Paget’s Disease of Vulva: A case report

Abstract

Extramammary Paget’s disease (EMPD) is a rare clinical entity which can be associated with other malignancies. The vulva is the mostly seen presentation in women. Lesions frequently presents in adnexal structures and clinically demonstrate as erythematous, well-demarcated plaques. Early detection is mandatory because the diagnosis is frequently delayed and there is a high incidence of associated invasive disease. With this case, we discuss the management of vulvar Paget's disease which is rarely seen and importance of investigating the accompanying secondary malignancies.

Keywords

• Paget’s disease of the vulva
• Extrammammary
• Paget’s disease

Kaynakça

1. 1. Onaiwu CO, Salcedo MP, Pessini SA, et al. Paget’s disease of the vulva: A review of 89 cases. Gynecologic Oncology Reports. 2017;19:46-49.
2. 2.Kanitakis J. Mammary and extramammary Paget's disease. J Eur Acad Dermatol Venereol. 2007;21(5):581-90.
3. 3.Van der Putte SCJ. Mammary-like glands of the vulva and their disorders. Int J Gynecol Pathol. 1994;13(2):150-60.
4. 4.Belousova IE, Kazakov DV, Michal M, Suster S. Vulvar toker cells: the long-awaited missing link: a proposal for an origin-based histogenetic classification of extramammary paget disease. Am J Dermatopathol. 2006;28(1):84-6.
5. 5.Niikura H, Yoshida H, Ito K, Takano T, Watanabe H, Aiba S, Yaegashi N. Paget’s disease of the vulva: clinicopathologic study of type 1 cases treated at a single institution. Int J Gynecol Cancer. 2006;16(3):1212-5.
6. 6. Fanning J, Lambert HC, Hale TM, Morris PC, Schuerch C. Paget's disease of the vulva: prevalence of associated vulvar adenocarcinoma, invasive Paget's disease, and recurrence after surgical excision. Am J Obstet Gynecol. 1999;180(1 Pt1):24-7.
7. 7.Wilkinson EJ, Brown HM. Vulvar Paget disease of urothelial origin: a report of three cases and a proposed classification of vulvar Paget disease. Hum Pathol. 2002;33(5):549-54.
8. 8. Shaco-Levy R, Bean SM, Vollmer RT, Jewell E, Jones EL, Valdes CL, et al. Paget disease of the vulva: a study of 56 patients. Eur J Obstet Gynecol Reprod Biol 2010;149(1):86–91.

9. 9. Van der Linden M, Meeuwis KA, Bulten J, Bosse T, van Poelgeest MI, de Hullu JA. Paget disease of the vulva. Crit Rev Oncol Hematol. 2016;101:60-74.
10. 10. Cooper SM, Wojnarowska F. Anogenital (Non-venereal) Disease. 4th ed. Dermatology vol.1. Bolognia JL, Schaffer JV, Cerroni L. China, Elsevier, 2018:1252.
11. 11.Lloyd J, Flanagan A. Mammary and extramammary Paget’s disease. Journal of Clinical Pathology. 2000;53(10):742-749.
12. 12. İşçimen A. Epidermal Keratinositik Prekanseröz veya Prekürsör Lezyonlar. 3. Baskı Dermatoloji Cilt 1. Yalçın Tüzün, Mehmet Ali Gürer, Server Serdaroğlu, Oya Oğuz, Varol L. Aksungur, İstanbul, Nobel 2008:1959-62.
13. 13.Chanda JJ. Extramammary Paget's disease: prognosis and relationship to internal malignancy. J Am Acad Dermatol. 1985;13(6):1009-14.
14. 14. Nakano S, Narita R, Tabaru A et al. Bile duct cancer associated with extramammary Paget’s disease. Am J Gastroenterol 1995; 90:507–8.
15. 15.Siesling S, Elferink MA, van Dijck JA et al. Epidemiology and treatment of extramammary Paget disease in the Netherlands. Eur J Surg Oncol 2007; 33:951–5.
16. 16. Van der Zwan JM, Siesling S, Blokx WA, Pierie JP, Capocaccia R. Invasive extramammary Paget's disease and the risk for secondary tumours in Europe. Eur J Surg Oncol. 2012;38(3):214-21.
17. 17. Nitecki R, Davis M, Watkins JC, Wu YE, Vitonis AF, Muto MG, Berkowitz RS, Horowitz NS, Feltmate CM. Extramammary Paget Disease of the Vulva: A Case Series Examining Treatment, Recurrence, and Malignant Transformation. Int J Gynecol Cancer. 2018;28(3) 632-638.
18. 18. Edey KA, Allan E, Murdoch JB, Cooper S, Bryant A. Interventions for the treatment of Paget's disease of the vulva. Cochrane Database Syst Rev. 2013;26:(10).