Intraepithelial adenocarcinoma involving the perianal skin.
Is associated with an underlying colorectal cancer in over 50% of cases
This can be anal or colorectal malignancy
Rare
Types
Primary
‘original Paget’s disease’, accounting for the majority of cases
Secondary
‘pagetoid extension’, an epidermotropic metastasis of an underlying neoplasm, most often from rectal carcinoma.
Prognosis
Surgical resection is the current mainstay of treatment; however, recurrence is common even with negative margins with a 31–61% risk in non-invasive cases
Prognosis is often determined by stage of underlying malignancy
Diagnosis
Biopsy
Gastroscopy, colonosopy, ureteroscopy and cystoscopy - association with other cancers
Treatment
Paucity of strong data to standardize the treatment