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

摘要：

目的　总结新生儿肾上腺神经母细胞瘤（NB）的临床特点，探索新生儿肾上腺NB的诊断与治疗。方法　收集2018年8月至2024年8月在广东省妇幼保健院住院手术、经病理确诊的10例新生儿肾上腺NB临床资料，包括年龄、性别、发现时间、分期、治疗方案等，并进行回顾性分析。结果　10例患儿中男4例，女6例，发现时间为产前孕31周至出生后1个月内，其中7例在产前B超检查时发现，3例出生后体检行腹部B超检查发现。肿瘤均来源于肾上腺，右侧6例、左侧4例。术前B超及CT检查提示，肾上腺区域类圆形实性肿块9例，囊性肿物1例。肿块短径1.9～4.1 cm，长径2.1～5.7 cm。根据国际神经母细胞瘤分期系统（INSS）分期：3例4S期，伴肝脏转移；7例1期。手术年龄12～84 d，中位数29 d。病理分型：7例为低分化型，3例为未分化型。所有患儿MYCN基因均不扩增，无1p/11q缺失。所有患儿危险度分组均归为极低危组，行原发肿瘤切除。2例伴肝脏转移患儿（1例肝内多发囊性占位，1例弥漫性肝肿大）按低危组方案化疗6个疗程。其余患儿行完整切除手术，术后无化疗，规律随访观察。10例患儿随访9～69个月均生存，其中7例1期患儿达到完全缓解，3例4S期患儿带瘤生存。结论　新生儿肾上腺NB有自身临床特点，多为1期、4S期，原发病灶完整切除是主要治疗方法，新生儿期手术安全有效，极低危组的化疗仍需个体化治疗，总体预后良好。

关键词:

神经母细胞瘤, 肾上腺, 新生儿, 治疗, 预后