INTRODUCTION
Neurobroma in isolation is a rare entity that involves the 
peripheral extremities most commonly. It usually occurs in 
patients with neurobromatosis. Neurobromatosis is an 
autosomal dominant inherited disorder with an incidence of 
approximately 1 in 3000 live births. It is characterized by 
hyper-pigmented lesions (café-au-lait spots), neurobromas, 
iris hamartomas, macrocephaly, central nervous system 
tumors, defects of skull and facial bones and vascular lesions. 
These neurobromas in the female gentital tract involve most 
commonly the vulva, clitoris and labia. They are rarely seen to 
involve the vagina, cervix, endometrium, myometrium and the 

(1)urinary tract.

Neurobromas are categorized as cutaneous, intraneural, 
massive soft tissue type and sporadic neurobromas or those 

(2, 3).in association with neurobromatosis type1  They are 
commonly asymptomatic. Vulva is a common site for epithelial 
and mesenchymal  tumors. Vulval neurobroma makes up to 

(4)5% of all benign vulvar lesions.

Majority of these are associated with Neurobromatosis type 1 
(NF 1) or Von Recklinghausen's disease. Vulval involvement is 

(5)seen in 18 % of these cases.  A few of these are seen after 
surgical trauma. They are thought to occur spontaneously in 
morphologically abnormal tissues that are subjected to 
trauma, exact etiology is however, unclear. Signicant 
complications from neurobroma of vulva are generally not 

.(6)noted because it is benign tumor . 

Although benign, some of the following are worrisome 
observations with them. 
Ÿ Stress of a neoplasm
Ÿ Cosmetic concerns
Ÿ Dyspareunia and bleeding
Ÿ Injury to vital structures while excision and surgical site 

infectionslater
Ÿ Recurrence
Ÿ Neurobromatosis type 1 related morbidity

Management  strategies  are as follows –
Ÿ Surgical excision - 
Ÿ Vulvectomy
Ÿ Myolysis –Procedure includes inserting a needle wherein 

the neoplasm is subjected to electric current or 
cryoablation.

Ÿ Tumor embolization by interventional radiology
Ÿ Radiofrequency ablation –Herein the tumor cells are 

subjected to radio waves

CASE REPORT
A 52-year-old nulligravida hypertensive female presented 
with a history of an insidious onset progressively increasing 
mass involving the vulva. The mass was not associated with 
pain. There was no history of trauma preceding the onset. 
There was no associated vaginal discharge, urinary 
symptoms or any such similar swellings in other areas. There 
was history of left sided hemiparesis and Bell's palsy 7 years 
ago. She also had Bronchial Asthma. There was also history of 
cervical lymphadenopathy 35 years ago for which AKT was 
given. She achieved menopause 3 years ago. On 
examination, there was a pedunculated, soft, non-tender, 
mobile mass on right labia majora about 4 X 3 cm. It was seen 
to involve labia minora and lower third of vagina. The left side 
was normal. Her routine blood workup was unremarkable. 
Ultrasound of abdomen and pelvis showed no abnormality. X-
ray Pelvis did not show any bony involvement. MRI Pelvis 
revealed a large well dened lobulated soft tissue mass in 
right labial region arising from circumferentially thickened 
mucosa of lower one third of vagina. Focal loss of fat planes 
with adjacent anterior wall of anal canal and adjacent right 
perineal muscles was observed. Few insignicant lymph 
nodes in bilateral internal iliac region were also noted. The 
mass biopsy was taken and sent for histopathological 
examination.  On histopathological examination the mass 
was benign. It was spindle shaped with cellular arrangement 
as fascicles and bundles. Individual tumor cells had spindle 
shaped plump nuclei showing waviness at certain places. 
Cytoplasm was eosinophilic with indistinct cellular margins. 
This was diagnostic of a Neurobroma. The mass was 
completely excised under spinal anesthesia and patient 
made an uneventful recovery. She is on routine follow-up in the 
outpatient clinic and is doing well with no signs of recurrence 
at one month follow up. 

Vulval mass 6x4 cm in Right Labial fold and lower one third 
of vagina 

Vulval mass excision under spinal anaesthesia

ISOLATED NEUROFIBROMA OF VULVA AND VAGINA: A RARE CASE 
REPORT

Original Research Paper

Dr Priya 
Bulchandani 

Resident at Dept of Obstetrics and gynecology at Grant Govt. Medical 
College & JJ group of hospitals, Mumbai

  X 39GJRA - GLOBAL JOURNAL FOR RESEARCH ANALYSIS

Gynaecology

Neurobroma is a disease of peripheral nervous system that involves most commonly the extremities. 
Amongst female genital tract, neurobroma involves most commonly the vulva, clitoris and labia but 

rarely may be seen in vagina, cervix, endometrium and myometrium as well as urinary tract. Isolated neurobroma of vagina is a 
rare association which is usually a part of Von Recklinghausen's disease. We present here a rare clinical entity of vulval 
neurobroma in a 52-year-old female with no other features of Von Recklinghausen's disease. The mass was excised surgically 
and sent for histopathological examination which conrmed the diagnosis. 

