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A patient with adenoid cystic carcinoma of Bartholin's gland is reported and the literature relevant to this disease reviewed. The clinical presentation is characterized by a vulvar mass that has existed for a prolonged period prior to the onset of symptoms, usually infection, pain, and burning. Histologically the tumor is characterized by a cribriform pattern and perineural invasion. Electron microscopy confirms the epithelial nature of this lesion. Conclusions concerning therapy are presented, including emphasis on initial radical vulvectomy and the fact that the efficacy of routine lymphadenectomy and adjuvant radiotherapy has not been demonstrated.
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