Recurrent atypical myxoid fibroepithelial polyp associated with vulvar Crohn's disease

Fibroepithelial polyps of the lower female genital tract are common lesions that can rarely exhibit atypical features including increased and atypical mitoses, bizarre nuclei, and hypercellularity, a combination of findings that may suggest malignancy. Five recurrent cases have been published to date, two of which were in pregnant females; the other three followed incomplete excisions. Our case is that of a 25-year-old female with Crohn's disease who developed multiple recurrences of polypoid and domed lesions of the labium minus following surgical excision. Histologic findings in the initial and recurrent lesions were consistent with atypical myxoid fibroepithelial polyps with underlying vulvar Crohn's disease. The lesions subsequently improved with standard Crohn's treatment including 5-amino-salicylic acid (Pentasa) and prednisone. The present case represents the only example of this entity associated with Crohn's disease, and it is the only reported recurrent case not associated with pregnancy, tamoxifen administration, or positive excision margins. The clinical, microscopic, and immunohistochemical findings of this case suggest that atypical fibroepithelial polyps of the lower female genital tract, cutaneous pleomorphic fibroma, and lesions such as fibroepithelial polyps of the anus may represent variants of the same atypical reparative process.