Download PDF
CC BY 4.0 · Rev Bras Ginecol Obstet 2019; 41(06): 412-416
DOI: 10.1055/s-0039-1687861
Case Report
Thieme Revinter Publicações Ltda Rio de Janeiro, Brazil

A Case of Chronic and Relapsing Paget Disease of the Vulva

Rita Bouceiro-Mendes 
1   Department of Dermatovenereology, Centro Hospitalar Universitário Lisboa Norte, Hospital de Santa Maria, Lisboa, Portugal
,
Maria Mendonça-Sanches
1   Department of Dermatovenereology, Centro Hospitalar Universitário Lisboa Norte, Hospital de Santa Maria, Lisboa, Portugal
,
Luís Soares-de-Almeida
1   Department of Dermatovenereology, Centro Hospitalar Universitário Lisboa Norte, Hospital de Santa Maria, Lisboa, Portugal
2   Institute of Molecular Medicine, Faculdade de Medicina de Lisboa, Lisboa, Portugal
,
Isabel Correia-Fonseca
1   Department of Dermatovenereology, Centro Hospitalar Universitário Lisboa Norte, Hospital de Santa Maria, Lisboa, Portugal
› Author Affiliations
Further Information

Publication History

05 December 2018

07 March 2019

Publication Date:
24 April 2019 (online)

   Permissions and Reprints

Abstract

Extramammary Paget disease is a rare neoplastic condition that more commonly affects postmenopausal Caucasian women. Although the vulvar area is the most frequently affected location, it corresponds solely to 1 to 2% of all vulvar malignancies. A 72-year-old female patient was observed in our outpatient clinic with a 2-year history of an erythematous and pruritic plaque on the vulva. Histopathology and immunohistochemistry studies were compatible with extramammary Paget disease of the vulva. Associated neoplastic conditions were excluded. Due to multiple relapses, the patient was submitted to three surgical interventions, including a total vulvectomy, and to external radiotherapy. The present case illustrates the chronic and recurrent nature of extramammary Paget disease despite aggressive procedures as well as the challenge in obtaining tumor-free resection margins.

Keywords

extramammary Paget disease - Paget disease of the vulva - total vulvectomy - radiotherapy
 

