Division of Pediatric Surgery, Texas Children's Surgical Oncology Program, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, BCM 185, One Baylor Plaza, Houston, TX 77030, USA; Department of Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX, USA.
Division of Pediatric Surgery, Texas Children's Surgical Oncology Program, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, BCM 185, One Baylor Plaza, Houston, TX 77030, USA; Department of Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX, USA.
J Pediatr Surg. 2022 Aug;57(8):1538-1543. doi: 10.1016/j.jpedsurg.2021.07.002. Epub 2021 Jul 7.
Ganglioneuroma (GN) and ganglioneuroblastoma-intermixed (GNB-I) represent benign variants of neuroblastic tumors in children; however, differentiating from more aggressive histological variants of GNB including the nodular subtype (GNB-N) prior to resection can be challenging, even with biopsy. Currently, no standard treatment guidelines exist. The purpose of this study was to identify pre-operative characteristics of benign neuroblastic tumors and evaluate outcomes for patients who underwent surgical resection or observation.
Retrospective chart review of children treated at a single institution between 2009 and 2019 for non-metastatic tumor with a tissue diagnosis of GN, GNB-N or GNB-I. Demographics, imaging, labs, operative details and outcomes were recorded and analyzed.
Of 53 patients, 45% were male. The most common tumor location was abdomen (49%), followed by thorax (34%). Forty-five percent had at least one image defined risk factor. Biopsy was performed in 32% (17/53) and upfront surgery in 68% (36/53). Three patients (3/53, 5.6%) with biopsy demonstrating GN tumors were observed due to high surgical risk. Pathology of resected specimens demonstrated GN in 52% (26/50) and GNB-I or GNB-N in 48% (24/50). The majority of GNB tumors (75% (18/24) were GNB-I and 25% (6/24) were GNB-N. Therefore, 88% of the resected tumors were benign spectrum neuroblastic tumors (GN & GNB-I). Seven (7/50, 14%) patients experienced perioperative complication (temporary paralysis, Horner's syndrome, chylothorax, vocal cord paralysis). Recurrence was noted in 1 patient with GN (1/50, 2%) and 3 with GNB-N (3/50, 6%). There were no tumor-related deaths. Patients with GN were older than those with GNB (8.8 years (IQR 6-11.25) vs 5.6 years for GN (IQR 3-7); p = 0.01). GNB tumors were also more likely to have calcifications on imaging (63% vs. 38%, p = .01) and more commonly had MIBG avidity (88% vs 66%, p = .04). There were no significant differences in tumor size or symptoms at presentation.
In children with neuroblastic tumors, older age, CT without tumor calcifications, lack of MIBG avidity, and/or normal urine catecholamines may indicate benign GN. Close observation could be considered for asymptomatic patients meeting these criteria with biopsy-proven GN, with resection reserved for progressive growth or symptom development. However, larger, multicenter studies are needed for further validation.
IV.
神经母细胞瘤（GN）和神经母细胞瘤混合（GNB-I）是儿童神经母细胞瘤良性变异型；然而，在切除前，即使进行活检，也很难与包括结节型（GNB-N）在内的更具侵袭性的组织学变异型区分开来。目前，尚无标准的治疗指南。本研究的目的是确定良性神经母细胞瘤的术前特征，并评估接受手术切除或观察的患者的结局。
回顾性分析 2009 年至 2019 年间在一家机构治疗的非转移性肿瘤患儿的病历，这些患儿的组织学诊断为 GN、GNB-N 或 GNB-I。记录并分析了患者的人口统计学、影像学、实验室、手术细节和结局。
53 例患儿中，45%为男性。最常见的肿瘤部位是腹部（49%），其次是胸部（34%）。45%的患儿至少有一个影像学定义的危险因素。32%（17/53）的患儿行活检，68%（36/53）的患儿行直接手术。由于手术风险高，有 3 名（3/53，5.6%）活检证实为 GN 肿瘤的患儿接受了观察治疗。切除标本的病理检查显示 52%（26/50）为 GN，48%（24/50）为 GNB-I 或 GNB-N。大多数 GNB 肿瘤（75%（18/24）为 GNB-I，25%（6/24）为 GNB-N。因此，88%（48/55）的切除肿瘤为良性神经母细胞瘤（GN 和 GNB-I）。7 例（7/50，14%）患儿发生围手术期并发症（暂时性瘫痪、霍纳综合征、乳糜胸、声带麻痹）。1 例 GN 患儿（1/50，2%）和 3 例 GNB-N 患儿（3/50，6%）出现复发。无肿瘤相关死亡。GN 患儿的年龄大于 GNB 患儿（8.8 岁（IQR 6-11.25） vs GN 患儿（IQR 3-7）；p=0.01）。GNB 肿瘤在影像学上更可能有钙化（63% vs. 38%，p=0.01），更常表现为 MIBG 摄取（88% vs. 66%，p=0.04）。肿瘤大小或症状在就诊时无显著差异。
在患有神经母细胞瘤的儿童中，年龄较大、CT 无肿瘤钙化、缺乏 MIBG 摄取以及/或正常尿儿茶酚胺可能提示为良性 GN。对于活检证实为 GN 且符合这些标准的无症状患者，可以考虑密切观察，保留手术用于肿瘤进展或症状出现。然而，需要更大规模的、多中心的研究来进一步验证。
IV 级。
