| Summary: | Vulvar malignant melanoma (VMM) is uncommon and poses a significant management challenge. Here, we presented a case of VMM managed by surgery, chemoradiation, and planned for targeted therapy. A 70-year-old woman with underlying diabetes mellitus and hypertension presented with a black-colored exophytic growth around her left vulva for two months. Initial biopsy confirmed malignant melanoma with positive staining for S100, HMB 45, and Melan A. An imaging study showed that the disease was localized to the vulva. She underwent bilateral radical vulvectomy and bilateral inguinofemoral lymph node dissection followed by radiotherapy. She had a locoregional disease recurrence, which was subsequently managed by palliative perineal radiotherapy, chemotherapy, and planned for immunotherapy. Vulvar malignant melanoma is a rare and aggressive tumor, with a poor overall prognosis, and high recurrence rate. Adjuvant chemotherapy, radiotherapy, and immunotherapy may be beneficial for local recurrence and distant metastasis cases. Molecular Analysis has a potential role in targeted therapy to improve the survival and outcome of the patient. Non- ©Copyright: the Author(s), 2022 Licensee PAGEPress, Italy.
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