prognosis and behavior with the classic Paget disease. Recognition and accurate diagnosis of this subtype is crucial for optimal patient management, given distinct treatment strategies compared with other entities in the differential diagnosis. |
| format | Article |
| id | doaj-art-6079e90bf33e4daa8050d36ae34f97d4 |
| institution | OA Journals |
| issn | 2352-5789 |
| language | English |
| publishDate | 2024-12-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Gynecologic Oncology Reports |
| spelling | doaj-art-6079e90bf33e4daa8050d36ae34f97d42025-08-20T01:55:42ZengElsevierGynecologic Oncology Reports2352-57892024-12-015610151410.1016/j.gore.2024.101514Invasive acantholytic anaplastic extramammary Paget disease: A previously unreported neoplasm in the vulva and review of the literatureFlora Mae G. Sta. Ines0Julia R. Salinaro1Mary Marchese2Cara A. Mathews3M. Ruhul Quddus4Department of Pathology and Laboratory Medicine, Women and Infants Hospital, Providence, RI 02905, United States; The Warren Alpert Medical School of Brown University, Providence, RI 02903, United States; Corresponding author at: Department of Pathology and Laboratory Medicine, Women & Infants Hospital, 101 Dudley St, Providence, RI, 02905, United States.Program in Women’s Oncology, Women and Infants Hospital, Providence, RI 02905, United States; The Warren Alpert Medical School of Brown University, Providence, RI 02903, United StatesProgram in Women’s Oncology, Women and Infants Hospital, Providence, RI 02905, United States; The Warren Alpert Medical School of Brown University, Providence, RI 02903, United StatesProgram in Women’s Oncology, Women and Infants Hospital, Providence, RI 02905, United States; The Warren Alpert Medical School of Brown University, Providence, RI 02903, United StatesDepartment of Pathology and Laboratory Medicine, Women and Infants Hospital, Providence, RI 02905, United States; The Warren Alpert Medical School of Brown University, Providence, RI 02903, United StatesThis report describes the first documented invasive acantholytic anaplastic extramammary Paget disease (AAEMPD) of the vulva. An 87-year-old female presented with a recurrent vulvar lesion refractory to topical imiquimod and treated with multiple wide local excisions (WLE). Microscopic examination of the final WLE specimen revealed unique histologic features, primarily supra-basal intraepidermal acantholysis with epidermal papillomatosis and hyperkeratosis. The epidermis, composed of two distinct cell populations, exhibited full-thickness atypia. Paget cells with high mitotic activity were present in the basal and parabasal layers surrounding benign squamous cells in the mid-squamous mucosa. The histologic features were suspicious of the EMPD involving a warty lesion and/or invasive squamous cell carcinoma. In addition to the intraepidermal component, dermal invasion was also present with lymphovascular space invasion. Immunohistochemical studies (KRT7, HER2, and GATA3 reactivity in Paget cells, p63 negativity, and rare mucin in Paget cells) supported the diagnosis of acantholytic anaplastic EMPD. AAEMPD, a rare variant of EMPD, shares similar prognosis and behavior with the classic Paget disease. Recognition and accurate diagnosis of this subtype is crucial for optimal patient management, given distinct treatment strategies compared with other entities in the differential diagnosis.http://www.sciencedirect.com/science/article/pii/S2352578924001930Extramammary Paget diseaseAcantholytic anaplasticInvasiveVulvaRecurrent |
| spellingShingle | Flora Mae G. Sta. Ines Julia R. Salinaro Mary Marchese Cara A. Mathews M. Ruhul Quddus Invasive acantholytic anaplastic extramammary Paget disease: A previously unreported neoplasm in the vulva and review of the literature Gynecologic Oncology Reports Extramammary Paget disease Acantholytic anaplastic Invasive Vulva Recurrent |
| title | Invasive acantholytic anaplastic extramammary Paget disease: A previously unreported neoplasm in the vulva and review of the literature |
| title_full | Invasive acantholytic anaplastic extramammary Paget disease: A previously unreported neoplasm in the vulva and review of the literature |
| title_fullStr | Invasive acantholytic anaplastic extramammary Paget disease: A previously unreported neoplasm in the vulva and review of the literature |
| title_full_unstemmed | Invasive acantholytic anaplastic extramammary Paget disease: A previously unreported neoplasm in the vulva and review of the literature |
| title_short | Invasive acantholytic anaplastic extramammary Paget disease: A previously unreported neoplasm in the vulva and review of the literature |
| title_sort | invasive acantholytic anaplastic extramammary paget disease a previously unreported neoplasm in the vulva and review of the literature |
| topic | Extramammary Paget disease Acantholytic anaplastic Invasive Vulva Recurrent |
| url | http://www.sciencedirect.com/science/article/pii/S2352578924001930 |
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