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...

Full description

Saved in:
Bibliographic Details
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
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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.
ISSN:1687-6121
1687-630X