A Scoping Review and Population Study Regarding Prevalence and Histopathology of Juvenile Vulvar Melanocytic Lesions. A Recommendation

Beth Morrel; Irene A M van der Avoort; Jeffrey Damman; Antien L Mooyaart; Suzanne G M A Pasmans

doi:10.1016/j.xjidi.2022.100140

A Scoping Review and Population Study Regarding Prevalence and Histopathology of Juvenile Vulvar Melanocytic Lesions. A Recommendation

JID Innov. 2022 Jun 22;2(5):100140. doi: 10.1016/j.xjidi.2022.100140. eCollection 2022 Sep.

Authors

Beth Morrel^{1

2}, Irene A M van der Avoort³, Jeffrey Damman⁴, Antien L Mooyaart⁴, Suzanne G M A Pasmans²

Affiliations

¹ Department of Obstetrics and Gynecology, Erasmus MC University Medical Center Rotterdam, Rotterdam, The Netherlands.
² Department of Dermatology, Center of Pediatric Dermatology, Sophia Children's Hospital, Erasmus MC University Medical Center Rotterdam, Rotterdam, The Netherlands.
³ Department of Obstetrics and Gynecology, Ikazia Hospital, Rotterdam, The Netherlands.
⁴ Department of Pathology, Erasmus MC University Medical Center Rotterdam, Rotterdam, The Netherlands.

Abstract

Cases of vulvar melanocytic lesions in juveniles are rarely reported. We analyze the evidence regarding vulvar melanocytic lesions in juveniles with or without vulvar lichen sclerosus to help decision making by clinicians and pathologists. A scoping review on vulvar melanocytic lesions with or without vulvar lichen sclerosus, including malignant vulvar melanomas, in females up to age 18 years was performed. In addition, the histopathology records of the cohort of all such lesions in The Netherlands from 1991 through 2020 were investigated, and a structured analysis of tissue samples of the subset of cases with lichen sclerosus was performed. The literature study performed confirms that vulvar melanomas in juveniles are extremely rare and that published case reports are often disputed. In The Netherlands, there are no cases of malignant vulvar melanomas up to age 18 years recorded from 1991 through 2020. Atypical histopathological features are often found in biopsies of vulvar nevi in juveniles, especially with concomitant lichen sclerosus, confirming earlier case studies in the literature. We conclude that even with atypical findings, vulvar melanocytic lesions in juveniles have a benign course. To avoid unnecessary and possibly mutilating procedures, we advise referral to an expert center and adaption of existing guidelines for vulvar melanocytic lesions in juveniles.

Keywords: AGN, atypical genital nevus; JVLS, juvenile vulvar lichen sclerosus; LS, lichen sclerosus; MVM, malignant vulvar melanoma; VLS, vulvar lichen sclerosus.