Plexiform Neurofibroma of Clitoris

Abstract

The most frequent genital presentation of neurofibromatosis in females is clitoromegaly. We report a case of a 5-year-old girl with neurofibromatosis type 1 with clitoral plexiform neurofibromatosis. Clitoroplasty was done, and the histopathology confirmed the diagnosis. Though rare, plexiform neurofibroma of clitoris should always be considered as a differential diagnosis in children with clitoromegaly before embarking on detailed investigations.

Extensive Pelvic Plexiform Neurofibroma Presenting As Clitoromegaly in a 3-Year-Old Female: Presentation and Management with MEK Inhibitor

“…5 Contiguous extension to the urinary tract, particularly the bladder, or to the retroperitoneum is possible with PN and subsequently, diagnostic imaging is important to evaluate the extent of the lesion. 1,3 Management of clitoral neurofibromas depends on the extent of involvement. Clitoroplasty with preservation of the neurovascular bundle and glandular tissue is the suggested treatment when possible.…”

“…Clitoroplasty with preservation of the neurovascular bundle and glandular tissue is the suggested treatment when possible. 3 Histopathological evaluation is essential, as malignant trans-formation of a clitoral neurofibroma has been reported. 6 Furthermore, patients should be followed for recurrence, especially if excision of the lesion was incomplete.…”

“…2 PN with genital involvement are typically congenital and in females most often present with clitoral enlargement. 3 We present a case of a 3-year-11-month-old female with a known diagnosis of NF1 who presented with clitoromegaly.…”

Extensive Plexiform Neurofibroma Presenting as Clitoromegaly in Neurofibromatosis Type 1

“…The patient underwent nerve sparing reduction clitoroplasty and kept under follow-up for recurrence. NF1 have plexiform neurofibromas which usually arise along the course of peripheral nerves (6) . The most frequent presenting sign in external genitalia is clitoromegaly which imitates penis in female and enlarged penis in males.…”

“…A mutation in the gene leads to uncontrolled cell proliferation in neurofibromas. Plexiform neurofibroma (PN) which is pathognomonic of NF1 can involve any organ in the body (6) . Neurofibromatosis divides as type1 and 2.…”