Atypical recurrent vulvar neurofibromatosis (von Recklinghausen's disease) with invasion / Philippine Journal of Obstetrics and Gynecology

ABSTRACT

Neurofibromatosis is defined by tumors called neurofibromas that grow along nerves in the body, or under the skin. It is an autosomal dominant disorder probably a neural crest origin, that affects all three germ layers therefore it can involve any organ system. Although eight subtypes have been proposed to date, the National Institutes of Health Consensus Development Conference has defined 2 distinct types: Neurofibromatosis type 1 or von Recklinghausen disease which affects 85 percent of patients, and Neurofibromatosis type 2, or bilateral acoustic neuroma/vestibular schwannomas which affects 10 percent of patients. Neurofibromatosis 1 is one of the most common genetic disorders that affect all races and both sexes equally. The estimated incidence rate of Neurofibromatosis in the Philippines has not yet been established due to lack of data. The clinical expression of Neurofibromatosis 1 is extremely variable bbut neurocutaneous manifestation is the most commonly observed. The cutaneous manifestation ranges from a small nodular to a giant pedunculated dermal lesion. Although recurrence of neurofibroma is common, the involvement of the female genital tract in neurofibromatosis 1 is uncommon and vulva is the most frequent genital location, but vaginal, cervical, uterine and ovarian neurofibromas have rarely been reported. This paper presents a case of the 47 year old nulligravid with recurrent vulvar neurofibroma for four times compounded by noticeable involvement of the abdomino-pelvic organs from which she underwent series of surgical excisions in order to preserve the normal appearance and much so to optimize the function of the organ affected.