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新生儿神经母细胞瘤需要积极治疗吗?

Neonatal neuroblastoma needs the aggressive treatment?

机构信息

Department of Pediatric Surgery, Inje University Haeundae Paik Hospital, Busan, Korea.

出版信息

World J Surg. 2012 Sep;36(9):2102-7. doi: 10.1007/s00268-012-1632-y.

Abstract

BACKGROUND

Routine antenatal ultrasound scans increased the detection of the neuroblastoma (NB) in neonates. We reviewed the treatment outcome and clinical presentation of neonatal NB.

METHODS

We included patients who had pathologically confirmed NB presented within 28 days after birth from January 1999 to December 2010.

RESULTS

There were 17 patients (8 females and 9 males), which consist of 16 % of total NB cases of children in our institution. Nine were followed from prenatal period as an abdominal mass and eight were presented postnatally (5 abdominal distensions, 2 tachypnea, and 1 persistent jaundice). The primary lesion was located in adrenal gland in ten patients, retroperitoneum in four, and posterior mediastinum in three. The tumor size was median 4.1 cm (range, 3-7). The stage of the patients were as follows: stage 1 in six, stage 2 in one, stage 3 in three, stage 4S in five, and stage 4 in two. Six patients were in the low-risk group, seven were intermediate-risk group, and four were high-risk group. Thirteen showed favorable histology among 15 specimens. Five patients (29.4 %) showed MYCN amplification. The median follow-up period was 78.4 months (range, 17.4-138.6). Fifteen of 17 (88.2 %) are alive without evidence of recurrences and two patients of stage 4S with MYCN amplification in high-risk group died.

CONCLUSIONS

The overall survival of neonatal NB is 88.2 %, but we observed a high ratio of stage 4 and stage 4S tumors and MYCN amplification. We suggested that early treatment might be better for neonatal NB more than 3 cm in size. Aggressive treatment for neonatal NB could bring more favorable outcome.

摘要

背景

常规产前超声扫描增加了新生儿神经母细胞瘤(NB)的检出率。我们回顾了新生儿 NB 的治疗结果和临床表现。

方法

我们纳入了 1999 年 1 月至 2010 年 12 月期间病理确诊为出生后 28 天内发病的 NB 患儿。

结果

共有 17 例患者(8 名女性,9 名男性),占我院儿童 NB 病例总数的 16%。9 例患者从产前开始因腹部肿块而被随访,8 例患者在产后出现症状(5 例腹部膨隆,2 例呼吸急促,1 例持续黄疸)。原发肿瘤位于肾上腺 10 例,腹膜后 4 例,后纵隔 3 例。肿瘤大小中位数为 4.1cm(范围 3-7cm)。患者分期如下:Ⅰ期 6 例,Ⅱ期 1 例,Ⅲ期 3 例,ⅣS 期 5 例,Ⅳ期 2 例。6 例患者为低危组,7 例为中危组,4 例为高危组。15 例标本中有 13 例组织学表现良好。5 例(29.4%)存在 MYCN 扩增。中位随访时间为 78.4 个月(范围 17.4-138.6)。17 例患者中 15 例(88.2%)存活且无复发迹象,2 例高危组、ⅣS 期且存在 MYCN 扩增的患儿死亡。

结论

新生儿 NB 的总体生存率为 88.2%,但我们观察到大量的Ⅳ期和ⅣS 期肿瘤以及 MYCN 扩增病例。我们建议,对于大于 3cm 的新生儿 NB,早期治疗可能更好。对于新生儿 NB 进行积极治疗可能会带来更好的结果。

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