Primary Vaginal Amelanotic Melanoma: A Diagnostic Conundrum

Primary vaginal amelanotic melanoma (PVAM) is an exceptionally rare aggressive malignancy having a poor prognosis. PVAM shows a high incidence of recurrence, regional spread, and early metastasis that contribute to a high mortality rate. The majority of primary vaginal malignant melanomas are pigmented, and <10% are amelanotic. Because of the absence of melanin pigment in tumor cells, PVAM may mimic other common vaginal malignancies having a more favorable prognosis and may lead to diagnostic conundrum. Knowledge regarding varied histological features and immunohistochemistry can help in establishing the correct diagnosis. Magnetic resonance imaging (MRI) provides valuable information related to the extent of vaginal malignancy and the involvement of regional lymph nodes. Conservative surgery is used for early-stage disease, whereas advanced stages are treated with radical surgery, chemotherapy, radiotherapy, immunotherapy, and targeted therapy.