Department of Pediatric Surgery, Munich University Hospital Dr. von Hauner Children's Hospital, Munich, Germany.
Department of Radiology, University Hospital Munich, Munich, Germany.
Eur J Pediatr Surg. 2021 Aug;31(4):362-366. doi: 10.1055/s-0040-1714658. Epub 2020 Aug 30.
Pelvic neuroblastoma (NB) is a rare entity and occurs in 2 to 5% of all NBs. Surgery in the pelvic area is-even for the experienced oncological surgeon-technically challenging, as injuries of bladder and/or rectal innervation may carry lifelong consequences for the patient. Several studies have proven the impact of image-defined risk factors (IDRFs) for outcome, complications and extent of resection in NB; however, the specific role of IDRF in pelvic NB has not been investigated yet.
Patient charts were retrospectively evaluated for International Staging System stage, IDRF status, MYCN amplification, and outcome parameters.
Between 2003 and 2019, 277 NBs were surgically resected in the department of pediatric surgery of Dr. von Hauner Children's Hospital. Out of these, 11 patients (3.9%) had pelvic NB. Evaluation of the preoperative imaging showed two patients without IDRF (stage L1) and eight patients in stage L2. One patient had stage M according to distant metastasis. Patients without IDRF underwent complete macroscopical resections, whereas complete tumor removal was not possible without mutilation in patients with IDRF. At time point of diagnosis, only patients with IDRF had functional neurological problems. Three patients developed perioperative complications; all of them had at least one IDRF. Three patients developed local recurrence during the course of the disease, all of them had at least one IDRF.
Our results indicate on a preliminary level the importance of IDRF as a prognostic tool for surgical removal of pelvic NB.
盆腔神经母细胞瘤(NB)是一种罕见的实体瘤,占所有 NB 的 2%至 5%。在盆腔区域进行手术,即使对经验丰富的肿瘤外科医生来说,技术上也具有挑战性,因为膀胱和/或直肠神经损伤可能会给患者带来终身后果。多项研究已经证明了影像定义的风险因素(IDRFs)对 NB 的预后、并发症和切除范围的影响;然而,IDRF 在盆腔 NB 中的具体作用尚未得到研究。
回顾性评估了小儿外科 Dr. von Hauner 儿童医院的患者病历表,以了解国际分期系统(ISS)分期、IDRF 状态、MYCN 扩增情况和结果参数。
在 2003 年至 2019 年期间,小儿外科共手术切除了 277 例 NB,其中 11 例(3.9%)为盆腔 NB。术前影像学评估显示,2 例患者无 IDRF(L1 期),8 例患者为 L2 期。1 例患者因远处转移而处于 M 期。无 IDRF 的患者进行了完全的大体切除,而有 IDRF 的患者则无法在不致残的情况下进行完全肿瘤切除。在诊断时,只有有 IDRF 的患者才有功能性神经问题。3 例患者发生围手术期并发症;他们都至少有一个 IDRF。3 例患者在疾病过程中出现局部复发,他们都至少有一个 IDRF。
我们的结果初步表明了 IDRF 作为预测盆腔 NB 手术切除的预后工具的重要性。