Primitive Neuroectodermal Tumour of Uterine Cervix: A Rare Case Report and Review of Literature

Abstract

Primitive Neuroectodermal Tumour (PNET) of the uterine cervix is a rare entity. Till date, less than 25 cases have been reported from 1987-2021. Histopathologically, tumour is composed of small round blue cells, developing from neuroectodermal cells. Awareness of this rare entity is important from clinical, radiological and pathological aspect for prompt management. A 67-year-old female presented with abnormal vaginal bleeding and lower abdominal pain since two months. Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) of the abdomen revealed the presence of a large mass measuring 71×63×47 mm in size along with, enlarged inguinal lymph nodes. Pathological examination of the tumour revealed a small round cell neoplasm, immunohistochemically positive for CD99 (Cluster of Differentiation) and Friend Leukaemia Intergration-1 (FLI-1). Diagnosis of PNET uterine cervix was rendered. Molecular testing for (Ewing Sarcoma breakpoint region 1) EWS/FLI-1 fusion transcripts could not be performed. Despite administration of the first cycle of adjuvant chemotherapy, the patient died from disease due to pulmonary metastasis. Due to unusual site and small round cell morphology, this entity may present as a diagnostic challenge to the pathologist.

Keywords

• inguinal lymph nodes
• metastasis
• neuroectodermal cells
• small round blue cell tumour
• uterine neoplasm