Syringoid eccrine carcinoma of the vulva: differential diagnosis, treatment, and follow-up

Purpose of Investigation: The aim of this study is to report an extremely rare case of a syringoid eccrine carcinoma (SEC) of the vulva occurred in a 33-year-old woman who referred to Cannizzaro Hospital Gynecology Unit, and to review other recent cases described in literature speculating on differential diagnosis, prognostic criteria, management, and follow up. Materials and Methods: Accepted criteria on Haematoxylin and Eosin (H&E) sections were used to make the diagnosis. Two pathologists evaluated formalin-tixed tissue sections independently, reaching the same conclusions. Fourteen manuscripts regarding various locations of syringoid eccrine carcinoma were reviewed. Results: Typical features are cells tadpole morphology and perincural and lymphovascular invasion. No standard of care has been established for the treatment of eccrine carcinoma. Wide surgical excision has been often effective. Conclusion: SEC should be taken into consideration in vulvar cancer differential diagnosis. A frequent follow up is essential to detect early recurrence or distant metastases.