P47 Management of extramammary paget disease: a current unmet need

Abstract

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