Vulvar Paget Disease

Authors

  • Pakhanan Dankamak Department of Obstetrics and Gynecology, Faculty of Medicine, Prince of Songkla University, Hat Yai, Songkhla 90110, Thailand.
  • Kulisara Nanthamongkolkul Department of Obstetrics and Gynecology, Faculty of Medicine, Prince of Songkla University, Hat Yai, Songkhla 90110, Thailand.
  • Ingporn Jiamset Department of Obstetrics and Gynecology, Faculty of Medicine, Prince of Songkla University, Hat Yai, Songkhla 90110, Thailand.
  • Thiti Atjimakul Department of Obstetrics and Gynecology, Faculty of Medicine, Prince of Songkla University, Hat Yai, Songkhla 90110, Thailand.
  • Athithan Rattanaburi Department of Obstetrics and Gynecology, Faculty of Medicine, Prince of Songkla University, Hat Yai, Songkhla 90110, Thailand.

DOI:

https://doi.org/10.31584/psumj.2024264403

Keywords:

Extramammary Paget disease, Paget disease, vulva, vulvar lesion, vulvar neoplasm, vulvar Paget disease

Abstract

Vulvar Paget disease (VPD) is a rare and difficult-to-diagnose condition that can be associated with other types of cancer, such as urinary tract cancer and gastrointestinal tract cancer. Accurate and thorough diagnosis is essential. The main treatment for VPD is surgery, although alternative treatments are available in cases for which surgery is not feasible. These alternative treatments include radiation therapy, topical medications, and chemotherapy for advanced and disseminated cases. Due to the recurrent nature of VPD, continuous monitoring and follow-up are necessary. This article aimed to gather and explore information about VPD. The article includes the etiology of the disease, clinical characteristics, pathological features, various investigations, current treatment options for VPD.

References

Paget J. On disease of the mammary areola preceding cancer of the mammary gland. St Bartholomew Hosp Res London 1874;10:87-9.

Dubreuilh W. Paget’s disease of the vulva. Br J Dermatol 1901;13:407-13.

Fardal RW, Kierland RR, Clagett OT, Woolner LB. Prognosis in cutaneous Paget’s disease. Postgrade Med 1964;36:584-93.

Van der Linden M, Meeuwis KA, Bulten J, Bosse T, van Poelgeest MIE, de Hullu JA. Paget disease of the vulva. Crit Rev Oncol Hematol 2016;101:60-74.

Kanitakis J. Mammary and extramammary Paget’s disease. J Eur Acad Dermatol Venereol 2007;21:581-90.

Willman JH, Golitz LE, Fitzpatrick JE. Vulvar clear cells of toker: precursors of extramammary Paget’s disease. Am J Dermatopathol 2005;27:185-8.

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;38:214-21.

Heng PS, Lu CL, Cheng CL, Lai FJ. Significant male predisposition in extramammary Paget disease: a nationwide population-based study in Taiwan. Br J Dermatol 2014;171:191-3.

De Magnis A, Checcucci V, Catalano C, Corazzesi A, Pieralli A, Taddei G, et al. Vulvar Paget disease: a large single-centre experience on clinical presentation, surgical treatment, and long-term outcomes. J Low Genit Tract Dis 2013;17:104-10.

Ishizuki S, Nakamura Y. Extramammary Paget’s disease: diagnosis, pathogenesis, and treatment with focus on recent developments. Curr Oncol 2021;28:2969-86.

Delport ES. Extramammary Paget’s disease of the vulva: an annotated review of the current literature. Australas J Dermatol 2013;54:9-21.

Berek JS, Hacker NF. Berek and Hacker’s Gynecologic Oncology. 7th ed. Philadelphia, PA: Lippincott Williams & Willkins; 2021;p505-6.

American College of Obstetricians and Gynecologists. Diagnosis and management of vulvar skin disorders: ACOG practice bulletin summary, number 224. Obstet Gynecol 2020;136:222-5.

Bornstein J. Benign disorders of the vulva & vagina. In: Alan H. DeCherney ea, editor. Current diagnosis & treatment. Obstetrics & gynecology. 11th ed. New York: McGraw-Hill; 2013;620-45.

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;33:549-54.

Kibbi N, Owen JL, Worley B, Wang JX, Harikumar V, Downing MB, et al. Evidence-based clinical practice guidelines for extramammary Paget disease. JAMA Oncol 2022;8:618-28.

Chiba H, Kazama T, Takenouchi T, Nomoto S, Yamada S, Tago O, et al. Two cases of vulval pigmented extramammary Paget’s disease: histochemical and immunohistochemical studies. Br J Dermatol 2000;142:1190-4.

Ito Y, Igawa S, Ohishi Y, Uehara J, Yamamoto AI, Iizuka H. Prognostic indicators in 35 patients with extramammary Paget’s disease. Dermatol Surg 2012;38:1938-44.

Zhao Y, Gong X, Li N, Zhu Q, Yu D, Jin X. Primary extramammary Paget’s disease: a clinicopathological study of 28 cases. Int J Clin Exp Pathol 2019;12:3426-32.

Kang Z, Zhang Q, Zhang Q, Li X, Hu T, Xu X, et al. Clinical and pathological characteristics of extramammary Paget’s disease: report of 246 Chinese male patients. Int J Clin Exp Pathol 2015;8:13233-40.

