Korean J Obstet Gynecol.  2011 Aug;54(8):474-479. 10.5468/KJOG.2011.54.8.474.

Paget's disease of the vulva treated by wide excision and reconstruction with skin grafts and flaps: Reports of three cases

• Cho DH
• Roh SG
• Lee JH

• KMID: 2274070
• DOI: http://doi.org/10.5468/KJOG.2011.54.8.474

Abstract

Extramammary Paget's disease is a rare, slow-growing, non-invasive intraepithelial adenocarcinoma that occurs mainly in the elderly outside of the mammary gland. Paget's disease of the vulva accounts for less than 1% of all vulvar malignancies. The standard treatment is wide local excision of the gross lesion. To prevent a relapse, wide excision with adequate margins is necessary not to leave pathologic lesions around margins of excision. However, since the excised area is too wide to cover all the exposed surface with a simple closure only, skin grafts and flaps can be very useful. We report successful treatment of three cases of extramammary Paget's disease of the vulva using frozen section analyses of the margins during operation in order to attain Paget's cells free margins and skin grafts and flaps to cover an extensive defects.

Keyword

Paget's disease; Vulva; Skin grafts and flaps

MeSH Terms

Adenocarcinoma
Aged
Frozen Sections
Humans
Mammary Glands, Human
Paget Disease, Extramammary
Recurrence
Skin
Transplants
Vulva

Figure

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  Fig. 1. Preoperative finding: eczematoid lesion on left labium major (A). Postoperative finding: wide local excision and coverage by split-thickness skin graft (B).

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  Fig. 2. Microscopic features of vulvar Paget's disease. Large pale tumor cells dispersed singly or in clusters of solid nests and glandular spaces within the epidermis (H&E, ×100) (A). Tumor cells have abundant pale cytoplasm with large nucleus and prominent nucleolus at high-power view (H&E, ×400) (B).

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  Fig. 3. Preoperative finding: reddish eczematoid lesion on both labium major (A). Postoperative finding: wide local excision and coverage by bilateral perineal perforator island flap (B).

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  Fig. 4. Preoperative finding: eczematoid lesion on both labium major (A). Postoperative finding: wide local excision and coverage by bilateral medial thigh perforator based V-Y advancement flap (B).

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