Metastatic extramammary paget disease, a remarkable clinical case and a brief review of a rare disease


Extramammary Paget Disease (EMPD) is an often-misdiagnosed rare disorder, whose cause remains unknown. Diagnosis is confirmed by skin biopsy. Primary treatment for EMPD is surgery. Recurrence is common in the first two years and prognosis is good if the disease is localized and there is no underlying associated cancer. Patients with invasive and metastatic EMPD are uncommon and exhibit a poor prognosis, even when there is good response to a first chemotherapy line. Multiple chemotherapeutic regimens, with varying levels of success, have been attempted, but standard of care is not established. The central nervous system seems to be a common metastatic site with better survival than visceral metastasis.We report a case of metastatic EMPD that addresses the difficulties associated with the treatment of this rare disease, that has no current guidelines.



PlumX Metrics


Download data is not yet available.

Author Biography

Rita Sampaio, Pathology Department, Centro Hospitalar Lisboa Ocidental, Lisboa




1. St Claire K, Hoover A, Ashack K, Khachemoune A. Extramammary Paget disease. Dermatol Online J. 2019;25:1–12. Available from:
2. Merritt BG, Degesys CA, Brodland DG. Extramammary Paget Disease. Dermatol Clin. 2019;37:261–7. Available from:
3. 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. Available from:
4. Lopes Filho LL, Lopes IMRS, Lopes LRS, Enokihara MMSS, Michalany AO, Matsunaga N. Mammary and extramammary Paget’s disease. An Bras Dermatol. 2015;90:219–31.
5. Schmitt AR, Long BJ, Weaver AL, McGree ME, et al. Evidence-Based Screening Recommendations for Occult Cancers in the Setting of Newly Diagnosed Extramammary Paget Disease. Mayo Clin Proc. 2018;93:877–83. Available from:
6. Fukuda K, Funakoshi T. Metastatic extramammary Paget’s disease: Pathogenesis and novel therapeutic approach. Front Oncol. 2018;8:1–8.
7. Ohara K, Fujisawa Y, Yoshino K, Kiyohara Y, et al. A proposal for a TNM staging system for extramammary Paget disease: Retrospective analysis of 301 patients with invasive primary tumors. J Dermatol Sci. 2016;83:234–9. Available from:
8. Long B, Schmitt AR, Weaver AL, McGree M, et al. A matter of margins: Surgical and pathologic risk factors for recurrence in extramammary Paget’s disease. Gynecol Oncol. 2017;147:358–63. Available from:
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. 2019;2019.
10. Cowan RA, Black DR, Hoang LN, Park KJ, et al. A pilot study of topical imiquimod therapy for the treatment of recurrent extramammary Paget’s disease. Gynecol Oncol. 2016 [cited 2020 Aug 5];142:139–43. Available from:
11. Kilts TP, Long B, Glasgow AE, Bakkum-Gamez JN, Habermann EB, Cliby WA. Invasive vulvar extramammary Paget’s disease in the United States. Gynecol Oncol. 2020; Available from:
12. Fusumae T, Fukuda K, Hirai I, Nakamura Y, et al. Outcomes in extramammary Paget’s disease patients with brain metastasis: a retrospective analysis. J Am Acad Dermatol. 2020; Available from:
13. Yoshino K, Fujisawa Y, Kiyohara Y, Kadono T, et al. Usefulness of docetaxel as first-line chemotherapy for metastatic extramammary Paget’s disease. J Dermatol. 2016;43:633–7.
14. Tolia M, Tsoukalas N, Sofoudis C, Giaginis 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;16:10–7. Available from:
15. Yao H, Xie M, Fu S, Guo J, et al. Survival analysis of patients with invasive extramammary Paget disease: Implications of anatomic sites. BMC Cancer. 2018;18:1–9.
Case Reports
Extramammary Paget Disease, Vulvar disease, metastatic stage, brain metastasis
  • Abstract views: 365

  • PDF: 131
  • HTML: 0
How to Cite
Fernandes, L., Graça, J., Vasconcelos de Matos, L., Sampaio, R., Baleiras, M. M., Ferreira, F., Mesquita Pinto, M., Miranda, H., & Martins, A. (2020). Metastatic extramammary paget disease, a remarkable clinical case and a brief review of a rare disease. Dermatology Reports, 12(2).