  • References

  • 1 Borghi C, Bogani G, Ditto A. , et al. Invasive Paget Disease of the Vulva. Int J Gynecol Cancer 2018; 28 (01) 176-182 . Doi: 10.1097/IGC.0000000000001131
    CrossrefPubMedGoogle Scholar
  • 2 Tolia M, Tsoukalas N, Sofoudis C. , et al. Primary extramammary invasive Paget's vulvar disease: what is the standard, what are the challenges and what is the future for radiotherapy?. BMC Cancer 2016; 16: 563 . Doi: 10.1186/s12885-016-2622-5
    CrossrefPubMedGoogle Scholar
  • 3 Matthews N, Wong V, Brooks J, Kroumpouzos G. Genital diseases in the mature woman. Clin Dermatol 2018; 36 (02) 208-221 . Doi: 10.1016/j.clindermatol.2017.10.012
    CrossrefPubMedGoogle Scholar
  • 4 Nitecki R, Davis M, Watkins JC. , et al. Extramammary Paget disease of the vulva: A Case Series Examining Treatment, Recurrence, and Malignant Transformation. Int J Gynecol Cancer 2018; 28 (03) 632-638 . Doi: 10.1097/IGC.0000000000001189
    CrossrefPubMedGoogle Scholar
  • 5 Chokoeva AA, Tchernev G, Castelli E. , et al. Vulvar cancer: a review for dermatologists. Wien Med Wochenschr 2015; 165 (7-8): 164-177 . Doi: 10.1007/s10354-015-0354-9
    CrossrefPubMedGoogle Scholar
  • 6 Lloyd J, Flanagan AM. Mammary and extramammary Paget's disease. J Clin Pathol 2000; 53 (10) 742-749 . Doi: 10.1136/jcp.53.10.742
    CrossrefPubMedGoogle Scholar
  • 7 van der Linden M, Meeuwis KAP, Bulten J, Bosse T, van Poelgeest MIE, de Hullu JA. Paget disease of the vulva. Crit Rev Oncol Hematol 2016; 101: 60-74 . Doi: 10.1016/j.critrevonc.2016.03.008
    CrossrefPubMedGoogle Scholar
  • 8 Dogan A, Hilal Z, Krentel H. , et al. Paget's Disease of the Vulva Treated with Imiquimod: Case Report and Systematic Review of the Literature. Gynecol Obstet Invest 2017; 82 (01) 1-7 . Doi: 10.1159/000449158
    CrossrefPubMed
  • 9 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; ; ( (10) CD009245 . Doi: 10.1002/14651858.CD009245.pub2
    PubMedGoogle Scholar
  • 10 Tomar TS, Sambasivan S, Nair RP, Thomas S, Ramadas PT. Extra Mammary Paget's Disease of Vulva-a Case Report. Indian J Surg Oncol 2016; 7 (04) 488-490 . Doi: 10.1007/s13193-016-0551-z
    CrossrefPubMed
  • 11 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 (05) 549-554 . Doi: 10.1053/hupa.2002.124788
    CrossrefPubMedGoogle Scholar
  • 12 Onaiwu CO, Salcedo MP, Pessini SA. , et al. Paget's disease of the vulva: A review of 89 cases. Gynecol Oncol Rep 2016; 19: 46-49 . Doi: 10.1016/j.gore.2016.12.010
    CrossrefPubMedGoogle Scholar
  • 13 Ito T, Kaku-Ito Y, Furue M. The diagnosis and management of extramammary Paget's disease. Expert Rev Anticancer Ther 2018; 18 (06) 543-553 . Doi: 10.1080/14737140.2018.1457955
    CrossrefPubMedGoogle Scholar
  • 14 Fan L, Zhu J, Tao X, Xu C. Intraepithelial Extramammary Paget's Disease of the Vulva: The Clinicopathological Characteristics, Management, and Outcome in a Study of 18 Female Patients. Dermatol Surg 2016; 42 (10) 1142-1146 . Doi: 10.1097/DSS.0000000000000903
    CrossrefPubMed
  • 15 Tagliaferri L, Casà C, Macchia G. , et al. The Role of Radiotherapy in Extramammary Paget Disease: A Systematic Review. Int J Gynecol Cancer 2018; 28 (04) 829-839 . Doi: 10.1097/IGC.0000000000001237
    CrossrefPubMed
  • 16 Del Castillo LF, Garcia C, Schoendorff C, Garcia JF, Torres LM, Garcia Almagro D. Spontaneous apparent clinical resolution with histologic persistence of a case of extramammary Paget's disease: response to topical 5-fluorouracil. Cutis 2000; 65 (05) 331-333
    PubMedGoogle Scholar
  • 17 Cowan RA, Black DR, Hoang LN. , et al. A pilot study of topical imiquimod therapy for the treatment of recurrent extramammary Paget's disease. Gynecol Oncol 2016; 142 (01) 139-143 . Doi: 10.1016/j.ygyno.2016.04.028
    CrossrefPubMedGoogle Scholar
  • 18 Eliezri YD, Silvers DN, Horan DB. Role of preoperative topical 5-fluorouracil in preparation for Mohs micrographic surgery of extramammary Paget's disease. J Am Acad Dermatol 1987; 17 (03) 497-505 . Doi: 10.1016/S0190-9622(87)70236-9
    CrossrefPubMedGoogle Scholar
  • 19 Bewley AP, Bracka A, Staughton RC, Bunker CB. Extramammary Paget's disease of the scrotum: treatment with topical 5-fluorouracil and plastic surgery. Br J Dermatol 1994; 131 (03) 445-446 . Doi: 10.1111/j.1365-2133.1994.tb08535.x
    CrossrefPubMedGoogle Scholar
  • 20 Ye JN, Rhew DC, Yip F, Edelstein L. Extramammary Paget's disease resistant to surgery and imiquimod monotherapy but responsive to imiquimod combination topical chemotherapy with 5-fluorouracil and retinoic acid: a case report. Cutis 2006; 77 (04) 245-250
    PubMedGoogle Scholar
  • 21 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 (05) 560-563 . Doi: 10.1111/1346-8138.14247
    CrossrefPubMedGoogle Scholar
  • 22 Chen K, Liang H, Peng J, Zheng Y. Successful treatment of metastatic extramammary Paget's disease with pemetrexed monotherapy systemically and 5-fluorouracil topically. Indian J Dermatol Venereol Leprol 2019; 85 (01) 56-59 . Doi: 10.4103/ijdvl.IJDVL_171_17
    CrossrefPubMedGoogle Scholar
  • 23 Flowers F. Imiquimod in the treatment of actinic keratoses and other intraepithelial neoplasms. Int J Dermatol 2002; 41 (Suppl. 01) 12-15 . Doi: 10.1111/j.1365-4632.2002.00019.x
    CrossrefPubMedGoogle Scholar
  • 24 Anton C, Luiz AV, Carvalho FM, Baracat EC, Carvalho JP. Clinical treatment of vulvar Paget's disease: a case report. Clinics (São Paulo) 2011; 66 (06) 1109-1111 . Doi: 10.1590/S1807-59322011000600033
    CrossrefPubMedGoogle Scholar
  • 25 Marchitelli C, Peremateu MS, Sluga MC. , et al. Treatment of primary vulvar paget disease with 5% imiquimod cream. J Low Genit Tract Dis 2014; 18 (04) 347-350 . Doi: 10.1097/LGT.0000000000000053
    CrossrefPubMedGoogle Scholar
  • 26 Mirer E, El Sayed F, Ammoury A, Lamant L, Messer L, Bazex J. Treatment of mammary and extramammary Paget's skin disease with topical imiquimod. J Dermatolog Treat 2006; 17 (03) 167-171 . Doi: 10.1080/09546630600788877
    CrossrefPubMedGoogle Scholar
  • 27 Luyten A, Sörgel P, Clad A. , et al. 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 (04) 644-650 . Doi: 10.1016/j.jaad.2013.12.008
    CrossrefPubMedGoogle Scholar
  • 28 Machida H, Moeini A, Roman LD, Matsuo K. Effects of imiquimod on vulvar Paget's disease: a systematic review of literature. Gynecol Oncol 2015; 139 (01) 165-171 . Doi: 10.1016/j.ygyno.2015.07.097
    CrossrefPubMedGoogle Scholar
  • 29 van der Linden M, Meeuwis K, van Hees C. , et al. The Paget Trial: A Multicenter, Observational Cohort Intervention Study for the Clinical Efficacy, Safety, and Immunological Response of Topical 5% Imiquimod Cream for Vulvar Paget Disease. JMIR Res Protoc 2017; 6 (09) e178 . Doi: 10.2196/resprot.7503
    CrossrefPubMed