Rare case of primary extraosseous Ewing’s sarcoma of the cervix: A rare case report

Abstract

Ewings sarcoma (ES) is an uncommon malignancy belonging to small round cell tumour family. It is an aggressive tumor with a 5-year survival rate of 60 percent. Majority of cases occur in the long bones, followed by the pelvis or ribs, and only less than 20% occur at extraosseous sites. Extraosseous ES is a rare diagnosis and is more likely to be seen in adolescents. Cervical EES is extremely uncommon, with only 28 cases reported in literature so far. Depending on the stage at diagnosis, survival in literature varies from 12 days - 4.2 years. Diagnosis is challenging and is predominantly based on immunophenotyping, along with presence of chromosomal translocations. There are no specific consensus for management, with most reported cases being treated in lines with osseus ES. We had a 75-year-old female, presenting to our hospital with complaints of scanty bleeding per vagina. Gynaecological examination and imaging revealed a localized tumor in cervix with parametrial invasion and no regional nodal involvement. Biopsy was suggestive of small round cell tumor. As the patient had a poorly differentiated malignancy, suspicious of high-grade sarcoma and a panel of immunohistochemistry (IHC) was performed, and it was finally concluded as Ewings sarcoma of the cervix. This case report highlights our experience in the diagnosis and management of this rare condition and challenges we encountered, along with detailed review of available literature.