Abstract
Purpose
This study aimed to evaluate the risk factors of recurrence and invasive disease in patients with extramammary Paget’s disease of the vulva (EPDV).
Methods
We performed a retrospective analysis of patients who were initially diagnosed with EPDV in Fudan University Shanghai Cancer Center between May 2006 and March 2019.
Results
Thirty-eight patients were initially diagnosed with EPDV in our institution. Among them, 29 had intraepithelial EPDV, 8 had intraepithelial EPDV with stromal invasion, and 1 had an underlying vulvar adenocarcinoma. In total, 8 (21%) patients had 12 recurrences. Of these eight patients, four had one recurrence, while other four had two recurrences. Intraepidermal EPDV recurred nine times, while intraepidermal EPDV with invasive disease recurred thrice. The first and second recurrence intervals were 62.1 (9–146) months and 22 (15–28) months, respectively. The rate of invasive disease was 23.7% (9/38) for primary EPDV and 25% (3/12) for recurrent ones. We determined that the presence of invasive disease was associated with a history of more than 10 years (p = 0.02) and inversely correlated with positive margins (p = 0.037), However, invasive disease had no statistical relations with age (p = 0.438), recurrence (p = 0.642), and lesion diameter (p = 0.08).
Conclusions
EPDV with a history of more than 10 years was associated with invasive disease. Close and long-term follow-up are recommended to identify those who require further treatment.
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Data availability
The raw/processed data required to reproduce these findings cannot be shared at this time since the data also forms part of an ongoing study.
Code availability
All statistical analyses were performed using SPSS Statistics (Version 22; IBM, NY, USA).
References
Kurman RJ, Carcangiu ML, Herrington CS (2014) Tumors of the vulva. In: (IARC) international agency for research on cancer. eds. WHO classification of tumors of female reproductive organs. 4th ed. Lyon, France; World Health Organization 236–237
van der Linden M, Meeuwis KA, Bulten J et al (2016) Paget disease of the vulva. Crit Rev Oncol Hematol 101:60–74
Nitecki R, Davis M, Watkins JC et al (2018) Extramammary Paget disease of the vulva: a case series examining treatment, recurrence, and malignant transformation. Int J Gynecol Cancer 28(3):632–638
Cai Y, Sheng W, Xiang L et al (2013) Primary extramammary Paget’s disease of the vulva: the clinicopathological features and treatment outcomes in a series of 43 patients. Gynecol Oncol 129(2):412–416
Shaco-Levy R, Bean SM, Vollmer RT et al (2010) Paget disease of the vulva: a study of 56 cases. Eur J Obstet Gynecol Reprod Biol 149(1):86–91
Onaiwu CO, Salcedo MP, Pessini SA et al (2016) Paget’s disease of the vulva: a review of 89 cases. Gynecol Oncol Rep 19:46–49
Hatta N, Yamada M, Hirano T et al (2008) Extramammary Paget’s disease: treatment, prognostic factors and outcome in 76 patients. Br J Dermatol 158(2):313–318
Wilkinson EJ, Brown HM (2002) Vulvar Paget disease of urothelial origin: a report of three cases and a proposed classification of vulvar Paget disease. Hum Pathol 33(5):549–554
Delport ES (2013) Extramammary Paget's disease of the vulva: an annotated review of the current literature. Australas J Dermatol 54(1):9–21
Borghi C, Bogani G, Ditto A et al (2018) Invasive Paget disease of the Vulva. Int J Gynecol Cancer 28(1):176–182
Gregori CA, Smith CI, Breen JL (1978) Extramammary Paget’s disease. Clin Obstet Gynecol 21(4):1107–1115
van der Linden M, Oonk MHM, van Doorn HC et al (2019) Vulvar Paget disease: a national retrospective cohort study. J Am Acad Dermatol 81(4):956–962
Liu G, Yuan B, Wang Y et al (2014) Clinicopathologic study of vulvar Paget’s disease in China. J Low Genit Tract Dis 18(3):281–284
Black D, Tornos C, Soslow RA et al (2007) The outcomes of patients with positive margins after excision for intraepithelial Paget's disease of the vulva. Gynecol Oncol 104(3):547–550
Nasioudis D, Bhadra M, Ko EM (2019) Extramammary Paget disease of the vulva: management and prognosis. Gynecol Oncol S0090–8258(19):31638–31645
Lee KY, Roh MR, Chung WG et al (2009) Comparison of Mohs micrographic surgery and wide excision for extramammary Paget’s disease: Korean experience. Dermatol Surg 35(1):34–40
Lee SJ, Choe YS, Jung HD et al (2011) A multicenter study on extramammary Paget’s disease in Korea. Int J Dermatol 50(5):508–515
Sopracordevole F, Di Giuseppe J, De Piero G et al (2016) Surgical treatment of paget disease of the vulva: prognostic significance of stromal invasion and surgical margin status. J Low Genit Tract Dis 20(2):184–188
Shaco-Levy R, Bean SM, Vollmer RT et al (2010) Paget disease of the vulva: a histologic study of 56 cases correlating pathologic features and disease course. Int J Gynecol Pathol 29(1):69–78
Pierie JP, Choudry U, Muzikansky A et al (2003) Prognosis and management of extramammary Paget’s disease and the association with secondary malignancies. J Am Coll Surg 196(1):45–50
Acknowledgements
We would like to thank Dr. Zhou Shuling for the pathology review.
Funding
This study was supported by the National Natural Science Foundation of China (Grant no. 81902643).
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SL: data curation; investigation; writing—original draft; writing—review and editing. JW: data curation; investigation; writing—original draft; writing—review and editing. HW: conceptualization; project administration; supervision; writing—review and editing. YR: conceptualization; funding acquisition; writing—review and editing.
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The study protocol was approved by the Ethical Committee of Fudan University Shanghai Cancer Center.
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Liang, S., Wang, J., Wang, H. et al. Clinical characteristics and risk factors of invasion in extramammary Paget’s disease of the vulva. Arch Gynecol Obstet 303, 541–546 (2021). https://doi.org/10.1007/s00404-020-05803-z
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DOI: https://doi.org/10.1007/s00404-020-05803-z