Peripheral primitive neuroectodermal tumor/primary cutaneous Ewing’s sarcoma (PPNET/ES) of the upper eyelid in an adult patient

Abstract Peripheral primitive neuroectodermal tumor (PPNET) and Ewing’s sarcoma (ES) are two variants of a highly malignant bone tumor usually affecting children and young adults with a tendency for early metastasis. Cutaneous localization of PPNET/ES is very uncommon in adult age and is normally confused with other tumors. We report the first recorded case of PPNET of the eyelid in an adult patient. The patient underwent one biopsy, two surgical wide excisions, and two series of 4 cycles of chemotherapy without radiotherapy. At 8 years of follow-up from the first wide excision, the patient is tumor free. Cutaneous PPNET/ES is rare and not considered in the usual differential diagnosis of skin lesions, especially those involving the eyelid. Once diagnosed, a multidisciplinary approach to treatment offers the highest survival rates.