Primary mucinous adenocarcinoma intestinal type of the vulva arising at site of prior vulvar Crohn’s disease: A case report and review of the literature

Abstract

Introduction: Vulvar mucinous adenocarcinoma is a rare subtype that likely arises from atopic gastrointestinal tissue. The evaluation and treatment of this condition often requires a multidisciplinary approach. Case Report: Our patient is a 55-year-old female with a history of Crohn’s disease who initially presented with a painless vulvar mass associated with occasional bleeding. Notably, the mass presented at the site of a prior rectovaginal fistula. A vulvar biopsy was performed with pathology consistent with intestinal type mucinous adenocarcinoma. Further workup was notable for imaging showing retroperitoneal and pelvic metastatic lymphadenopathy, hypermetabolic liver and bony metastases, and pulmonary nodules concerning for metastases. Following discussion at tumor board, the patient was recommended treatment with 5-fluorouracil, leucovorin, oxaliplatin (FOLFOX), and bevacizumab. Unfortunately, prior to starting chemotherapy, the patient presented to the emergency department with dizziness and weakness and was found to have brain metastases. She ultimately elected to be discharged home on hospice care. Conclusion: Vulvar mucinous adenocarcinoma is a rare subtype tumor with limited literature on treatment options. For these rare cases, clinicians can consider treatment modalities for colon carcinomas rather than vulvar carcinomas.