# Adenoid Cystic Carcinoma of the Vulva: A Case Report

## TL;DR

A rare case of vulvar adenoid cystic carcinoma is reported, highlighting the challenges in treatment and the importance of further research.

## Contribution

This case report provides new clinical insights into managing a rare form of vulvar cancer with no established treatment guidelines.

## Key findings

- A 52-year-old woman was diagnosed with ACC of the vulva through biopsy and immunohistochemical analysis.
- The patient underwent surgery and radiotherapy with no recurrence observed for three years.
- The case emphasizes the need for improved guidelines for treating this rare malignancy.

## Abstract

Adenoid cystic carcinoma (ACC) of the vulva represents a highly uncommon type of female malignancy. Due to the absence of specific treatment guidelines, such cases are typically managed by the treatment protocols for vulvar cancer. Here, we report the case of a 52-year-old woman who presented with a painful right vulvar mass, leading to a diagnosis of ACC of the vulva after biopsy and immunohistochemical analysis. She underwent vulvectomy, bilateral inguinal lymphadenectomy, and targeted radiotherapy, and no evidence of recurrence has been found for three years, with ongoing monitoring for post-radiation effects. This case adds valuable insights into the management of ACC of the vulva and underscores the need for further research and guideline development to optimize care for future patients.