Secondary Perianal Extramammary Paget's Disease Originating from a High-Grade Tubular Villous Adenoma Treated with Sphincter-Preserving Wide Local Excision and VY Advancement Flap: A Case Report.
- 1Department of Gastrointestinal Surgery, Sapporo Kojinkai Memorial Hospital, Sapporo, Hokkaido, Japan.
- 2Department of Surgery, Doukikai Tomakomai Hospital, Tomakomai, Hokkaido, Japan.
Abstract
Secondary extramammary Paget's disease (EMPD) of the perianal region most commonly arises from an invasive colorectal adenocarcinoma; pagetoid spread from a non-invasive precursor, such as a high-grade tubular-villous adenoma (TVA), is rare. A 70-year-old man presented with a high-grade TVA of the anal canal and concurrent perianal EMPD. Initial immunohistochemistry of the perianal lesion showed CK7(-), CK20(+), and CAM5.2(+), supporting secondary EMPD of colorectal origin. Adenoma resection achieved negative margins (R0), with no invasive malignancy on imaging or endoscopy. Given staged resection, histological continuity could not be confirmed, and the lesions were initially managed as independent entities. Wide local excision with VY flap reconstruction was subsequently performed. The excised specimen showed diffuse CK20(+) and CDX2(+) with focal CK7 positivity, suggesting a colorectal-type secondary origin. GATA3 was positive in the Paget lesion but negative in the adenoma; this isolated discordant finding is discussed in the main text. The patient has remained recurrence-free for 3 years with preserved anal function. Focal CK7 positivity should not be interpreted as evidence against a secondary origin when CK20/CDX2 are diffusely coexpressed. When the precursor is suggested to be noninvasive, sphincter-preserving wide local excision with VY flap reconstruction appears feasible as an alternative to APR.
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BibTeX
@article{inoue2026secondary,
title = {{Secondary Perianal Extramammary Paget's Disease Originating from a High-Grade Tubular Villous Adenoma Treated with Sphincter-Preserving Wide Local Excision and VY Advancement Flap: A Case Report.}},
author = {Takahiro Inoue and Kyoichi Uemura and Yasuhiro Mizushima},
journal = {Journal of the anus, rectum and colon},
year = {2026},
month = oct,
doi = {10.23922/jarc.2026-055},
url = {https://europepmc.org/article/MED/42952659},
note = {CC BY-NC-ND},
}
RIS
TY - JOUR
TI - Secondary Perianal Extramammary Paget's Disease Originating from a High-Grade Tubular Villous Adenoma Treated with Sphincter-Preserving Wide Local Excision and VY Advancement Flap: A Case Report.
AU - Takahiro Inoue
AU - Kyoichi Uemura
AU - Yasuhiro Mizushima
PY - 2026
DA - 2026/10/25
JO - Journal of the anus, rectum and colon
DO - 10.23922/jarc.2026-055
UR - https://europepmc.org/article/MED/42952659
AB - Secondary extramammary Paget's disease (EMPD) of the perianal region most commonly arises from an invasive colorectal adenocarcinoma; pagetoid spread from a non-invasive precursor, such as a high-grade tubular-villous adenoma (TVA), is rare. A 70-year-old man presented with a high-grade TVA of the anal canal and concurrent perianal EMPD. Initial immunohistochemistry of the perianal lesion showed CK7(-), CK20(+), and CAM5.2(+), supporting secondary EMPD of colorectal origin. Adenoma resection achieved negative margins (R0), with no invasive malignancy on imaging or endoscopy. Given staged resection, histological continuity could not be confirmed, and the lesions were initially managed as independent entities. Wide local excision with VY flap reconstruction was subsequently performed. The excised specimen showed diffuse CK20(+) and CDX2(+) with focal CK7 positivity, suggesting a colorectal-type secondary origin. GATA3 was positive in the Paget lesion but negative in the adenoma; this isolated discordant finding is discussed in the main text. The patient has remained recurrence-free for 3 years with preserved anal function. Focal CK7 positivity should not be interpreted as evidence against a secondary origin when CK20/CDX2 are diffusely coexpressed. When the precursor is suggested to be noninvasive, sphincter-preserving wide local excision with VY flap reconstruction appears feasible as an alternative to APR.
SN - 2432-3853
ER -