Pediatric vulvar malignancies: rare but important to know

Malignancies of the vulva in the pediatric population are exceptionally rare, which makes it difficult to gain any insight into their clinicopathologic profile. In this review, we summarize all published cases of a vulva malignancy in pediatric patients (≤21 years) reported in the English language literature for the 50-year period between 1970 and 2020. We estimate that less than 100 malignancies have been reported in total, approximately 50% of which were rhabdomyosarcomas. Invasive squamous cell carcinomas, yolk sac tumors, Ewing sarcoma/primitive neuroectodermal tumors (ES/PNET) and melanomas each represented approximately 10% of reported cases. For rhabdomyosarcoma, the alveolar and embryonal subtypes were reported with equal frequency, with both representing 70% of cases combined. The average patient age was 9.8 years. 48% and 35% were Intergroup Rhabdomyosarcoma Study clinical groupings I and III respectively. Managements were generally multimodal, and overall outcomes for the group were favorable. For invasive squamous cell carcinoma, the patients were all in their teenage years, with an average age at diagnosis of 15.2 years. A small subset of cases were associated with human papillomavirus and immunosuppression, and it is possible that immunosuppression has a role in vulvar squamous carcinogenesis in this population. One case was associated with lichen sclerosus. The patients with yolk sac tumors ranged in age from less than 1 year to 20 years (mean 12) and 67% of cases were stage I at presentation. An insufficient number of cases have been reported to define their prognosis, although some cases were notably aggressive. The few reported cases of melanoma are distinctive only because they were all associated with lichen sclerosus, suggestive of some role for the latter in their pathogenesis. The average age of patients reported with ES/PNET was 15 years (range 3.3 to 20). At least half of the reported cases were advanced stage at presentation, and patient outcomes were notably poor: 62.5% were dead of disease at follow-up. Pediatric vulvar malignancies are rare and are mostly comprised of 5 entities. Their accurate pathologic classification is necessary to facilitate optimal management.