摘要
目的总结以腹泻为首发症状的神经母细胞瘤(NB)的临床特点、腹泻症状缓解与治疗、转归情况,提高对以腹泻为主要表现NB的认识。方法回顾性分析2015年-2017年北京儿童医院血液肿瘤中心病房收治的以腹泻为首发症状的NB患者临床特点及治疗、转归情况。结果入组患者共11例,中位确诊年龄为18月,中低危组(7/11),高危组(4/11)。肿瘤原发部位见于肾上腺区或腹膜后(9/11)及纵隔内(2/11),无骨髓或中枢转移,尿HVA、VMA中位值较正常升高约2.4～3.6倍,血清LDH、NSE中位值较正常范围升高约1.1～2.1倍。11例患儿中6例首先进行了手术切除肿瘤,并均在术后使腹泻得到了控制。其余5例患者先进行了术前化疗,除2例患者腹泻症状在接受化疗前自行缓解外,其余3例患者在化疗期间腹泻症状得到一定缓解,其中有1例同时应用了奥曲肽对症治疗,直到手术切除肿瘤后腹泻完全停止。所有患者化疗5～8个周期,目前中位随访时间为18个月,总体生存率及无事件生存率均为100%。结论以腹泻为首发症状的NB确诊年龄偏小,中低危组所占比例较高,肿瘤标志物的中位水平较正常值升高不显著。手术切除肿瘤是最有效缓解腹泻症状的治疗方法。
Objective To summarize the clinical features,treatment,and prognoses of neuroblastoma(NB)with diarrhea as the first symptom,providing advice for diagnosis and treatment of the special kind of NB.Methods The clinical features,treatment,and prognoses of NB patients with diarrhea as the initial symptom who were admitted to Hematology-Oncology Center,Beijing Children′s Hospital from 2015 to 2017,were studied retrospectively.Results A total of 11 patients were included in the study.The median age at diagnosis was 18 months,7/11 patients were classified as low or intermediate risk group,while 4/11 were classified as high risk group.The primary tumor was located in retroperitoneum or adrenal area(9/11)and mediastinum(2/11),no bone marrow or central metastases was found,urinary HVA,VMA,serum LDH,NSE was slightly elevated.The median follow-up time was 18 months,and the overall survival rate and event-free survival rate were both 100%.Symptoms of diarrhea in 2/11 patients resolved spontaneously before chemotherapy,and 9/11 patients stopped diarrhea after surgical resection.Conclusions Compared to other kind of NB cases,NB with diarrhea as the first symptom has a smaller age at diagnosis,with higher proportion of low or intermediate group,a higher median level of tumor markers than normal,with better prognosis.Reduce part of the assessment and intensified chemotherapy should be considered.The effect of chemotherapy on symptomatic diarrhea is poor.Surgical therapy for the tumor is the most effective treatment for relieving the symptoms of diarrhea.
作者
王欣迪
段超
黄程
徐樨巍
伏利兵
秦红
马晓莉
WANG Xindi;DUAN Chao;HUANG Cheng;XU Xiwei;FU Libing;QIN Hong;MA Xiaoli(Beijing Key Laboratory of Pediatric Hematology Oncology,National Discipline of Pediatrics,Ministry of Education,MOE Key Laboratory of Major Diseases in Children,Hematology Oncology Center,Beijing Children's Hospital,Capital Medical University,National Center for Children's Health,Beijing 100045,China;Department of Gastroenterology,Beijing Children's Hospital,Capital Medical University,Beijing 100045,China;Department of Pathology,Beijing Children's Hospital,Capital Medical University,Beijing 100045,China;Department of Oncosurgery,Beijing Children's Hospital,Capital Medical University,Beijing 100045,China)
出处
《中国小儿血液与肿瘤杂志》
CAS
2018年第6期318-322,共5页
Journal of China Pediatric Blood and Cancer
