Diagnosis and treatment of pediatric spinal primitive neuroectodermal tumors

Abstract

Objective To explore the clinical characteristics and treatments of pediatric spinal primitive neuroectodermal tumor(PNET) and to improve its clinical cognition and therapeutic options. Methods From January 2013 to December 2015, 1 boy and 2 girls underwent microsurgery. Their clinical data with pathologically confirmed spinal PNET were analyzed retrospectively. Their ages were 6, 7 and 9 years respectively. The most common symptom was progressively declined muscular strength. Preoperative enhanced magnetic resonance imaging(MRI) was routinely performed. Tumors were extradural(n=1) and intradural extramedullary(n=2). Tumor removal was complete(n=2) or subtotal(n=1). All resected specimens were examined by immunohistochemistry. One patient received postoperative chemotherapy. Results All of them achieved temporary postoperative relief. Histopathological tumors were characterized by uniformly small round or oval cells. Homer-Wright rosettes were observed in one patient. Immunohistochemical outcome of one patient of cPNET behaved as CD99(-) while another two cases of CD99(+ ) belonged to pPNET. Two patients died during a follow-up period of 5-14 months. Another one patient survived without tumor relapse after a 8-month follow-up. Conclusions PNET is composed of small-round tumor cells. With malignant progression and poor prognosis, it frequently occurs in children and adolescents. It should be highly suspected as PNET when progressively declined muscle strength and huge intraspinal tumor occur in children and adolescents. A definite diagnosis of PNET depends upon pathological outcomes. A combination of surgical resection and chemoradiotherapy is recommended. Key words: Ectoderm; Neoplasms; Spinal Canal