Sacral intraspinal extradural primitive neuroectodermal tumor

Abstract Background context Intraspinal primitive neuroectodermal tumors (PNETs) are an exceedingly rare entity. A recent literature research revealed 28 cases reported. Only a few tumors in the literature were extradural in location, in the cervical and thoracic spine. The average survival after combination treatment including chemotherapy, radiation, and surgical resection is 20 months for the cases reported in the literature. Purpose We report a case of a patient with sciatica and cauda equine–like symptoms. Study design Case report. Methods Urgent sacral decompression and resection of the tumor was performed with rapid pain relief for the patient. Results Histology revealed a sacral extradural small blue-cell tumor, consistent with ES/PNET family tumors. An oncological workup revealed that the tumor presentation was metastatic with pulmonary and abdominal nodules. The patient underwent combination chemotherapy with vincristine, doxorubicin, and cyclophosphamide with mesna for 4 months. Conclusions The patient was without disease after excision, two courses of 4-month chemotherapy, and one course of 5-week radiation to the sacrum at 2 years.