|LETTER TO THE EDITOR
|Year : 2015 | Volume
| Issue : 4 | Page : 1045
Giant vulval neurofibroma: A rare entity with review of literature
Lalita Negi, Shailja Puri Wahal, Anchana Gulati, Vijay Kaushal
Department of Pathology, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India
|Date of Web Publication||15-Feb-2016|
Shailja Puri Wahal
Department of Pathology, Indira Gandhi Medical College, Shimla - 171 001, Himachal Pradesh
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Negi L, Wahal SP, Gulati A, Kaushal V. Giant vulval neurofibroma: A rare entity with review of literature. J Can Res Ther 2015;11:1045
Vulva is a common site for epithelial and mesenchymal tumors. Common mesenchymal tumors are leiomyoma, lipoma, fibroma, neurofibroma, vascular tumors and glandular cyst. Vulval neurofibroma makes up 5% of all benign vulvar lesions. Majority of vulvar neurofibromas are associated with Neurofibromatosis type 1 (NF 1) or Von Recklinghausen's disease. We report a case of giant vulvar neurofibroma in a 25-year-old woman without any history of NF.
A 25-year-old married woman presented with 3 months history of large painless progressive increasing vulval mass. There was no history of trauma, vaginal discharge, urinary symptoms or similar swelling in any other part of the body. On examination, there was a pedunculated, soft, non-tender, mobile mass on right labium majus. The left labial fold was normal. Her laboratory investigations were within the normal limits. The abdominal and pelvic ultrasound revealed normal abdominal and pelvic organs. An X-ray of the pelvic bone was done. The pubic bones did not show invasion or destruction. The mass was excised and send for histopathological examination. A split skin graft was performed later for the skin defect.
Grossly, a mass covered by corrugated skin measuring 17 × 8 × 6 cm was received. The cut surface was solid gray-white with mucoid areas [Figure 1]. Microscopic examination revealed soft-tissue mass covered by stratified squamous epithelium. The sub-epithelial tissue showed bundles and fascicles of spindle shaped cells with bland, wavy to spindle nuclei, separated by loose myxoid areas [Figure 2]. There was sprinkling of mast cells along with thick walled blood vessels. On the basis of histopathological findings, a diagnosis of giant neurofibroma of vulva was given.
|Figure 1: Vulvar neurofibroma covered with corrugated skin. Cut surface shows gray-white solid areas with foci of mucoid degeneration|
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|Figure 2: Spindle cells with thin, spindle shaped, wavy nuclei arranged in bundles and fascicles, interspersed with mast cells along with thick walled blood vessels (H and E, ×40)|
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Vulval involvement is found in about 18% of women with NF 1 while approximately half of all vulval neurofibromas are found in women with NF 1. Although these tumors are usually small in size (<3 cm in diameter) and slow growing, giant rapidly growing solitary ones have been reported in the literature. Solitary, genital neurofibromas have been described by Venter et al. and Gordon et al. presenting with intractable chronic pelvic pain and dyspareunia respectively. Kane et al. in their study have reported a case of isolated vulval neurofibroma in an 18-month-old female child. Amita et al. and Sa'adatu et al. have reported cases of giant neurofibroma in young females.
Neurofibromas involving the female genital tract commonly involve the clitoris and the labia but vagina, cervix endometrium, myometrium and ovary may be affected and may be associated with urinary tract NF. Vulval neurofibromas have been associated with trauma such as episiotomies or other vulval injuries. The association with trauma  and urinary tract NF  should be borne in mind when managing patients with this condition. A history of trauma and urinary symptoms should be sought. It has also been suggested that examination of the genitourinary tract including cystoscopy should be undertaken in such patients.
The vulvar location of the lesion, its size, the absence of other cutaneous or visceral lesions and family history leads us to present this case of giant vulvar neurofibroma. The course of genital neurofibroma can sometime be severe with transformation into sarcoma when they are seen in the context of NF 1. Solitary lesions follow a more benign course and no cases of transformation have so far been reported. The treatment of such lesions is mostly surgical but the possibility of recurrence justifies a prolonged and regular follow-up.
For a long term follow-up, the patients should be advised to report in case of development of cutaneous lesion or any symptoms of the genito-urinary tract. Patients who express NF-1 tumor suppressor gene should definitely be followed up closely. In addition, other family members especially siblings and children should also be followed for cutaneous lesions and for over-expression of NF-1 gene.
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[Figure 1], [Figure 2]