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

Abstract

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.