Frozen section-guided wide local excision in the treatment of penoscrotal extramammary Paget's disease.

ABSTRACT

OBJECTIVE: To analyse the clinicopathological characteristics of penoscrotal extramammary Paget's disease (EMPD) and to discuss the outcomes after frozen section-guided wide local excision. PATIENTS AND METHODS: From 1990 to 2005, at our institution, 38 patients with penoscrotal EMPD received wide local excision with intraoperative frozen-section analysis. Their medical records were reviewed for patient demographics, lesion characteristics, surgical margin status, and clinical outcome. RESULTS: No patients had EMPD secondary to a non-cutaneous malignancy; 23 patients had intraepithelial EMPD, 12 had invasive EMPD and three had EMPD with underlying adnexal adenocarcinoma. The median (range) largest diameter of the lesion was 6 (1-20) cm. Of 38 patients, 12 (32%) had positive frozen-section results and had extended surgical excision until a negative margin was obtained. Additional margin examination of the gross specimen and final examination of the frozen sections showed that five patients had false-negative results. There was a microscopic positive margin in 15 of 38 (40%) patients when a conventional 2 cm clinical tumour-free border was maintained. Skin erythematous patches were significantly correlated with the spread of disease (P = 0.03). After a median (range) follow-up of 33.5 (3-140) months, six of 38 (16%) patients had recurrent disease, of whom only two had recurrent skin lesions, while four had systemic progression. CONCLUSION: Frozen section-guided wide local excision gave an acceptable recurrence rate in the treatment of penoscrotal EMPD. Primary EMPD with dermal invasion should be actively monitored for possible aggressive behaviour.