Kaneko M, Iwakawa M, Ikebukuro K, Ohkawa H
Department of Pediatric Surgery, Institute of Clinical Medicine, University of Tsukuba, Ibaraki, Japan.
J Pediatr Surg. 1998 Nov;33(11):1690-4. doi: 10.1016/s0022-3468(98)90611-x.
BACKGROUND/PURPOSE: The prognosis of neuroblastoma (NB) patients when detected before 1 year of age is excellent, particularly in patients whose disease is detected by screening. In some institutions patients with stage I or II NB detected by screening have been observed closely without any treatment. Most showed tumor regression or maturation and are surviving without tumor resection. Resection of infantile NB sometimes is complicated by vascular accidents resulting in vanishing kidney or intestinal infarction. The role of surgery in the treatment of infantile NB therefore is becoming controversial. The authors have treated infants who have easily resectable primary tumors with resection and sampling for lymph node metastases. If the resection of the primary tumor seemed difficult, only biopsy was performed initially followed by chemotherapy with or without resection. The authors analyzed the clinical outcome of the infantile NB in their institution to elucidate the role of surgery in this particular group of patients.
Thirty-six patients less than 12 months of age were treated between 1982 and 1997. Twenty-five patients who showed no symptoms had NB detected by screening at 6 months. Five patients had stage IVS disease in early infancy. No patients had bone or remote lymph node metastases. N-mycamplification was not detected in any of the 28 tumors examined. There were four diploid and 12 near-triploid tumors.
Complete resection was possible in 13 stage I and II patients without any complications. Obvious lymph node metastases were not resected in 13 patients with stage II or III NB. Partial resection leaving gross residual disease was accomplished in three patients with stage III NB. Surgical resection was not attempted in four patients because of unresectability in three patients with stage III or IVS disease and because of marked tumor shrinkage after a short course of chemotherapy in one patient with stage III NB involving the celiac axis. A neonatal cervicomediastinal stage III NB recurred very rapidly after partial resection but started to regress spontaneously 2 months postoperatively. Another neonatal stage II cervical NB progressed to stage IVS with hepatomegaly causing respiratory distress but regressed spontaneously. All the patients are alive and disease free without surgical morbidity.
All of the 21 patients who underwent partial tumor resection with or without chemotherapy are alive and doing well without tumor, indicating that complete resection is not required in this particular group of patients.
背景/目的:神经母细胞瘤(NB)患者若在1岁前被检测出,预后极佳,尤其是通过筛查发现疾病的患者。在一些机构中,经筛查发现的Ⅰ期或Ⅱ期NB患者仅进行密切观察,未接受任何治疗。多数患者显示肿瘤消退或成熟,无需肿瘤切除即可存活。婴儿NB切除有时会并发血管意外,导致肾消失或肠梗死。因此,手术在婴儿NB治疗中的作用正变得具有争议性。作者对原发性肿瘤易于切除的婴儿患者进行了肿瘤切除及淋巴结转移采样。若原发性肿瘤切除似乎困难,则最初仅进行活检,随后进行化疗,化疗后可选择是否切除肿瘤。作者分析了本机构中婴儿NB的临床结局,以阐明手术在这一特定患者群体中的作用。
1982年至1997年间,对36例年龄小于12个月的患者进行了治疗。25例无症状患者在6个月时通过筛查发现NB。5例患者在婴儿早期患有ⅣS期疾病。无患者发生骨或远处淋巴结转移。在所检查的28个肿瘤中,未检测到N - myc扩增。有4个二倍体肿瘤和12个近三倍体肿瘤。
13例Ⅰ期和Ⅱ期患者实现了完整切除,无任何并发症。13例Ⅱ期或Ⅲ期NB患者未切除明显的淋巴结转移灶。3例Ⅲ期NB患者进行了部分切除,残留肉眼可见的肿瘤。4例患者未尝试手术切除,其中3例Ⅲ期或ⅣS期疾病患者因无法切除,1例Ⅲ期NB累及腹腔干的患者在短期化疗后肿瘤明显缩小。1例新生儿颈部纵隔Ⅲ期NB在部分切除后复发非常迅速,但术后2个月开始自发消退。另一例新生儿Ⅱ期颈部NB进展为ⅣS期,肝脏肿大导致呼吸窘迫,但也自发消退。所有患者均存活且无疾病,无手术并发症。
21例接受部分肿瘤切除(无论是否化疗)的患者均存活且状况良好,无肿瘤,这表明该特定患者群体无需完整切除。