Table 2: Patient demographics, pathology, PPD recurrence, synchronous or metachronous malignancies, survival, and immunophenotypes of secondary perianal Paget’s diseasea.

CaseAge (years)SexSurgical treatmentContinuous lesion or synchronous malignancyPPD recurrenceSurvival and follow-upCause of deathCK7 (6/13)CK20 (10/13)CDX2 (12/13)GCDFP-15 (0/13)

1172FAPRRectal mucinous adenocarcinoma (synchronous, not continuous)NoneDeath, 5 monthsRespiratory failure--+-
1271FWLE+LARRectal mucinous adenocarcinoma (synchronous, not continuous)Yes, 10 years laterAlive, 11 yearsNone-++-
1373MAPRAnorectal adenocarcinoma (synchronous, not continuous)NoneAlive, 4 yearsNone+++-
1477FWLEAnorectal carcinoma (continuous); HCC or cholangiocarcinoma (synchronous)NoneDeath, 5 monthsLiver tumor (suspected HCC or cholangiocarcinoma) with obstructive jaundice and septic shock+++-
1577MAPRAnorectal mucinous carcinoma (continuous)NoneDeath, 2 yearsAnorectal mucinous carcinoma with bone metastasis+++-
1677MAPRAnorectal adenocarcinoma (continuous)NoneDeath, 4 yearsAnorectal carcinoma with bone and lung metastasesF++-
1773MAPRAnorectal adenocarcinoma (continuous)NoneAlive, 5 yearsNone--+-
1871MN/AAnorectal adenocarcinoma (continuous)UnknownUnknown, lost follow-upNone-++-
1950FAPRAnorectal mucinous carcinoma (continuous)NoneDeath, 2 yearsAnorectal mucinous carcinoma with abdominal cavity, lung, and cerebellar metastases+++-
2088MN/AAnorectal adenocarcinoma (continuous)NoneDeath, 1 yearCongestive heart failure-++-
2181MAPRAnorectal adenocarcinoma (continuous)NoneAlive, 3 yearsNone-++-
2279MAPRAnorectal adenocarcinoma (continuous)UnknownUnknown, lost follow-upNone+---
2364FWLERectal adenocarcinoma (continuous)NoneAlive, 1 yearNone-++-

aF: focally positive. PPD: perianal Paget’s disease; APR: abdominal peritoneal resection; WLE: wide local excision; LAR: low anterior resection; HCC: hepatocellular carcinoma; N/A: not applicable.