Abstract

ABSTRACT Peripheral primitive neuroectodermal tumor of cranium or primary cranial Ewing’s sarcoma (ES) is an isolated phenomenon. So far, <100 cases are documented. The literature revealed good outcomes in favor of cranial ES due to less chances of distant metastasis; however, standardized treatment strategy is yet to developed. Here, we depicted the aggressive course of cranial ES by reporting the same in an 18-year-old young boy having right-sided scalp swelling of the 10-month duration. Despite providing combined modalities of treatment, the young boy departed his life with a short time of 12 months after diagnosis. Our case revealed the importance of early diagnosis in cranial ES. Strong emphasis should be given to employ different therapeutic approaches at the right time. Combination chemotherapy should be started as early possible and adjuvant radiotherapy is always recommended in subtotal resection. More case studies in future will also guide about independent prognostic markers of cranial ES.

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