Primitive neuroectodermal tumors of the abdominal wall and vulva in children: Report of two cases and review of the literature  

作　　者：Qiong-Qian Xu Wen-Wen Xing Gang Chen Yi-Wu Dang Yi-Ge Luo Peng Chen Song-Wu Liang Jia-Bo Chen 

机构地区：[1]Department of Pediatric Surgery,the First Affiliated Hospital of Guangxi Medical University,Nanning 530021,Guangxi Zhuang Autonomous Region,China [2]Department of Pathology,the First Affiliated Hospital of Guangxi Medical University,Nanning 530021,Guangxi Zhuang Autonomous Region,China

出　　处：《World Journal of Clinical Cases》2019年第21期3671-3682,共12页世界临床病例杂志

基　　金：Supported by the Guangxi Natural Science Foundation Project,No.2014GXNSFAA118202

摘　　要：BACKGROUND Primitive neuroectodermal tumors are rare,highly malignant small round cell tumors belonging to the Ewing sarcoma family.The purpose of this article is to present clinical manifestation,histology,treatment,and prognosis of two primitive neuroectodermal tumors(PNETs)in extremely rare anatomic locations,the abdominal wall and vulva.CASE SUMMARY Case 1 was a 66-month-old girl with lesions on the abdominal wall;tumor size was about 3.4 cm×6.1 cm×2 cm.The patient underwent radical resection of the tumor.After the operation,an alternating vincristine,doxorubicin,and cyclophosphamide/ifosfamide and etoposide(IE)regimen was given for eight cycles,and the patient survived for 66 mo without progression.Case 2 was a 40-month-old girl,with a vulvar lesion;tumor size was about 3.3 cm×5 cm×2.5 cm.The tumor was partially resected by surgery.The family left treatment after two cycles of vincristine,pirarubicin,and cyclophosphamide/IE chemotherapy,and the patient died at home six months after surgery.CONCLUSION PNET is a rare,fast-growing,highly malignant tumor that requires histologic and molecular analyses for exact diagnosis,and multimodal treatment is required to achieve a good prognosis.BACKGROUND Primitive neuroectodermal tumors are rare, highly malignant small round cell tumors belonging to the Ewing sarcoma family.The purpose of this article is to present clinical manifestation, histology, treatment, and prognosis of two primitive neuroectodermal tumors(PNETs) in extremely rare anatomic locations,the abdominal wall and vulva.CASE SUMMARY Case 1 was a 66-month-old girl with lesions on the abdominal wall; tumor size was about 3.4 cm × 6.1 cm × 2 cm.The patient underwent radical resection of the tumor.After the operation, an alternating vincristine, doxorubicin, and cyclophosphamide/ifosfamide and etoposide(IE) regimen was given for eight cycles, and the patient survived for 66 mo without progression.Case 2 was a 40-month-old girl, with a vulvar lesion; tumor size was about 3.3 cm × 5 cm × 2.5 cm.The tumor was partially resected by surgery.The family left treatment after two cycles of vincristine, pirarubicin, and cyclophosphamide/IE chemotherapy,and the patient died at home six months after surgery.CONCLUSION PNET is a rare, fast-growing, highly malignant tumor that requires histologic and molecular analyses for exact diagnosis, and multimodal treatment is required to achieve a good prognosis.

关 键 词：PRIMITIVE neuroectodermal TUMOR Therapy PROGNOSIS Case REPORT 

分 类 号：R73[医药卫生—肿瘤]