Objectives Fibrosarcoma like tumours of the uterine cervix affecting premenopausal women with neurotrophic tyrosine kinase receptor (NTRK) gene rearrangements have recently been described in the literature. They are rare tumours and to our knowledge there are only 18 cases reported, none of which has occurred in the paediatric population. We describe the first case of a paediatric patient with a NTRK fusion positive fibrosarcoma-like tumour of the uterine cervix who was successfully managed with neo-adjuvant entrectinib and subsequently went on to have conservative, fertility sparing surgery.

Methods This case report reviews the case of a 13-year-old patient who presented with a 9cm NTRK-1 rearranged cervical sarcoma with fibrosarcoma like morphology. At presentation the lesion filled her vagina and pelvis and any attempt at surgical removal would have been morbid and led to loss of fertility.

Results Based upon evidence that has shown good tolerability and responses of paediatric solid tumours with NTRK gene fusions to NTRK inhibitors, both in the neoadjuvant and upfront setting, this patient was managed with neo-adjuvant entrectinib. Following a dramatic reduction in tumour size confirmed by imaging, she underwent conservative fertility sparing surgery with final histopathology showing no residual disease.

Conclusions This case highlights the importance of the investigation of NTRK fusions in fibrosarcoma like tumours of the uterine cervix, as this may open up treatment options for patients and avoids potentially morbid extensive surgery, which may impair fertility.