An increased occurrence of this squamous cell cancer is associated with maternal ingestion of diethylstilboestrol during pregnancy. Invasive epithelial and in situ vaginal cancers have many of the same risk factors as cervical cancer, including a strong relationship to HPV infection (Daling et al. 2002). A vaginal intraepithelial neoplasia has been reported in a carrier of a deleterious PALB2 mutation (K.A. Schrader, personal communication).
Rare types of cancer of the vagina that have been reported are Brenner Tumours and leiomyomas. The latter has been reported to occur as part of a dominantly inherited syndrome of multiple adult-onset schwannomas, multiple nevi, and multiple vaginal leiomyomas. The nevi appear to be congenital in this condition. Giant hypertrophy of the labia minora and with neoplastic fibroblast and epithelial proliferation has been described in Ramon syndrome of arthritis, deafness, and pigmentary changes in the retina.
Primary vulval and vaginal extraosseous Ewing sarcoma and peripheral neuroectodermal Tumours have been described (Vang et al. 2000).