Primitive Neuroectodermal Tumor of Cervix: Report of a Rare Case and Review of Literature

Abstract

Background -Primitive neurocetodermal tumor / Ewing's sarcoma (PNET/ES) of uterine cervix is extremely rare tumor and only 27 cases have so far been reported in the literature. We hereby present one case report diagnosed by histopathology and IHC and review of literature. Objective -A young girl aged 20 years was admitted. Her routine investigations and CT and MRI scan of chest, abdomen and pelvis were performed. Biopsy was taken from vaginal mass and histopathologist reported as round cell tumor. The slides were reviewed by another pathologist and found to be rhabdomyosarcoma. Thus immunohistopathology was done and a diagnosis of PNET/ES was established. Methods -The patient presented with complains of vaginal bleeding, generalized body ache, poor nutritional intake, found extensive loco regional disease with metastases to liver, lung and multiple bones, stage FIGO IV, performance score ECOG -4. Owing to her low general condition, we could not plan any anti-cancer treatment like chemotherapy or radiation therapy. She was discharged on symptomatic treatment. Results -Her biopsy and immunohistopathology turn out to be PNET/ES of uterine cervix. Conclusion -The rarity of this entity poses a challenge to pathologists and oncologists. The PNET/ES of uterine cervix can be potentially curable if presented at an early stage and found operable. IHC plays an important role in establishing this rare diagnosis.