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RECURRENT EXTRAMAMMARY PAGET’S DISEASE OF THE VULVA WITH PERIURETHRAL AND ANAL INVOLVEMENT
Abstract
Introduction
Extramammary Paget's disease (EMPD) of the vulva is a rare neoplasm that usually arise from the apocrine gland bearing areas with high rates of recurrence. We report a case of a 67-year-old female who previously underwent wide excision of primary EMPD five years prior. The lesions recurred four years after, and showed a 14 by 18 cm erythematous lesion with red patches and plaques. "Cake-icing appearance" of the lesion spread from 2 cm above the urethral meatus up to 3 cm below the anal opening, to the right genitocrucal fold and 3 cm from the left genitocrural fold. A wide excision involving distal urethrectomy, partial vulvectomy, anal mucosectomy with split-thickness skin grafting and sigmoid loop colostomy was done.
Description
A 2-centimeter margin was obtained around the lateral extent of the lesions. The incision involved a depth of 1 cm of subcutaneous tissues. The distal urethra was excised en bloc with the skin lesions including a 1-cm margin of anal mucosa. Following mucosectomy, the anal mucosa was then mobilized and pulled towards the external anal sphincters, to which it was anchored using circumferential interrupted Vicryl 3-0 sutures. Frozen section was done to check for adequacy of margins. It noted involvement at the 7 o’clock position of the anal mucosa, hence additional mucosa was excised. Laparoscopic sigmoid loop colostomy was performed followed by Split thickness skin grafting.
Conclusion/Implications
Wide local excision remains the mainstay treatment of EMPD. Positive margins may not be associated with recurrence or overall survival; limiting the resection margins may be considered.