Magda Zanelli, Richard Wing-Cheuk Wong, Gloria Manzotti, Renzo Boldorini, Claudia Veggiani, Maria Paola Bonasoni, Loredana De Marco, Moira Ragazzi, Angela Falbo, Giacomo Santandrea, Andrea Palicelli, Stefano Ricci, Vincenzo Dario Mandato, Maria Carolina Gelli, Lorenzo Aguzzoli, Giuditta Bernardelli, Federica De Giorgi, Aleksandra Asaturova, Martina Bonacini, Maria Giulia Disanto, Antonio De Leo, Dario de Biase, Lucia Giaccherini, Alessandra Bisagni, Naveena Singh, Mila Gugnoni, Giovanni D'Ippolito, Giulia Dalla Dea, Laura Ardighieri, Laura Carpenito, Francesca Sanguedolce, Federica Torricelli, Eleonora Zanetti, Maurizio Zizzo, Valentina Mastrofilippo, Filomena Giulia Sileo, Margherita Goia, Palicelli A., Giaccherini L., Zanelli M., Bonasoni M.P., Gelli M.C., Bisagni A., Zanetti E., De Marco L., Torricelli F., Manzotti G., Gugnoni M., D'ippolito G., Falbo A.I., Sileo F.G., Aguzzoli L., Mastrofilippo V., Bonacini M., De Giorgi F., Ricci S., Bernardelli G., Ardighieri L., Zizzo M., De Leo A., Santandrea G., de Biase D., Ragazzi M., Dea G.D., Veggiani C., Carpenito L., Sanguedolce F., Asaturova A., Boldorini R., Disanto M.G., Goia M., Wong R.W.-C., Singh N., and Mandato V.D.
Simple Summary Vulvar squamous cell carcinoma (VSCC) is the most frequent malignant vulvar tumor, with a peak incidence in the 7–8th decades of life. However, VSCCs can also occur in young women. This unfortunate event is even rarer and more worrisome in pregnant women, being hard to manage for gynecologists, oncologists, and radiotherapists. Very few cases have been reported and we felt the need for an updated review on this topic. Thus, we performed a systematic literature review of VSCCs diagnosed during pregnancy, discussing the clinic-pathologic features, the implications in pregnancy outcomes, and the effects of such a diagnosis in the management of mothers and their babies. Abstract According to our systematic literature review (PRISMA guidelines), only 37 vulvar squamous cell carcinomas (VSCCs) were diagnosed during pregnancy (age range: 17–41 years). The tumor size range was 0.3–15 cm. The treatment was performed after (14/37, 38%), before (10/37, 27%), or before-and-after delivery (11/37, 30%). We found that 21/37 (57%) cases were stage I, 2 II (5%), 11 III (30%), and 3 IVB (8%). HPV-related features (condylomas/warts; HPV infection; high-grade squamous intraepithelial lesion) were reported in 11/37 (30%) cases. We also found that 9/37 (24%) patients had inflammatory conditions (lichen sclerosus/planus, psoriasis, chronic dermatitis). The time-to-recurrence/progression (12/37, 32%) ranged from 0 to 36 (mean 9) months. Eight women died of disease (22%) 2.5–48 months after diagnosis, 2 (5%) were alive with disease, and 23 (62%) were disease-free at the end of follow-up. Pregnant patients must be followed-up. Even if they are small, newly arising vulvar lesions should be biopsied, especially in women with risk factors (HPV, dermatosis, etc.). The treatment of VSCCs diagnosed in late third trimester might be delayed until postpartum. Elective cesarean section may prevent vulvar wound dehiscence. In the few reported cases, pregnancy/fetal outcomes seemed to not be affected by invasive treatments during pregnancy. However, clinicians must be careful; larger cohorts should define the best treatment. Definite guidelines are lacking, so a multidisciplinary approach and discussion with patients are mandatory.