Treatment challenges associated with primary extramammary Paget's disease

Extramammary Paget's disease (EMPD) is a rarely described skin neoplasm primarily affecting the anogenital region. The diagnosis of EMPD is often delayed due to misdiagnosis which results in delayed treatment. Surgical excision is the mainstay of treatment in patients diagnosed with EMPD. However, cancerous remains are relatively often found due to difficulties with defining the tumor margins during surgery. This results in multiple re-excisions and potential dissemination. We here present two cases of primary EMPD in males and challenges associated with EMPD treatment. Mapping biopsies can provide valuable knowledge and prohibit multiple unsuccessful re-excisions, in patients where the tumor border is difficult to define. A mapping strategy can also contribute to a reduced skin excision, which is crucial in the genital region. Furthermore, we find that PET-CT is an important tool in the disease staging and, hence, selection of adequate treatment strategies. Level of evidence: Level V, therapeutic study.