Table 1: Patient demographics, pathology, PPD recurrence, synchronous or metachronous malignancies, survival, and immunophenotypes of primary perianal Paget’s diseasea.

CaseAge (years)SexSurgical treatmentMetachronous malignancyPPD recurrenceSurvival and follow-upCause of deathCK7 (7/10)CK20 (6/10)CDX2 (6/10)GCDFP-15 (3/10)

169MAPRCholangiocarcinoma (metachronous, 11 years)NoneDeath, 11 yearsCholangiocarcinoma-++-
283FWLENoneNoneAlive, 11 yearsNone-++-
376MWLENoneNoneDeath, 2 monthsSepsis+--+
475MWLENoneYes, 1 year laterDeath, 2 yearsUnknown (died after discharge)-++-
587FWLENoneNoneDeath, 4 yearsRespiratory failure+F+-
677MWLENoneNoneDeath, 9 yearsRespiratory failure+-F-
785MWLENoneNoneDeath, 7 yearsUnknown (died after discharge)F++-
862FWLEUrothelial carcinoma (metachronous, 2 years)NoneDeath, 3 yearsUrothelial carcinoma with multiple metastases+---
960FWLE then APRNoneYes, 4 years laterDeath, 6 yearsDOD, PPD with lymph nodes and lung metastases++-+
1069MWLERectal small-cell carcinoma (metachronous, 3 years)NoneDeath, 3 yearsRectal small-cell carcinoma with lymph node metastases+--+

aF: focally positive. PPD: perianal Paget’s disease; APR: abdominal peritoneal resection; WLE: wide local excision; DOD: died of disease.