Ohnishi T, Watanabe S. The use of cytokeratins 7 and 20 in the diagnosis of primary and secondary extramammary Paget’s disease. Br J Dermatol 2000;142:243-7.

Liao X, Liu X, Fan X, Lai J, Zhang D. Perianal Paget’s disease: a clinicopathological and immunohistochemical study of 13 cases. Diagn Pathol 2020;15:29.

Saad RS, Ghorab Z, Khalifa MA, Xu M. CDX2 as a marker for intestinal differentiation: its utility and limitations. World J Gastrointest Surg 2011;3:159-66.

Perrotto J, Abbott JJ, Ceilley RI, Ahmed I. The role of immunohistochemistry in discriminating primary from secondary extramammary Paget disease. Am J Dermatopathol 2010;32:137-43.

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 2019;6:CD009245.

Chung PH, Leong JY, Voelzke BB. Surgical experience with genital and perineal extramammary Paget’s Disease. Urology 2019;128:90-5.

Mohs FE, Blanchard L. Microscopically controlled surgery for extramammary Paget’s disease. Arch Dermatol 1979;115:706-8.

Hendi A, Brodland DG, Zitelli JA. Extramammary Paget’s disease: surgical treatment with Mohs micrographic surgery. J Am Acad Dermatol 2004;51:767-73.

Chang MS, Mulvaney PM, Danesh MJ, Feltmate CM, Schmults CD. Modified peripheral and central Mohs micrographic surgery for improved margin control in extramammary Paget disease. JAAD Case Rep 2020;7:71-3.

Kato T, Fujimoto N, Fujii N, Tanaka T. Mapping biopsy with punch biopsies to determine surgical margin in extramammary Paget’s disease. J Dermatol 2013;40:968-72.

Nakamura Y, Ishitsuka Y, Nakamura Y, Xu X, Hori-Yamada E, Ito M, et al. Modified gluteal-fold flap for the reconstruction of vulvovaginal defects. Int J Dermatol 2010;49:1182-7.

Tsutsui K, Takahashi A, Muto Y, Mizuta H, Jinnai S, Nakama K, et al. Outcomes of lymph node dissection in the treatment of extramammary Paget’s disease: a single-institution study. J Dermatol 2020;47:512-7.

Burrows NP, Jones DH, Hudson PM, Pye RJ. Treatment of extramammary Paget’s disease by radiotherapy. Br J Dermatol 1995;132:970-2.

Hata M, Koike I, Wada H, Miyagi E, Kasuya T, Kaizu H, et al. Radiation therapy for extramammary Paget’s disease: treatment outcomes and prognostic factors. Ann Oncol 2014;25:291-7.

Hata M, Koike I, Wada H, Miyagi E, Kasuya T, Kaizu H, et al. Postoperative radiation therapy for extramammary Paget’s disease. Br J Dermatol 2015;172:1014-20.

Miller RL, Gerster JF, Owens ML, Slade HB, Tomai MA. Imiquimod applied topically: a novel immune response modifier and new class of drug. Int J Immunopharmacol 1999;21:1-14.

Marchitelli C, Peremateu MS, Sluga MC, Berasategui MT, Lopez DG, Wernicke A, et al. Treatment of primary vulvar Paget disease with 5% imiquimod cream. J Low Genit Tract Dis 2014;18:347-50.

Fontanelli R, Papadia A, Martinelli F, Lorusso D, Grijuela B, Merola M, et al. Photodynamic therapy with M-ALA as non surgical treatment option in patients with primary extramammary Paget’s disease. Gynecol Oncol 2013;130:90-4.

Hata M, Koike I, Wada H, Minagawa Y, Kasuya T, Matsui T, et al. Radiation therapy for lymph node metastasis from extramammary Paget’s disease. J Eur Acad Dermatol Venereol 2014;28:873-7.

Yoshino K, Fujisawa Y, Kiyohara Y, Kadono T, Murata Y, Uhara H, et al. Usefulness of docetaxel as first-line chemotherapy for metastatic extramammary Paget’s disease. J Dermatol 2016;43:633-7.

Kato H, Watanabe S, Kariya K, Nakamura M, Morita A. Efficacy of low-dose 5-fluorouracil/cisplatin therapy for invasive extramammary Paget’s disease. J Dermatol 2018;45:560-3.

Kato J, Hida T, Yamashita T, Kamiya S, Horimoto K, Sato S, et al. Successful TS-1 monotherapy as the second-line treatment for advanced extramammary Paget’s disease: a report of two cases. J Dermatol 2018;45:80-2.

Ichiyama T, Gomi D, Fukushima T, Kobayashi T, Sekiguchi N, Sakamoto A, et al. Successful and long-term response to trastuzumab plus paclitaxel combination therapy in human epidermal growth factor receptor 2-positive extramammary Paget’s disease: a case report and review of the literature. Mol Clin Oncol 2017;7:763-6.

Downloads

Published

2024-04-23

How to Cite

1.
Dankamak P, Nanthamongkolkul K, Jiamset I, Atjimakul T, Rattanaburi A. Vulvar Paget Disease. PSU Med J [Internet]. 2024 Apr. 23 [cited 2024 Dec. 22];4(1):25-34. Available from: https://he01.tci-thaijo.org/index.php/PSUMJ/article/view/264403

Issue

Vol. 4 No. 1 (2024): Jan-Apr

Section

Review Articles

License

Copyright (c) 2024 Author and Journal

Creative Commons License

This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.