Ewing sarcoma of the spermatic cord - A rare extra-skeletal presentation

Ewing sarcoma (ES) is an aggressive small round cell malignancy that commonly arises from bone, whereas extraskeletal Ewing Sarcoma (EES) is uncommon. Involvement of the spermatic cord is exceedingly rare and may clinically mimic benign inguinoscrotal conditions, leading to diagnostic delay. We report a 57-year-old male presenting with a progressively enlarging painless left inguinoscrotal mass. Imaging revealed a large heterogeneous solid-cystic lesion extending into the pelvis with compression of adjacent structures. Fine needle aspiration suggested a round cell tumor, while core biopsy demonstrated sheets of monotonous small round cells with brisk mitoses and necrosis. Immunohistochemistry showed diffuse membranous CD99 positivity along with nuclear NKX2.2 and FLI-1 expression, confirming the diagnosis of ES. Staging evaluation showed no distant metastasis. The patient was initiated on neoadjuvant Vincristine, Actinomycin D, and Cyclophosphamide/Ifosfamide and Etoposide (VAC/IE) chemotherapy with planned interval surgical resection. This case highlights the importance of considering rare malignant tumors in atypical inguinoscrotal masses and emphasizes the role of combined morphology and immunohistochemistry in diagnosis.