Perianal Paget’s Disease: The 17-Year-Experience of a Single Institution in Taiwan

Aim. To determine the incidence, prognosis, and immunophenotypes (CK7, CK20, CDX2, and GCDFP-15) of primary or secondary perianal Paget’s diseases (PPDs). Methods. Twenty-three PPD patients were recruited, including 10 primary and 13 secondary PPDs. Immunophenotypes of PPD were analyzed. Results. In...

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Main Authors: Yu-Chen Wang, Anna Fen-Yau Li, Shung-Haur Yang, Hsiu-Hsun Ma, Wen-Yih Liang
Format: Article
Language:English
Published: Wiley 2019-01-01
Series:Gastroenterology Research and Practice
Online Access:http://dx.doi.org/10.1155/2019/2603279
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author Yu-Chen Wang
Anna Fen-Yau Li
Shung-Haur Yang
Hsiu-Hsun Ma
Wen-Yih Liang
author_facet Yu-Chen Wang
Anna Fen-Yau Li
Shung-Haur Yang
Hsiu-Hsun Ma
Wen-Yih Liang
author_sort Yu-Chen Wang
collection DOAJ
description Aim. To determine the incidence, prognosis, and immunophenotypes (CK7, CK20, CDX2, and GCDFP-15) of primary or secondary perianal Paget’s diseases (PPDs). Methods. Twenty-three PPD patients were recruited, including 10 primary and 13 secondary PPDs. Immunophenotypes of PPD were analyzed. Results. In 23 PPD patients, 14 (60.9%) were male and the median age was 75 years. Three (13.0%, 2 primary and 1 secondary PPDs) had recurrence and two (8.7%, both primary PPDs) had invasive PPDs. The colorectal cancers (CRCs) in secondary PPD cases were located in anorectal area for 9 patients while 4 were located in the rectum; 5, 2, 4, and 2 were in stages I, II, III, and in uncertain stage, respectively. The distant metastasis rates of CRC in the secondary PPD patients during follow-up were 40% (2/5), 0% (0/2), and 50% (2/4) for stages I, II, and III, respectively. Other synchronous or metachronous malignancies included cholangiocarcinoma, urothelial carcinoma, anorectal small-cell carcinoma, and unknown hepatic malignancy. One primary PPD patient died from the metastases of invasive Paget’s disease while 3 secondary PPD patients died from the metastases of CRCs during follow-up. Immunohistochemical staining showed CK7 (7/10 and 6/13), CK20 (6/10 and 10/13), CDX2 (6/10 and 12/13), and GCDFP-15 (3/10 and 0/13) positivities in primary and secondary PPD patients, respectively. The immunophenotypes were not statistical significantly related to synchronous CRC (P=0.402, 0.650, 0.127, and 0.068 for CK7, CK20, CDX2, and GCDFP-15, respectively). Conclusions. The incidence of concurrent CRC in PPD patients is not low. An adequate survey for CRC should be considered for PPD patients at initial diagnosis. In this series of study, stage I CRC with PPD would have a higher metastatic rate, thus indicating aggressive treatment and follow-up. The CK7, CK20, CDX2, and GCDFP-15 immunostaining results for the PPD patients were not predictive of primary or secondary type.
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spelling doaj-art-f0e08ecb232d400badaf78a0394717092025-02-03T00:59:40ZengWileyGastroenterology Research and Practice1687-61211687-630X2019-01-01201910.1155/2019/26032792603279Perianal Paget’s Disease: The 17-Year-Experience of a Single Institution in TaiwanYu-Chen Wang0Anna Fen-Yau Li1Shung-Haur Yang2Hsiu-Hsun Ma3Wen-Yih Liang4Department of Pathology, Show Chwan Memorial Hospital, Changhua 500, TaiwanDepartment of Pathology and Laboratory Medicine, Taipei Veterans General Hospital, Taipei 112, TaiwanSchool of Medicine, National Yang-Ming University, Taipei 112, TaiwanDepartment of Pathology and Laboratory Medicine, Taipei Veterans General Hospital, Taipei 112, TaiwanDepartment of Pathology and Laboratory Medicine, Taipei Veterans General Hospital, Taipei 112, TaiwanAim. To determine the incidence, prognosis, and immunophenotypes (CK7, CK20, CDX2, and GCDFP-15) of primary or secondary perianal Paget’s diseases (PPDs). Methods. Twenty-three PPD patients were recruited, including 10 primary and 13 secondary PPDs. Immunophenotypes of PPD were analyzed. Results. In 23 PPD patients, 14 (60.9%) were male and the median age was 75 years. Three (13.0%, 2 primary and 1 secondary PPDs) had recurrence and two (8.7%, both primary PPDs) had invasive PPDs. The colorectal cancers (CRCs) in secondary PPD cases were located in anorectal area for 9 patients while 4 were located in the rectum; 5, 2, 4, and 2 were in stages I, II, III, and in uncertain stage, respectively. The distant metastasis rates of CRC in the secondary PPD patients during follow-up were 40% (2/5), 0% (0/2), and 50% (2/4) for stages I, II, and III, respectively. Other synchronous or metachronous malignancies included cholangiocarcinoma, urothelial carcinoma, anorectal small-cell carcinoma, and unknown hepatic malignancy. One primary PPD patient died from the metastases of invasive Paget’s disease while 3 secondary PPD patients died from the metastases of CRCs during follow-up. Immunohistochemical staining showed CK7 (7/10 and 6/13), CK20 (6/10 and 10/13), CDX2 (6/10 and 12/13), and GCDFP-15 (3/10 and 0/13) positivities in primary and secondary PPD patients, respectively. The immunophenotypes were not statistical significantly related to synchronous CRC (P=0.402, 0.650, 0.127, and 0.068 for CK7, CK20, CDX2, and GCDFP-15, respectively). Conclusions. The incidence of concurrent CRC in PPD patients is not low. An adequate survey for CRC should be considered for PPD patients at initial diagnosis. In this series of study, stage I CRC with PPD would have a higher metastatic rate, thus indicating aggressive treatment and follow-up. The CK7, CK20, CDX2, and GCDFP-15 immunostaining results for the PPD patients were not predictive of primary or secondary type.http://dx.doi.org/10.1155/2019/2603279
spellingShingle Yu-Chen Wang
Anna Fen-Yau Li
Shung-Haur Yang
Hsiu-Hsun Ma
Wen-Yih Liang
Perianal Paget’s Disease: The 17-Year-Experience of a Single Institution in Taiwan
Gastroenterology Research and Practice
title Perianal Paget’s Disease: The 17-Year-Experience of a Single Institution in Taiwan
title_full Perianal Paget’s Disease: The 17-Year-Experience of a Single Institution in Taiwan
title_fullStr Perianal Paget’s Disease: The 17-Year-Experience of a Single Institution in Taiwan
title_full_unstemmed Perianal Paget’s Disease: The 17-Year-Experience of a Single Institution in Taiwan
title_short Perianal Paget’s Disease: The 17-Year-Experience of a Single Institution in Taiwan
title_sort perianal paget s disease the 17 year experience of a single institution in taiwan
url http://dx.doi.org/10.1155/2019/2603279
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