Primary Cutaneous and Subcutaneous Neuroectodermal Tumor/Ewing's Sarcoma: Long-Term Survival after Local Excision

Primitive neuroectodermal tumor/Ewing´s sarcoma (PNET/ES) is an uncommon type of small blue cell neoplasms; it rarely originates and develops in cutaneous and subcutaneous tissue and this characteristic seems to be linked to a better prognosis. However, there is no consensus on the treatment and combined therapies are the most extended choice, either chemotherapy and surgery, or radiotherapy and surgery. Further studies are needed to conclude if we should treat Primary Cutaneous Ewing Sarcoma as we treat Ewing Sarcoma. We present two cases of PNET, treated exclusively with surgical excision with negative margins, who are free of local or extended disease 13 and 26 years after surgery.

Keywords: Ewing´s Sarcoma, PNET, malignant primitive neuroectodermal tumor, cutaneous, surgery.