Prognostic Factors of Extramammary Paget’s Disease
Most patients with extramammary Paget’s disease (EMPD) show a good prognosis; however, some patients develop fatal metastases. Early detection is important for improving prognosis, due to the difficulties associated with the treatment of distant EMPD metastases. Several studies have emphasized the importance of the invasion level of the primary lesion for predicting the presence of metastasis, and deeper invasion or increased thickness is correlated with poorer prognosis. Vascular tumor invasion of the primary lesion can also predict the risk of metastasis. Lymph node metastasis is a strong indicator for poor prognosis, and the number of lymph node metastases affects patient outcome, in that there is a significant difference in survival between patients with zero or one lymph node metastasis and those with more than two lymph node metastases. Serum markers may be able to predict the presence of systemic metastases, and carcinoembryonic antigen and cytokeratin 19 fragment 21-1 reflect disease progression and may be clinically valuable. Although several genetic alterations have been determined for EMPD, factors determining prognosis should be further explored.
KeywordsInvasion Genitalia Lymph node Metastasis Serum marker
Compliance with Ethical Standards
Conflict of Interest
The author declares that he has no conflict of interest.
Human and Animal Rights and Informed Consent
This article does not contain any studies with human or animal subjects performed by any of the authors.
References and Recommended Reading
Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
- 3.•• Ohara K, Fujisawa Y, Yoshino K, Kiyohara Y, Kadono T, Murata 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. Important proposal of TNM staging system.Google Scholar
- 6.•• Ito T, Kaku Y, Nagae K, Nakano-Nakamura M, Nakahara T, Oda Y, et al. Tumor thickness as a prognostic factor in extramammary Paget’s disease. J Dermatol. 2015;42:269–75. Important trial on prognostic factor in EMPD.Google Scholar
- 12.• Iacoponi S, Zalewski K, Fruscio R, Diaz-De la Noval B, De Iaco P, Ceccaroni M, et al. Prognostic factors for recurrence and survival among patients with invasive vulvar Paget disease included in the VULCAN study. Int J Gynaecol Obstet. 2016;133:76–9. Multicenter study on prognostic factors.Google Scholar
- 21.van der Linden M, Schuurman MS, Bulten J, Massuger L, IntHout J, van der Aa MA, et al. Stop routine screening for associated malignancies in cutaneous non-invasive vulvar Paget disease? Br J Dermatol. 2018.Google Scholar
- 49.Guo H, Qi RQ, Lv YN, Wang HX, Hong YX, Zheng S, et al. miR-31 is distinctively overexpressed in primary male extramammary Paget’s disease. Oncotarget. 2016;7:24559–63.Google Scholar
- 55.• Fujisawa Y, Yoshino K, Kiyohara Y, Kadono T, Murata Y, Uhara H, et al. The role of sentinel lymph node biopsy in the management of invasive extramammary Paget’s disease: multi-center, retrospective study of 151 patients. J Dermatol Sci. 2015;79:38–42. Multicenter trial on sentinel lymph node biopsy in invasive EMPD.CrossRefPubMedGoogle Scholar