关键词:

神经母细胞瘤, 肾上腺, 新生儿, 治疗, 预后

Abstract:

Objective　To summarize the clinical characteristics of neonatal adrenal neuroblastoma (NB) and explore the diagnosis and treatment of neonatal adrenal NB. Methods　Clinical data were collected from 10 cases of neonatal adrenal NB diagnosed by pathology at Guangdong Provincial Maternal and Child Health Hospital from August 2018 to August 2024, including age, sex, time of discovery, staging, treatment regimens, etc, and a retrospective analysis was conducted. Results　Among the 10 patients, there were 4 males and 6 females, with the time of discovery ranging from prenatal at 31 weeks of gestation to within 1 month after birth. 7 cases were identified by prenatal ultrasound, and 3 cases were found through abdominal ultrasound due to postnatal abdominal distension. All tumors originated from the adrenal gland, with 6 on the right side and 4 on the left. Preoperative ultrasound and CT scans indicated a round solid mass in the adrenal region in 9 cases and a cystic lesion in 1 case, with the shortest diameter ranging from 1.9 to 4.1 cm and the longest diameter from 2.1 to 5.7 cm. According to the International Neuroblastoma Staging System (INSS), there were 3 cases of stage 4S with liver metastasis and 7 cases of stage 1. The age at surgery ranged from 12 to 84 days, with a median of 29 days. Pathological classification revealed 7 cases as poorly differentiated and 3 as undifferentiated. All patients showed no MYCN gene amplification and had no 1p/11q deletions. All patients were classified into the very low-risk group and underwent primary tumor resection. Among them, 2 cases with liver metastasis (1 with multiple cystic lesions in the liver and 1 with diffuse hepatic enlargement) received chemotherapy for 6 cycles according to the low-risk group protocol, while the remaining cases underwent complete resection without postoperative chemotherapy and were followed up. All 10 patients were followed for 9 to 69 months and remained alive, with 7 stage 1 patients achieving complete remission and 3 stage 4S patients living with the tumor. Conclusions　NB has its clinical specificities, mostly stage one and 4S. Currently, the main treatment is complete resection of the primary tumor lesion. In this study, tumor resection in the neonatal period was found to be safe and effective, but individualized chemotherapy was needed for children in the very low-risk group. The overall prognosis of children with NB is good.

Key words:

Neuroblastoma, Adrenal gland, Newborn, Treatment, Prognosis