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. 2021 Sep;12(3):524-529.
doi: 10.1007/s13193-021-01366-w. Epub 2021 Jun 5.

Impact of Surgery on Extramammary Paget's Disease Vulva: a Case Series

J S Anjana  1 , P Rema  1 , S Suchetha  1 , J Siva Ranjith  2 , Amrita B Rao  3 , T R Preethi  4
Affiliations

Impact of Surgery on Extramammary Paget's Disease Vulva: a Case Series

J S Anjana et al. Indian J Surg Oncol. 2021 Sep.

Abstract

Extramammary Paget's disease is a rare neoplasm that may affect the vulva. It usually presents as a pruritic red eczematous lesion with islands of hyperkeratosis that is often mistaken for other benign conditions. There are no specific guidelines for the management of this disease. Surgical excision is the standard treatment but recurrences are common in spite of extensive surgery with negative margins. We report here a series of 7 cases of Paget's disease vulva treated by primary surgery. The series highlights some of the challenges in the diagnosis and management of Paget's disease vulva.

Keywords: Hyperkeratosis; Paget’s disease vulva; Surgery.

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Figures

Fig. 1
Fig. 1
Vulval lesion of case 1
Fig. 2
Fig. 2
Surgical defect after wide excision in case 1
Fig. 3
Fig. 3
Gracilis myocutaneous flap reconstruction in case 1
Fig. 4
Fig. 4
H & E staining: A tumor cells infiltrating epidermis; B Paget’s cells
Fig. 5
Fig. 5
Immunohistochemistry. A CK7 strong positive. B CK20 focal positive. C Tumor cells showing p40 negative
Fig. 6
Fig. 6
A Recurrent lesion of case 5. B Response after 8 weeks of imiquimod
See this image and copyright information in PMC

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