Fibroma of the vulva

Case contributed by Ashesh Ishwarlal Ranchod
Diagnosis almost certain

Presentation

Progressive, painless enlargement of the vulva over a 3-month duration.

Patient Data

Age: 5 years
Gender: Female
mri

There is asymmetric, unilateral right labia majora / vulval enlargement. There is a discrete unilateral mass lesion identified within the right labia majora with extension and involvement of the superior perineum. The lesion is isointense to the muscle, remaining of low signal intensity on T1 and T2 imaging. It remains low signal intensity on STIR imaging.
There are no lipomatous or cystic components present. There is fairly homogeneous enhancement post-contrast administration. The mass measures at least 3.54 x 1.82 x 5.19 cm's (W X AP X CC) There are no flow voids or phleboliths. The lesion remains right of the midline. There is no intrapelvic extension.There is no bony infiltration or regional occult metastases.

There is benign bilateral inguinal lymphadenopathy. The bladder and uterus are normal.
The visualized bowel appears normal.

Case Discussion

Based on the imaging appearance the following differential diagnosis was offered:

  • pediatric vulval (pre-pubertal) fibroma
  • CALME (childhood asymmetry labium majus enlargement)
  • less likely hamartoma
  • less likely but possible embryonal rhabdomyosarcoma/sarcoma botryoides
  • extranodal Rosai-Dorfman disease

Histology confirmed hyalinized fibromatosis with positive Beta Catenin suggesting a histological diagnosis of a vulval fibroma in a pediatric patient.

Occasional ectopic breast fibroadenoma of the vulva is a rare differential diagnosis in an adult patient.

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