Malignant peripheral nerve sheath tumour (MPNST) of the cervix: differential diagnosis and a favourable oncological outcome with multimodality treatment

Malignant peripheral nerve sheath tumours (MPNSTs) are aggressive sarcomas that occur rarely in the cervix. Considering the varied clinical features and the absence of a pathognomonic immunohistochemical marker, it is always challenging to diagnose these tumours. Treatment has not been standardised as yet, but a combination of surgery, radiotherapy and chemotherapy is used to treat MPNSTs of the cervix. Here, we report a case of a woman in her 40s with a 10×7 cm polypoidal lesion in the cervix, diagnosed as an MPNST. She was treated with surgery, ifosfamide-based chemotherapy and external beam radiotherapy before developing pulmonary metastases. She was treated with stereotactic body radiotherapy for the pulmonary metastases, and the patient has been on follow-up since then. We are also summarising the clinicopathological findings, surgical treatment and follow-up of all reported cases of cervical MPNSTs to date.