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 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.

A case of anal canal carcinoma with pagetoid spread that was curatively resected by multiple endoscopic and surgical treatments

“…However, the anorectal border could not be recognized even with NBI magnification, requiring multiple surgical treatments. A mapping biopsy is usually performed when the anorectal lesion is macroscopically visible, 2 as in the second local excision in this case. If the tumor boundary cannot be recognized grossly or endoscopically, as in this case, the extent of resection should be determined using intraoperative consultation, for example.…”

“…Extra-mammary Paget's Disease (EMPD) has been reported in several extramammary sites, such as the axilla, thighs, groin, perineum, scrotum, vulva, and perianal area. 1,2 If it presents in the perianal area, it is referred to as perianal Paget's Disease (PPD). 3 PPD often shows pagetoid spread (PS), a pattern of tumor spread into neighboring organs around the epidermis due to intraepithelial progression.…”

“…Extra‐mammary Paget's Disease (EMPD) has been reported in several extramammary sites, such as the axilla, thighs, groin, perineum, scrotum, vulva, and perianal area 1,2 . If it presents in the perianal area, it is referred to as perianal Paget's Disease (PPD) 3 .…”

How to Surveil Perianal Paget's Disease: A Case Report

“…It is characterized by the presence of malignant glandular epithelial cells (Paget’s cells) within the squamous epithelium. EMPD is found in apocrine glandular epithelial tissues including the vulva, penis, perineum, perianal, and axilla [ 4 ].…”

“…The association between PPD (both primary and secondary) and underlying malignancy is well-established but poorly understood. The rate of associated malignancy with PPD ranges from 33% to 86% [ 4 , 6 ]. The finding of PPD should prompt a physician to undertake a diligent search for an underlying malignancy.…”