Clinical analysis of children primitive neuroectodermal tumor / 医学研究生学报

ABSTRACT

Objective Primitive neuroectodermal tumor ( PNET) is a small round cell tumor occurring mostly in children or young adults and categorized into the Ewing sarcoma family of tumors, the purpose of the study was to investigate the clinical features, treatment and prognosis of Children PNET. Methods A retrospective study was performed on clinical data of 13 children with PNET hospitalized in our hospital from March 2010 to Octorber 2014.In order to analyze the clinical effects and prognostic results, statistical analysis was made on their clinical manifestations, CT and ultrasonic results, therapeutic schemes, postoperative pathology and immu-nohistochemical staining results, along with telephone and outpaitient follow-up of average 19 months. Results Of the 13 patients, 4 cases were central nervous system PNET(CNS-PNET) and 9 cases were peripheral PNET(pPNET).The clinical manifestations of the former were headache, vomiting, convolsion, movement disorder or decreased muscle strength, while the latter mainly showed mas-ses and abdominal distention.3 cases with metastasis to lung, lymph nodes and bones respectively showed masses on surface or in cavi-ty in imaging.Homer-Wright pseudorosette textures were found in 12 patients.Immunohistochemical results showed 11 cases with CD99(+), 9 cases with VIM(+) and 8 cases Syn( +).Among 11 patients underwent chemotherapy, 2 cases were lost to follow-up, 7 cases were in stable condition without occurrence and the other 2 cases recurred 4-6 months after chemotherapy.3 of 4 cases with CNS-PNET survived with the sequelae of decreased muscle strength, convolsion and movement disorder.1 case with CNS-PNET and 1 case with pPNET died 7 months and 16 months after diagnosis respectively. Conclusion PNET in children is a very highly malignant tumor with low long-term survival rate.Immunohistochemi-cal examination including CD99 and VIM is of important diagnostic value and CNS-PNET is prone to sequelae.