Unusual neoplasms of the vulva: an update

Unusual vulvar neoplasms comprise a small minority of general surgical pathology cases; however the four unusual vulvar tumours presented herein were diagnosed at our institution within the last few years, underscoring the importance of recognizing these entities. We have focused our discussion on mesenchymal lesions, but also included extramammary Paget disease. Our cases include: extramammary Paget disease, solitary fibrous tumour, plexiform schwannoma and epithelioid sarcoma, proximal-type. Pathogenesis, clinical presentation, morphology, differential diagnosis, ancillary studies, treatment and prognosis, will be discussed.