ABSTRACT

KEYWORDS : 

Dr Tushar Palve*
Asstt.Proff. at Dept of Obstetrics and gynecology at Grant Govt. Medical 
College & JJ group of hospitals, Mumbai *Corresponding Author

VOLUME-8, ISSUE-11, NOVEMBER-2019 • PRINT ISSN No. 2277 - 8160 • DOI : 10.36106/gjra



40 X GJRA - GLOBAL JOURNAL FOR RESEARCH ANALYSIS

DISCUSSION
Vulval involvement is seen in approximately 18% of women 
with NF1 . Neurobromas in the female genital tract commonly 
involve the vulva, clitoris and the labia. Rarely they involve 
vagina, cervix, endometrium, myometrium and ovaries. They 

.may also be seen in the urinary tract  Vulval neurobromas 
may result as a sequelae of surgical trauma like episiotomy or 
other vulval injuries. History of trauma and symptoms related 
to urinary tract should be sought. Genitourinary system 
examination including cystoscopy should also be considered 
while managing. The cases with NF1 may transform into 
malignant sarcomas.  However, solitary lesions are usually 
benign with no reported cases of malignant transformation. 
Reported cases have been usually small in size (<3 cm in 
diameter) and slow growing. Rarely, they may be giant lesions 
with orid growth pattern.

(3)They are usually asymptomatic but Venter et al.  and Gordon 
.(7)et al  have reported giant lesions with intractable chronic 

pelvic pain and dyspareunia.

Surgical excision seems to be the sole treatment modality, 
however, the risk of recurrence entails a close follow-up 
involving both history and clinical examination.

In this case, the vulvar location of the isolated lesion and its 
large size in absence of other cutaneous or visceral lesions or 
positive family history is striking.

Solitary, genital neurobromas have been described by 
(3) (7)Venter et al. and Gordon et al. presenting with intractable 

chronic pelvic pain and dyspareunia respectively. But the rate 
of growth of the tumor in our case was not very large compared 

(3)to two cases reported by Venter et al

(8)Kane et al.  in their study have reported a case of isolated 
vulval neurobroma in an 18-month-old female child. In our 
report patient was 52 years with both vulval and vaginal 

(10)involvement Sa'adatu et al.  have reported case of 
neurobroma of labium majus in young female as globular 
pedunculated cutaneous tissue.

(9)Amita et al.  and Negi et al. have reported a case of giant (11)

vulval neurobroma of 17x8x6 cm presenting as soft tissue 
(12)mass Hussein L Kidanto et al. have reported a large 

neurobroma of labia majora presenting as globular 
(13)pedunculated mass of 20x20 cm Nibhoria S et al reported an 

Isolated Vaginal Neurobroma presenting as Vaginal Wall 
Cyst as a rare case report with Review of Literature . Our 
patient presented with mass in vulva and vagina.

(14) (15)Mourali et al and Yayli et al  reported a 71 year old patient 
with vaginal neurobroma with Von Recklinghausen disease. 
Our patient was at 52 years old and hasn't had Von 
Recklinghausen disease.

(16)Baulies et al  reported a 20-year-old woman with a history of 
type-1  neurobromatos is  wi th  a  vaginal  nodule 
neurobroma. But our patient did not have history of type-1 
neurobromatosis.

( 1 7 )Sharma et  al  reported huge localized vaginal 
neurobromatosis as an unusual cause of postmenopausal 
bleeding . In our report, the bleeding was not a manifestation.

(18)Eusebi and Schönauer  reported Pigmented vaginal 
neurobroma. In our report there were no pigmentations .

(19)Gold  published about Neurobromatosis of the bladder 
and vagina. In our report the vagina and vulva was involved.

(20)Drescher and Herzog  published about neurobromatosis of 
the vulva and vagina. In our report patient did not have any 

features of von Recklinghausen disease.

(21)Marmey and Lacroix  published about Recklinghausen 
disease and pregnancy. But our patient was not pregnant at 
presentation and the neurobroma was solitary.

(22)Belvederi et al  reported Anatomo-clinical ndings on a case 
of neurobroma of the vagina. 

During follow up, the patients should be advised to report in 
case of development of cutaneous lesion or any symptoms of 
the genitourinary tract. 

CONCLUSION 
To conclude isolated neurobromas of female genital tract is 
not a common entity. The evaluation should consist of history 
and examination for presence of NF1 features and exclude 
genitourinary involvement. The rare incidence of these being 
in isolation with risk of recurrence and rare likelihood of 
malignant transformation emphasizes formulation of plan for 
close follow up of these patients post-surgical excision. 
Although rare, they should be considered as a differential of 
benign solid tumors of vagina.

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