A review of 14 cases of perianal Paget’s disease: Characteristics of anorectal cancer with Pagetoid spread
Abstract Background: Perianal Paget's disease (PPD) is intraepithelial invasion of the perianal skin and is frequently associated with underlying anorectal carcinoma. The relatively rare nature of this disease has made it difficult to develop treatment recommendations. This study aimed to clarify the clinicopathological characteristics, treatment options that allow preservation of anorectal function, and long-term results.Methods: The National Cancer Center Hospital database was searched for all cases of perianal Paget's disease diagnosed between 2006 and 2021. In the 14 cases identified, we reviewed the diagnosis, management, and outcomes of adenocarcinoma with Pagetoid spread, including suspected or recurrent cases.Results: All 14 cases met the inclusion criteria. The median follow-up period after diagnosis was 4.5 (range, 0.1–13.0) years. Pagetoid spread before initial treatment was suspected in 12 cases (85.7%). Underlying rectal cancer was identified in 6 cases and no primary tumor was detected in the other 6 cases. Seven patients had recurrent disease, with median time to recurrence of 34.6 (range, 19.2–81.7) months. The time to first relapse was 3 months and that to the second relapse was 6 months. The overall 5-year survival rate was 90.0%.Conclusions: Skin biopsy and immunohistological examination are essential to differentiate PPD with and without underlying anorectal carcinoma. If wide local excision is performed to preserve the anus, it is necessary to search carefully for distant metastasis and arrange for regular follow-up, even if recurrence occurs after a long period. Local resection may achieve long-term survival in patients with locoregional recurrence if distant recurrence does not occur within a short interval..
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Preprint| Erscheinungsjahr: |
2023 |
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| Erschienen: |
2023 |
| Enthalten in: |
ResearchSquare.com - (2023) vom: 16. Okt. Zur Gesamtaufnahme - year:2023 |
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| Sprache: |
Englisch |
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| Beteiligte Personen: |
Imaizumi, Jun [VerfasserIn] |
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| doi: |
10.21203/rs.3.rs-1507489/v1 |
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| PPN (Katalog-ID): |
XRA035681586 |
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| 245 | 1 | 0 | |a A review of 14 cases of perianal Paget’s disease: Characteristics of anorectal cancer with Pagetoid spread |
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| 520 | |a Abstract Background: Perianal Paget's disease (PPD) is intraepithelial invasion of the perianal skin and is frequently associated with underlying anorectal carcinoma. The relatively rare nature of this disease has made it difficult to develop treatment recommendations. This study aimed to clarify the clinicopathological characteristics, treatment options that allow preservation of anorectal function, and long-term results.Methods: The National Cancer Center Hospital database was searched for all cases of perianal Paget's disease diagnosed between 2006 and 2021. In the 14 cases identified, we reviewed the diagnosis, management, and outcomes of adenocarcinoma with Pagetoid spread, including suspected or recurrent cases.Results: All 14 cases met the inclusion criteria. The median follow-up period after diagnosis was 4.5 (range, 0.1–13.0) years. Pagetoid spread before initial treatment was suspected in 12 cases (85.7%). Underlying rectal cancer was identified in 6 cases and no primary tumor was detected in the other 6 cases. Seven patients had recurrent disease, with median time to recurrence of 34.6 (range, 19.2–81.7) months. The time to first relapse was 3 months and that to the second relapse was 6 months. The overall 5-year survival rate was 90.0%.Conclusions: Skin biopsy and immunohistological examination are essential to differentiate PPD with and without underlying anorectal carcinoma. If wide local excision is performed to preserve the anus, it is necessary to search carefully for distant metastasis and arrange for regular follow-up, even if recurrence occurs after a long period. Local resection may achieve long-term survival in patients with locoregional recurrence if distant recurrence does not occur within a short interval. | ||
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