Extraskeletal Ewing's sarcoma of the scalp

Extraskeletal Ewing's sarcoma/primitive neuroectodermal tumour (EES/PNET) i s a rare disease entity. Scalp EES/PNET has been reported rarely. We report a case of an 11-year-old boy who had painful and rapidly growing subcutane ous nodes over the scalp and neck. The final diagnosis was EES/PNET after b iopsy and immunohistochemical assay. The patient underwent surgical excisio n, chemotherapy and radiotherapy with a dose of 2000 cGy. Now he has been f ree of disease for two years. Early awareness and treatment of this rare di sease, and wide resection followed by chemotherapy and radiotherapy might i mprove patients' long-term survival.