Abstract
INTRODUCTION: Adenoid cystic carcinoma of vulva (ACC-vulva) is an extremely rare entity with <100 cases reported in the literature so far. OBJECTIVE: To study the clinical profile and outcome of ACC-vulva treated at a tertiary cancer care centre in South India. METHODS: This is a retrospective, record-based study of histopathologically confirmed cases of ACC-vulva treated at our centre from January 2005 to March 2016. RESULTS: Only four patients were diagnosed with ACC-vulva during the 11-year period under study. The longest duration of follow-up was 129 months. The age at diagnosis ranged from 32 to 43 years, with a median of 40 years. All patients were married, parous and premenopausal and presented with a painless unilateral vulval swelling. All patients had involvement of the Bartholin's gland site with normal overlying skin. In all patients, wide excision was performed. Unilateral inguinal node dissection was done in one case. Perineural infiltration was documented in two cases, while positive excision margins were present in three cases. None of the patients had any lymph node involvement at diagnosis or during follow-up. Two patients had recurrence of disease. The disease-free interval was 23 months for one patient and 118 months for the other. In both, local (vulval) and distant (multiple lung) metastases were detected simultaneously. CONCLUSION: Adenoid cystic carcinoma of vulva is an extremely rare, slowly progressing neoplasm mostly involving the Bartholin's gland. The usual treatment includes wide excision and adjuvant radiotherapy (if required). There may be late local and distant recurrence.