TY - JOUR
T1 - Perianal Paget's disease
T2 - A clinicopathological and immunohistochemical study of 13 cases
AU - Liao, Xiaoyan
AU - Liu, Xiuli
AU - Fan, Xuemo
AU - Lai, Jinping
AU - Zhang, Dongwei
N1 - Publisher Copyright:
© 2020 The Author(s).
PY - 2020/3/24
Y1 - 2020/3/24
N2 - Background: Perianal Paget's disease (PPD) is rare and mostly described in clinical literature as case reports or small series. Methods: We investigated the clinicopathologic and immunohistochemical features of PPD in a total of 13 cases retrieved from multiple academic institutions. Results: The median age at diagnosis was 75 (range 50-86) years. Males were predominant with a male to female ratio of 2.25:1. Four (30.8%) cases were classified as primary PPD due to lack of synchronous or metachronous underlying malignancies, while nine (69.2%) were classified as secondary PPD with concurrent invasive adenocarcinoma (n = 8) or tubular adenoma with high-grade dysplasia (n = 1). Immunohistochemically, there is no differential expression of CK7 or CK20 in Paget's cells between primary and secondary PPD; however, GCDFP-15 was only positive in primary PPD (3/3 vs. 0/6, P = 0.012), while CDX2 was only positive in secondary PPD (0/3 vs. 7/7, P = 0.008), suggesting different cell origin. All patients received local surgical resection with or without adjuvant therapy. After a median follow-up of 47 months, one patient with secondary PPD (7.7%) died of disease progression from underlying adenocarcinoma. Conclusions: PPD occurs in elderly patients with male predominance and is frequently associated with underlying malignancies. Differential expression of CDX2 and GCDFP-15 may help distinguishing primary vs. secondary PPD, which is important for management as the presence of an underlying malignancy impacts clinical course and prognosis. Surgical excision remains the major treatment strategy for PPD. Long-term follow-up is required to monitor the disease recurrence and metastasis.
AB - Background: Perianal Paget's disease (PPD) is rare and mostly described in clinical literature as case reports or small series. Methods: We investigated the clinicopathologic and immunohistochemical features of PPD in a total of 13 cases retrieved from multiple academic institutions. Results: The median age at diagnosis was 75 (range 50-86) years. Males were predominant with a male to female ratio of 2.25:1. Four (30.8%) cases were classified as primary PPD due to lack of synchronous or metachronous underlying malignancies, while nine (69.2%) were classified as secondary PPD with concurrent invasive adenocarcinoma (n = 8) or tubular adenoma with high-grade dysplasia (n = 1). Immunohistochemically, there is no differential expression of CK7 or CK20 in Paget's cells between primary and secondary PPD; however, GCDFP-15 was only positive in primary PPD (3/3 vs. 0/6, P = 0.012), while CDX2 was only positive in secondary PPD (0/3 vs. 7/7, P = 0.008), suggesting different cell origin. All patients received local surgical resection with or without adjuvant therapy. After a median follow-up of 47 months, one patient with secondary PPD (7.7%) died of disease progression from underlying adenocarcinoma. Conclusions: PPD occurs in elderly patients with male predominance and is frequently associated with underlying malignancies. Differential expression of CDX2 and GCDFP-15 may help distinguishing primary vs. secondary PPD, which is important for management as the presence of an underlying malignancy impacts clinical course and prognosis. Surgical excision remains the major treatment strategy for PPD. Long-term follow-up is required to monitor the disease recurrence and metastasis.
KW - Clinicopathologic features
KW - Histology
KW - Immunohistochemistry
KW - Paget's disease
KW - Perianal
KW - Prognosis
UR - http://www.scopus.com/inward/record.url?scp=85082380335&partnerID=8YFLogxK
U2 - 10.1186/s13000-020-00952-w
DO - 10.1186/s13000-020-00952-w
M3 - Article
C2 - 32209119
AN - SCOPUS:85082380335
SN - 1746-1596
VL - 15
JO - Diagnostic Pathology
JF - Diagnostic Pathology
IS - 1
M1 - 29
ER -