Extraskeletal cervical Ewing sarcoma in a child mimicking tuberculous lymphadenitis: a diagnostic pitfall and review of the literature

Extraskeletal Ewing sarcoma (EES) is a rare and aggressive malignant tumor belonging to the Ewing sarcoma family of tumors, characterized by undifferentiated small round cells and recurrent chromosomal translocations involving the EWSR1 gene. Cervical localization in children is exceptional and may mimic infectious or inflammatory conditions, particularly tuberculous lymphadenitis in endemic regions. 
We report the case of an 11-year-old child presenting with a rapidly enlarging left laterocervical mass associated with constitutional symptoms. Initial imaging suggested a nodal conglomerate with deep cervical extension. A first biopsy revealed granulomatous inflammation with caseous necrosis, leading to a presumptive diagnosis of tuberculosis and initiation of antitubercular therapy. Clinical deterioration, tumor progression, and compressive symptoms prompted diagnostic reassessment. Repeat pathological evaluation with immunohistochemistry confirmed extraskeletal Ewing sarcoma. The patient was treated with alternating VDC/IE chemotherapy, resulting in an initial favorable clinical response.
This case highlights a major diagnostic pitfall and emphasizes the importance of reconsidering the diagnosis in cases of discordant clinical evolution. Early multidisciplinary management, repeat biopsy, and the use of immunohistochemical and molecular tools are essential to avoid diagnostic delay and improve outcomes.