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

Overview

Journal Indian J Surg Oncol

Specialty Oncology

Date 2021 Oct 18

PMID 34658580

Authors

J S Anjana

P Rema

S Suchetha

J Siva Ranjith

Amrita B Rao

T R Preethi

Affiliations

Soon will be listed here.

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.

References

Delport E . Extramammary Paget's disease of the vulva: An annotated review of the current literature. Australas J Dermatol. 2012; 54(1):9-21. DOI: 10.1111/j.1440-0960.2012.00898.x. View

Luyten A, Sorgel P, Clad A, Gieseking F, Maass-Poppenhusen K, Lelle R . Treatment of extramammary Paget disease of the vulva with imiquimod: a retrospective, multicenter study by the German Colposcopy Network. J Am Acad Dermatol. 2014; 70(4):644-650. DOI: 10.1016/j.jaad.2013.12.008. View

van der Linden M, Meeuwis K, Bulten J, Bosse T, van Poelgeest M, de Hullu J . Paget disease of the vulva. Crit Rev Oncol Hematol. 2016; 101:60-74. DOI: 10.1016/j.critrevonc.2016.03.008. View

de Magnis A, Checcucci V, Catalano C, Corazzesi A, Pieralli A, Taddei G . Vulvar paget disease: a large single-centre experience on clinical presentation, surgical treatment, and long-term outcomes. J Low Genit Tract Dis. 2013; 17(2):104-10. DOI: 10.1097/LGT.0b013e31826569a9. View

Karam A, Dorigo O . Treatment outcomes in a large cohort of patients with invasive Extramammary Paget's disease. Gynecol Oncol. 2012; 125(2):346-51. DOI: 10.1016/j.ygyno.2012.01.032. View

Tagliaferri L, Casa C, Macchia G, Pesce A, Garganese G, Gui B . The Role of Radiotherapy in Extramammary Paget Disease: A Systematic Review. Int J Gynecol Cancer. 2018; 28(4):829-839. DOI: 10.1097/IGC.0000000000001237. View

Murata Y, Kumano K . Extramammary Paget's disease of the genitalia with clinically clear margins can be adequately resected with 1 cm margin. Eur J Dermatol. 2005; 15(3):168-70. View

Shaco-Levy R, Bean S, Vollmer R, Jewell E, Jones E, Leilani Valdes C . Paget disease of the vulva: a study of 56 cases. Eur J Obstet Gynecol Reprod Biol. 2009; 149(1):86-91. DOI: 10.1016/j.ejogrb.2009.11.003. View

Dogan A, Hilal Z, Krentel H, Cetin C, Hefler L, Grimm C . Paget's Disease of the Vulva Treated with Imiquimod: Case Report and Systematic Review of the Literature. Gynecol Obstet Invest. 2016; 82(1):1-7. DOI: 10.1159/000449158. View

10.

Onaiwu C, Salcedo M, Pessini S, Munsell M, Euscher E, Reed K . Paget's disease of the vulva: A review of 89 cases. Gynecol Oncol Rep. 2017; 19:46-49. PMC: 5220256. DOI: 10.1016/j.gore.2016.12.010. View

11.

Hendi A, Brodland D, Zitelli J . Extramammary Paget's disease: surgical treatment with Mohs micrographic surgery. J Am Acad Dermatol. 2004; 51(5):767-73. DOI: 10.1016/j.jaad.2004.07.004. View

12.

Lloyd J, Flanagan A . Mammary and extramammary Paget's disease. J Clin Pathol. 2000; 53(10):742-9. PMC: 1731095. DOI: 10.1136/jcp.53.10.742. View