P47 Management of extramammary paget disease: a current unmet need

Abstract Extramammary Paget disease (EMPD) is a rare epithelial malignancy predominantly affecting the anogenital skin. Primary EMPD carries risk of invasion and metastasis, as well in contiguous organs. No UK guidelines for management exist, resulting lack consensus staging screening associated cancers. We collected consecutive cases histologically diagnosed at regional centre (2017–2022). The aim was to characterize presentation, treatment. identified 22 patients [12 (55%) women, 10 (45%) men; mean age 72 years]. Affected sites were vulva (n = 10; 45%), male genitalia 7; 32%), perianal area/buttocks 3; 14%), suprapubic area 2; 9%) axilla 1; 4%). One patient had vulval anal EMPD. All referred from primary care multiple secondary services, including dermatology 9; 41%), gynaecology urology colorectal surgery 4.5%). Nine (41%) jointly managed with dermatology. Sixteen (73%) discussed multidisciplinary team (MDT) meeting; nine specialist skin cancer MDT seven (32%) other MDTs (anal cancer, colorectal, urology, oncology, gynae-oncology). Screening performed 14 (64%). Eleven (50%) histological evidence their EMPD, which (82%) underwent advanced imaging computed tomography, positron emission tomography or magnetic resonance imaging. Treatment directed by stage (Table 1). Three (14%) developed metastatic died despite radiochemotherapy chemotherapy surgery. Our data confirm low prevalence but mortality appears high. complex involving Patients are best conjunction relevant MDTs. A greater understanding this needed standardize care. Table 1Treatment received our cohort corresponding extramammary (EMPD)TreatmentPatients, n (%)Tumour classificationEMPD stagingTopical agents5 (23)TisIntraepithelial (in situ) EMPDSurgery12 (54)T1Tumour thickness ≤ 4 mm no lymphovascular invasionChemotherapy/radiotherapy ± surgery5 (23)T2Tumour >