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Case Reports

Abstract

Syringomatous carcinoma of the vulva is a rare malignant tumor originating from the eccrine sweat glands, known for its infiltrative growth and tendency for local recurrence. Typically, these tumors manifest as non-ulcerated nodules or plaques, primarily in the head and neck region, occasionally extending to the trunk. We present a unique case of a 35-year-old female with a dark mole measuring 1.5 x 1.0 cm on the vulva. Complete excision was performed, revealing nests and cords of tumor cells infiltrating the dermis, displaying significant cytologic atypia characterized by irregular nuclear contours and variably prominent nucleoli. Extensive pigment deposition and stromal sclerosis were also observed. Immunohistochemical staining demonstrated positivity for epithelial markers, including keratins (AE1/AE3) and epithelial membrane antigen (EMA), supporting the diagnosis of syringomatous carcinoma. CK7 and carcinoembryonic antigen (CEA) were negative, while SOX10 and Pan Melanin highlighted admixed, cytologically bland melanocytes within the epidermis and neoplastic nests. This case represents a highly unusual presentation of syringomatous carcinoma with associated melanocyte colonization. Due to limited data on optimal management strategies, a multidisciplinary approach involving gynecologic oncologists, dermatopathologists, and radiation oncologists is essential for treatment decisions. Long-term follow-up is crucial, considering the potential for local recurrence and metastatic spread, emphasizing the importance of comprehensive clinical management for favorable patient outcomes in this rare malignancy.