What is Neurofibrosarcoma of the Vulva?Neurofibrosarcoma of the vulva is an extremely rare type of soft tissue sarcoma. It is also known as malignant peripheral nerve sheath tumour (MPNST) of the Vulva. These tumours typically originate from cells that surround and protect our nerves. When it occurs in the vulva, it involves the external female genitalia, including the clitoris, the labia, and the opening of the vagina. This type of cancer originates from Schwann cells, which are cells that generate the protective myelin sheath that covers peripheral nerves. When these cells become cancerous, they can form tumours that put pressure on and damage the nerves.
SymptomsSymptoms of neurofibrosarcoma of the vulva can include:
- A lump or mass in the vulvar region
- Pain or discomfort in the vulva
- Unusual bleeding or discharge
- Changes in the skin of the vulva
Disease Aetiology (Causes)The exact cause of neurofibrosarcoma of the vulva is unknown. It may develop sporadically or in association with Neurofibromatosis type 1, a genetic condition that causes tumours to grow along nerves.
DiagnosisDiagnosis of neurofibrosarcoma of the vulva typically involves:
- Detailed medical history and physical examination
- Biopsy of the suspicious area to examine the cells under a microscope
- Imaging tests such as ultrasound, CT scan, or MRI to determine the extent and spread of the cancer
TreatmentThe treatment for neurofibrosarcoma of the vulva often includes:
- Surgery: The main treatment usually involves the removal of the tumour and a margin of healthy tissue surrounding it.
- Radiation therapy: This can be used post-surgery to kill any remaining cancer cells, or as a primary treatment if surgery is not an option.
- Chemotherapy: Given the aggressive nature of neurofibrosarcomas, chemotherapy may be used, particularly if the cancer has spread to other parts of the body.