Partially Plexiform Neurofibroma of the Labia Minora and Clitoral Hood-A Prognostic Dilemma.

BACKGROUND: Unlike solitary neurofibromas, neurofibromatosis type 1 (NF1 or von Recklinghausen disease) has been associated with an increased risk of malignancies. Differentiation between these 2 entities may not be straightforward. CASE REPORT: We present the exceptional case of a girl with one congenital plexiform neurofibroma of the labia minora and clitoral hood in whom the diagnosis NF1 could not been made. Although this presented a prognostic dilemma, we advised against a screening program for any of the malignancies associated with NF1. Still, long-term follow-up seems indicated for recurrence of the partially plexiform neurofibroma and a possible occurrence of malignant peripheral nerve sheath tumor.