Malignant peripheral nerve sheath tumor of the cervix in an adolescent with neurofibromatosis type 1: A case report and review of literature.

Malignant peripheral nerve sheath tumors (MPNSTs) of the cervix are rare, particularly in patients with neurofibromatosis type 1 (NF1). This report describes a cervical MPNST in an 18‐year‐old patient with no history of sexual activity, abnormal vaginal discharge, and prolonged menstruation. She had more than six café‐au‐lait spots on her body since birth and was diagnosed with NF1 at 2 years of age. Positron emission tomography‐computed tomography revealed a large pelvic mass and lung and bone metastases. Biopsy confirmed MPNST. Immunohistochemical staining showed diffuse positivity for CD10, approximately 30% positivity for cyclin D1, partial positivity for α‐SMA, desmin, and MyoD1, and negativity for myogenin, S‐100, and SOX‐10. A cancer gene panel identified several genetic abnormalities, but none were actionable mutations. Despite systemic chemotherapy, the tumor progressed rapidly, and the patient died 8 weeks post‐admission. Early diagnosis of MPNST is crucial. In patients with NF1, even mild symptoms can indicate MPNST. [ABSTRACT FROM AUTHOR]