Localized neurofibromatosis of the female genital system: A case report and review of the literature

Abstract

Neurofibromatosis within the female genital tract is uncommon. The vulva is the most frequent genital location, but it has rarely been reported in the context of the vagina, uterine cervix or ovaries. In spite of its rarity, neurofibroma is a neoplasm that should be considered in the differential diagnosis of pelvic masses, especially in patients with neurofibromatosis. In this paper we describe the case of a 71-year-old patient with pelvic pain and a uterine mass who underwent a hysterectomy after having been diagnosed with an 11-cm neurofibroma occupying the myometrium of the entire uterine corpus. There were no neurofibromas in the endometrium, serosa, fallopian tubes or ovaries. The patient had an unknown von Recklinghausen's disease.

A Neurofibromatosis Noonan Syndrome Patient Presenting with Abnormal External Genitalia

“…PNFs in external genital organs have rarely been described in NF1 patients (12,13) and to date there have been no reports of NFNS patients with PNFs invading the external genitalia. In the current literature, the hormonal analyses of all NF1 patients with genital abnormality have been found to be normal (12,13,14,15).…”

Unusual location of a plexiform neurofibroma in the fallopian tube: A case report

“…In cases of abdominal cavity neurofibromatosis, multiple neurofibromas of the gastrointestinal tract are often observed. However, neurofibromatosis of the female reproductive organs is rare and has been described in only a few reports involving mainly the ovaries and the uterus . To the best of our knowledge, neurofibroma of the fallopian tube has never been reported.…”

“…Neurofibromatosis is a heterogeneous neuroectodermal disease characterized by the presence of neurofibromas, multiple café au lait spots, intertriginous freckles and Lisch nodules . Riccardi's classification of neurofibromatosis distinguishes eight subtypes of neurofibromatosis .…”

Plexiform neurofibromatosis of vulva

“…3 Among the genitourinary neurofibromas, vulva is the most commonly affected genital organ, whereas involvement of the uterine cervix, endometrium and ovaries have been very rarely reported. 4 It may occur as solitary or multiple lesions as part of Von Recklinghausen's disease. We report a case of plexiform neurofibromatosis affecting labia majora in a 15 years old school girl.…”