Clinical analysis of neuroblastoma with pulmonary or pleural involvement

Objective To analyze the clinical characteristics of children with neuroblastoma (NB) complica-ted with lung or pleural metastasis, further to explore the correlation between characteristics and short-term outcome of NB, so as to provide a basis for clinical diagnosis and treatment. Methods A retrospective analysis was performed concerning the age of onset, clinical features, treatment and outcome of 36 patients with NB who were admitted at Blood Tumor Center, Beijing Children′s Hospital of Capital Medical University from December 2007 to December 2017.The diagnostic criteria, therapeutic regimen and therapeutic efficacy criteria of the enrolled children were all based on the NB protocol of Beijing Children′s Hospital of Capital Medical University (BCH-NB-2007), the clinical stage was based on international clinical stage of neuroblastoma (INSS stage), and stratified treatment was conducted according to the BCH-NB risk grouping standard.The follow-up period lasted till October 31, 2018. Results (1)The common clinical features of grouped children: 36 patients were selected into the group, accounted for 5.99% (36/601 cases) in total hospitalized NB children, they were less than 10 years old, 10 cases under 18 months, and the median age was 29.5 months (9-105 months); 20 cases were male, and 16 cases were female; the primary tumor was located in the retroperitoneal site in 19 cases, accounting for 52.78%, 9 cases in adrenal site, accounting for 25.00%, and 8 cases in mediastinal site, accounting for 22.22%.Risk groups: 29 cases were in high-risk group, 6 cases were in medium-risk group and 1 case was in low-risk group.The main symptoms were of pain onset in 8 cases, fever in 6 cases, local mass in 6 cases, abdominal mass in 4 cases, mediastinal mass in 3 cases, paleness in 3 cases, subcutaneous nodules in 2 cases, abdominal distension in 2 cases, lower limb swelling in 1 case, and diarrhea in 1 case. Among them, 16 cases had respiratory system symptoms first, accounting for 44.4%.(2)Laboratory examination: there were 35 patients of neuron-specific enolase (NSE) ≥25 μg/L on the initial diagnosis, of which 11 cases were more than 370 μg/L, the value of serum lactate dehydrogenase (LDH) ≥717.5 U/L in 25 patients, accounting for 69.44%, and 10 cases were accompanied by N-myc gene amplification.(3)Imaging examination: on the first diagnosis, chest/abdomen CT showed 75.75%(24/33 cases) of pleural or lung involvement, PET-CT showed 81.8% (27/33 cases) of pleural or lung involvement, B-ultrasound showed 41.67%(5/12 cases) of pleural or lung involvement; 3 imaging examinations showed: 1 positive in 16 cases, 2 positive in 16 cases, and 3 positive in 4 cases.(4)Comparison of clinical features of pleural and pulmonary involvement: among the 30 children with pleural involvement, 6 cases had respiratory symptoms, 3 cases had respiratory symptoms on the 6 cases with pulmonary involvement only, and 4 cases with pleural and pulmonary involvement had no respiratory symptoms at first diagnosis.(5)Treatment and outcome: 2 cases died because of critical condition after diagnosis, 5 cases didn′t receive the law treatment, 29 cases accepted law stratification treatment, among them, 1 case in low risk group, INSS-Ⅳ, alleviate current (CR); 4 cases in the moderate group, INSS-Ⅳ, CR in 2 cases, partial response (PR) in 1 case, progress in 1 case, new tumor foci occurring after chemotherapy discontinuation for 13 months. Twenty-four cases were in the high-risk group, event occurred in 7 cases (29.17%) of them, 1 case had progression by postoperative evaluation, 4 cases of progression at 1.5, 2.0, 3.0 and 6.0 months after cessation of chemotherapy, and 2 cases had recurrence at 11 and 17 months after cessation of chemotherapy.Overall survival rate (OS) was 41.4% for all children analyzed by Kaplan-Merier, and 32.9% of them were predicted to have 3-year event-free survival. Conclusions Children with pulmonary or pleural metastasis of neuroblastoma have no specific respiratory symptoms.CT scan might be a useful method for diagnosing the group Ⅳ children with pulmonary or pleural metastasis of neuroblastoma.Moreover, there seemed to be no significant correlation between the N-myc gene expression and survival prognosis of these children. Key words: Neuroblastoma; Pleural; Pulmonary; Clinical feature; Prognosis