Division of Pediatric Surgery, Department of Surgery, Children's Hospital Los Angeles, Los Angeles, California.
Department of Radiology, Children's Hospital Los Angeles, Los Angeles, California.
J Surg Res. 2020 May;249:8-12. doi: 10.1016/j.jss.2019.12.009. Epub 2020 Jan 7.
Pelvic neuroblastomas are rare and often present in children as massive tumors whose surgical resection can be associated with significant morbidity, given sacral nerve root involvement and close proximity to pelvic vascular structures. We sought to examine the characteristics of patients with pelvic neuroblastoma and the effect of extent of surgical resection on survival and surgical outcomes.
After institutional review board approval, a retrospective chart review was performed at Children's Hospital Los Angeles from 2000 to 2018. Collected data included tumor location, size, image-defined risk factors histology, stage and risk classification, amplification of the oncogene MYCN or N-myc, use of preoperative chemotherapy, and extent of surgical resection. Outcome variables included postoperative complications and survival.
Ten patients with primary pelvic neuroblastoma tumors were identified. The median age at diagnosis was 4.2 y (3 mo to 11 y). Four patients presented with a localized pelvic tumor (stage I or stage II) and underwent upfront tumor resection. Six patients presented with advanced disease (stage III or stage IV) and underwent neoadjuvant chemotherapy, followed by partial resection (30%-90% debulked). One patient experienced a complication: lower extremity hypotonia after tumor resection. One patient died from extensive metastatic disease for which no resection was attempted. The mean postoperative follow-up was 3.9 y with 90% overall survival.
Our data show that patients who undergo gross total resection for localized pelvic neuroblastoma or neoadjuvant chemotherapy, followed by partial resection for advanced disease have excellent survival. We recommend that small localized pelvic neuroblastoma undergo gross total resection and large unresectable tumors undergo neoadjuvant chemotherapy, followed by partial debulking resection to avoid neurovascular morbidity.
盆腔神经母细胞瘤较为罕见，通常表现为巨大肿瘤，由于涉及骶神经根且紧邻盆腔血管结构，手术切除可能会导致严重的发病率。我们旨在研究盆腔神经母细胞瘤患者的特征，以及手术切除范围对生存和手术结果的影响。
经机构审查委员会批准，我们对 2000 年至 2018 年期间洛杉矶儿童医院的病历进行了回顾性图表审查。收集的数据包括肿瘤位置、大小、影像学定义的危险因素、组织学、分期和风险分类、原癌基因 MYCN 或 N-myc 扩增、术前化疗的使用情况以及手术切除范围。主要结局变量包括术后并发症和生存情况。
共确定了 10 例患有原发性盆腔神经母细胞瘤的患者。诊断时的中位年龄为 4.2 岁（3 个月至 11 岁）。4 例患者表现为局限性盆腔肿瘤（Ⅰ期或Ⅱ期），并接受了肿瘤切除术。6 例患者患有晚期疾病（Ⅲ期或Ⅳ期），并接受了新辅助化疗，然后进行部分切除术（30%-90%肿瘤切除）。1 例患者术后发生并发症：肿瘤切除后下肢张力减退。1 例患者因广泛转移疾病而死亡，未尝试手术切除。平均术后随访 3.9 年，总体生存率为 90%。
我们的数据表明，对于局限性盆腔神经母细胞瘤患者，行根治性切除术，或对于晚期疾病患者，先进行新辅助化疗，然后进行部分切除术，可获得极好的生存。我们建议对于小的局限性盆腔神经母细胞瘤行根治性切除术，对于大的无法切除的肿瘤行新辅助化疗，然后进行部分肿瘤切除术，以避免神经血管并发症。
