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 an 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 aims to analyze t...
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BMC
2023-01-01
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Series: | World Journal of Surgical Oncology |
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Online Access: | https://doi.org/10.1186/s12957-022-02872-z |
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author | Jun Imaizumi Konosuke Moritani Yasuyuki Takamizawa Manabu Inoue Shunsuke Tsukamoto Yukihide Kanemitsu |
author_facet | Jun Imaizumi Konosuke Moritani Yasuyuki Takamizawa Manabu Inoue Shunsuke Tsukamoto Yukihide Kanemitsu |
author_sort | Jun Imaizumi |
collection | DOAJ |
description | Abstract Background Perianal Paget’s disease (PPD) is an 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 aims to analyze the clinical and pathological features of perianal Paget’s disease (PPD) and to explore rational treatment options and follow-up for this disease. 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 patients 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 the median time to recurrence of 34.6 (range, 19.2–81.7) months. The time to the first relapse was 3 months, and that to the second relapse was 6 months. The overall 5-year survival rate was 90.0%. Conclusions Endoscopic and radiologic evaluation, as well as immunohistologic examination, should be performed. is to differentiate PPD with and without underlying anorectal carcinoma. The time to first recurrence varies widely, and long-term and regular follow-up for more than 5 years is considered necessary for local recurrence and distant metastasis. |
first_indexed | 2024-04-10T21:02:25Z |
format | Article |
id | doaj.art-b5474f96180f4f1eb4425947d1f1472a |
institution | Directory Open Access Journal |
issn | 1477-7819 |
language | English |
last_indexed | 2024-04-10T21:02:25Z |
publishDate | 2023-01-01 |
publisher | BMC |
record_format | Article |
series | World Journal of Surgical Oncology |
spelling | doaj.art-b5474f96180f4f1eb4425947d1f1472a2023-01-22T12:15:44ZengBMCWorld Journal of Surgical Oncology1477-78192023-01-012111710.1186/s12957-022-02872-zA review of 14 cases of perianal Paget’s disease: characteristics of anorectal cancer with pagetoid spreadJun Imaizumi0Konosuke Moritani1Yasuyuki Takamizawa2Manabu Inoue3Shunsuke Tsukamoto4Yukihide Kanemitsu5Department of Colorectal Surgery, National Cancer Center HospitalDepartment of Colorectal Surgery, National Cancer Center HospitalDepartment of Colorectal Surgery, National Cancer Center HospitalDepartment of Colorectal Surgery, National Cancer Center HospitalDepartment of Colorectal Surgery, National Cancer Center HospitalDepartment of Colorectal Surgery, National Cancer Center HospitalAbstract Background Perianal Paget’s disease (PPD) is an 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 aims to analyze the clinical and pathological features of perianal Paget’s disease (PPD) and to explore rational treatment options and follow-up for this disease. 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 patients 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 the median time to recurrence of 34.6 (range, 19.2–81.7) months. The time to the first relapse was 3 months, and that to the second relapse was 6 months. The overall 5-year survival rate was 90.0%. Conclusions Endoscopic and radiologic evaluation, as well as immunohistologic examination, should be performed. is to differentiate PPD with and without underlying anorectal carcinoma. The time to first recurrence varies widely, and long-term and regular follow-up for more than 5 years is considered necessary for local recurrence and distant metastasis.https://doi.org/10.1186/s12957-022-02872-zPagetoid spreadExtramammary paget’s diseaseAnorectal cancerCK20GCDFP-15CDX-2 |
spellingShingle | Jun Imaizumi Konosuke Moritani Yasuyuki Takamizawa Manabu Inoue Shunsuke Tsukamoto Yukihide Kanemitsu A review of 14 cases of perianal Paget’s disease: characteristics of anorectal cancer with pagetoid spread World Journal of Surgical Oncology Pagetoid spread Extramammary paget’s disease Anorectal cancer CK20 GCDFP-15 CDX-2 |
title | A review of 14 cases of perianal Paget’s disease: characteristics of anorectal cancer with pagetoid spread |
title_full | A review of 14 cases of perianal Paget’s disease: characteristics of anorectal cancer with pagetoid spread |
title_fullStr | A review of 14 cases of perianal Paget’s disease: characteristics of anorectal cancer with pagetoid spread |
title_full_unstemmed | A review of 14 cases of perianal Paget’s disease: characteristics of anorectal cancer with pagetoid spread |
title_short | A review of 14 cases of perianal Paget’s disease: characteristics of anorectal cancer with pagetoid spread |
title_sort | review of 14 cases of perianal paget s disease characteristics of anorectal cancer with pagetoid spread |
topic | Pagetoid spread Extramammary paget’s disease Anorectal cancer CK20 GCDFP-15 CDX-2 |
url | https://doi.org/10.1186/s12957-022-02872-z |
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