[Clinical efficacy of combined therapy in children with stage 4 neuroblastoma]. / 儿童4期神经母细胞瘤联合治疗的临床疗效观察.

OBJECTIVES: To study the early clinical efficacy of combined therapy of stage 4 neuroblastoma. METHODS: A retrospective analysis was performed on the medical data and follow-up data of 14 children with stage 4 neuroblastoma who were diagnosed in Hong Kong University-Shenzhen Hospital from January 2016 to June 2021. RESULTS: The median age of onset was 3 years and 7.5 months in these 14 children. Among these children, 9 had positive results of bone marrow biopsy, 4 had N-Myc gene amplification, 13 had an increase in neuron-specific enolase, and 7 had an increase in vanilmandelic acid in urine. Based on the results of pathological examination, differentiated type was observed in 6 children, undifferentiated type in one child, mixed type, in one child and poorly differentiated type in 6 children. Of all the children, 10 received chemotherapy with the N7 regimen (including 2 children receiving arsenic trioxide in addition) and 4 received chemotherapy with the Rapid COJEC regimen. Thirteen children underwent surgery, 14 received hematopoietic stem cell transplantation, and 10 received radiotherapy. A total of 8 children received Ch14.18/CHO immunotherapy, among whom 1 child discontinued due to anaphylactic shock during immunotherapy, and the other 7 children completed Ch14.18/CHO treatment without serious adverse events, among whom 1 child was treated with Lu177 Dotatate 3 times after recurrence and is still undergoing chemotherapy at present. The median follow-up time was 45 months for all the 14 children. Four children experienced recurrence within 2 years, and the 2-year overall survival rate was 100%; 4 children experienced recurrence within 3 years, and 7 achieved disease-free survival within 3 years. CONCLUSIONS: Multidisciplinary combined therapy is recommended for children with stage 4 neuroblastoma and can help them achieve better survival and prognosis.

Thoracoscopic repair of oesophageal atresia: experience of 33 patients from two tertiary referral centres.

BACKGROUND: With advances in minimally invasive surgery, thoracoscopic repair of oesophageal atresia has become popular in many centres worldwide and indeed has been described as the pinnacle of neonatal surgery. Here, we report our experience in two tertiary referral centres. METHODS: Thoracoscopic technique was introduced in 2007. Thus, a retrospective review of all patients diagnosed with oesophageal atresia was carried out. Patients who had thoracoscopic repair were included, and those who had open repair due to co-morbidities were excluded. Patient demographics, operative data, complications, and associated anomalies were noted. RESULTS: A total of thirty-three patients underwent thoracoscopic repair during the time period. Thirty-one were successfully repaired thoracoscopically. Two patients had conversions due to intra-operative instability. The mean body weight of the neonates was 2.58 kg. The mean operative time was 146 min. Three patients suffered from minor anastomotic leaks, which healed on conservative management. Seven patients had anastomotic strictures, which responded successfully to endoscopic dilatation. Two patients died in the post-operative period due to pneumonia. One patient had a recurrent fistula 3 months after the primary repair, and he subsequently underwent a successful second repair. CONCLUSIONS: In experienced hands, thoracoscopic repair of oesophageal atresia is at least as good as open surgery but with less surgical trauma. Standard of post-operative care contributes significantly to post-operative outcome. Thoracoscopic technique is now our preferred approach.

[Clinicopathologic and prognostic study of pediatric immature teratoma].

OBJECTIVE: To study the clinicopathologic features and biologic behavior of pediatric immature teratoma. METHODS: The clinical data, pathologic features, immunohistochemical findings (for cyclin D1, P27 and Ki-67) and follow-up information of 39 cases of pediatric immature teratoma were analyzed. RESULTS: Amongst the 39 cases studied, 12 arose in the sacrococcygeal region, 12 in testis, 5 in retroperitoneum, 4 in ovary, 4 in abdomen and 2 in mediastinum. Histologically, 16 cases were of grade 1, 8 cases of grade 2 and 15 cases of grade 3. Seven of the cases contained foci of yolk sac tumor. Immature neuroepithelial features used in histologic grading included the presence of primitive neural tubules, immature rosettes, undifferentiated neuroblastoma cells and primitive neuroectodermal structures. Immunohistochemical study showed that cyclin D1 was positive in 3 cases of grade 1 tumors, 4 cases of grade 2 tumors and 9 cases of grade 3 tumors. The positivity rates for p27 were 8, 3 and 6 cases respectively, while those for Ki-67 were 3, 4 and 13 cases respectively. Follow-up data were available in 30 cases. Three of them, including 2 cases with histologic grade 3 (with or without yolk sac tumor component), recurred after operation. CONCLUSIONS: The expression of cyclin D1 and Ki-67 is a useful adjunct in histologic grading. On the other hand, p27 overexpression shows little correlation with tumor grade. The prognosis of immature teratoma in children is different from that in adults. Sacrococcygeal immature teratoma occurring in patients younger than 1 year old and with low histologic grade do not require postoperative chemotherapy if the tumor is completely excised. Similarly, for testicular immature teratoma occurring in patients below 1 year of age, regardless of tumor grading, need no adjunctive therapy. On the other hand, ovarian immature teratoma with high histologic grade requires postoperative chemotherapy, regardless of age of the patients. The presence of microscopic foci of yolk sac tumor is a useful predictor of recurrence in pediatric immature teratoma.