Background: Rectovaginal fistula in long-standing Crohn's disease is possibly associated with malignant transformation to mucinous adenocarcinoma of the vagina. However, there have been no previously reported cases documenting vulvar cancer in association with rectovaginal fistula in Crohn's disease. We report 2 cases of vulvar mucinous adenocarcinoma associated with Crohn's disease. Both showed vulvar symptoms after the development of rectovaginal fistula. CASE 1: A 48-year-old woman, with a 30-year history of Crohn's disease including a rectovaginal fistula, developed persistent pyoderma gangrenosum. Further workup revealed metastatic vulvar mucinous adenocarcinoma. CASE 2: A 37-year-old woman with long-standing Crohn's disease including numerous episodes of perianal or rectovaginal fistulas complained of a vulvar mass suspicious for an abscess. Biopsy showed mucinous adenocarcinoma.
Conclusion: Vulvar lesions or symptoms in the setting of rectovaginal fistula in Crohn's disease are an important clinical feature and the possible development of vulvar cancer should be considered.
2009 S. Karger AG, Basel.