Neurofibroma of the external genitalia, extreme enlargement of the clitoris

Abstract

Neurofibromatosis of the genitourinary tract is rare, with a prevalence of 0.65%, and it is exceedingly rare to involve the external genitalia. Involvement of the clitoris, labia majora, and prepuce was reported with clitoromegaly being the most frequently occurring. Herein, we are reporting the case of a 6-year-old girl who was diagnosed with a neurofibroma of the clitoris; measuring 9.4 cm in its largest dimension. To the best of our knowledge, this is the largest clitoral neurofibroma reported in the literature. Due to the rarity of such cases and reports limitations in the literature, the diagnosis of neurofibroma of the external genitalia requires a high index of suspicion by health-care providers. Surgical excision and postoperative follow-up for possible recurrence remain the gold standard of management.

Keywords: Clitoris; neurofibromatosis; plexiform neurofibroma.

Figure 1

(a) Sagittal section of pelvic MRI showing a large clitoral mass containing numerous tiny individual nodules. (b and c) Axial and coronal sections show a soft-tissue mass centered at the clitorial region encasing the urethra. MRI: Magnetic resonance imaging

Figure 2

Intraoperative photography demonstrating cliteromegaly

Figure 3

Completely excised clitoral mass