Clinical analysis and prognosis of children with neuroblastoma with pancreatic infiltration

Abstract

Objective To summarize the clinical data and characteristics of neuroblastoma (NB) with pancrea-tic infiltration and to assess the clinical features and the prognosis of NB. Methods According to NB protocol at Beijing Children′s Hospital, Capital Medical University(BCH-NB-2007), based on Hong Kong NB protocol, the patients were divided into 3 groups of low-risk (LR) group, medium risk (MR) group and high-risk (HR) group.All children were followed up till March 31, 2017.Diagnosis of pancreatic infiltration of NB was made by abdominal enhancement of CT, enhanced magnetic resonance imaging (MRI) or 18-fluorodeoxyglucose-positron emission tomography-computed tomography(18F-FDG-PET/CT), any of which could suggest NB pancreatic infiltration or postoperative pathology prompted NB to infiltrate the pancreas.Retrospective summary and analysis of indicators were performed, which included the initial diagnosis of primary tumor and metastatic tumor site, tumor markers, clinical stage, risk group, imaging features and treatment. Results (1)Totally 50 eligible patients were included: 27 females, 23 males, median age of 33 months (7-129 months), 10 cases ≤18 months, 40 cases>18 months; 3 cases were of International Neuroblastoma Staging System(INSS)-Ⅲ, 47 cases of INSS-Ⅳ; 2 caes of LR, 3 cases of MR, 45 cases of HR; 28 cases had a fever, 27 cases with abdominal mass, 14 cases with abdominal pain, 9 cases with limb pain, 5 cases with vomiting, 4 cases with diarrhea, and 1 case with jaundice.Forty-nine cases of primary tumor were located in the retroperitoneal adrenal gland, and 1 case in the pelvic cavity.Thirty-two cases had tumor diameter≥10 cm.(2)Tumor markers and imaging features: the median serum lactate dehydrogenase(LDH) value in 50 cases was 669 U/L(263-6 762 U/L), of them 19 cases >1 000 U/L.A total of 80% cases had neuron specific enolase(NSE) >0.15 ng/L.Nine cases had elevated amylase (AMY), and 7 cases had elevated lippase (LPS), and all the levels were elevated in 5 cases.A total of 41 cases had pancreas infiltration by abdominal ultrasound, 44 cases had pancreas infiltration by abdominal enhancement computed tomography (CT), 100%(14/14 cases)of pancreas infiltration was confirmed by abdominal reconstruction enhancement nuclear imaging MRI, and NB pancreas infiltration was proved in 41.3%(19/46 cases)by 18F-FDG-PET/CT.Comparison of the above 4 imaging studies: one imaging examination index was positive in 7 cases, accounting for 14.0%, 2 positive in 26 cases, accounting for 52.0%, 3 positive in 15 cases, accounting for 30.0%, and 4 positive in 2 cases, accounting for 4.0%.(3)Treatment outcomes: totally 50 cases received treatment, including 2 cases of LR, all cases were of INSS-Ⅲ, and 1 case with complete remission (CR). Three cases of MR belonging to INSS-Ⅳ had complete resection of the tumor, 1 case had recurrence and died, and the other two were stable.Forty-five cases with HR, median follow-up lasting for 15 (4-53) months, 16 cases had occurrence(35.6%), 3 cases were relapsed after stopping treatment for 2, 3, 18 months, respectively; tumor progressed in 12 patients during treatment, and 1 case got severe intracranial infection and gave up treatment before death.Kaplan-Meier analysis showed the expected 3-year event free survival(EFS) rate was 22.1%, and 3-year overall survival(OS) rate was 38.5%. Conclusions Preliminary results show that 90% with pancreatic infiltration of NB belong to Ⅳ HR group of children, and almost primary tumor is almost located in the retroperitoneal ragion.NB with pancreatic infiltration clinical manifestations is hidden and nonspecific.More than half of the children have no obvious abdominal pain or vomiting, and so imaging examination is needed to determine the situation of pancreatic metastasis further.Abdominal reconstruction enhancement MRI has a high sensitivity and specificity for pancreatic metastatic lesions, which can be used as the basis for early diagnosis.The overall prognosis is poor.The expected 3-year EFS rate can be 22.1%, 3-year OS rate was 38.5%. Key words: Neuroblastoma; Pancreatic infiltration; Clinical feature; Child