Division of Haematology & Oncology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
Pediatr Blood Cancer. 2018 May;65(5):e26964. doi: 10.1002/pbc.26964. Epub 2018 Jan 25.
Ganglioneuromas (GNs) usually demonstrate favorable histological and clinical features. Surgery is often performed due to clinical symptoms and/or theoretical concerns that GN may transform into neuroblastoma (NB); however, several studies have identified significant GN-surgical morbidities.
We compared the natural history, biological and clinical features of GN and ganglioneuroblastoma-intermixed (GNB-I) managed by surgery or observation to inform management and surveillance.
This retrospective study includes patients (n = 67) with histological diagnosis of GN (50/67) and GNB-I (17/67) at the Hospital for Sick Children between 1990 and 2014. Clinical, pathological features, tumor dimensions, and management were recorded.
Median age and maximal tumor diameter were 6 years (1.3-17.8) and 6.3 cm (1.4-16.9), respectively. Of the 67 patients, 46 (69%) had upfront surgery and 21 (31%) were observed. Of the 21 observed patients 4 later underwent resection. There were post-operative complications in 15 of the 50 (30%) surgical patients. The presence of imaging-defined risk factors correlated with complications (P = 0.005). Observed patients were older (median 8.4 vs. 5.3 years) and diagnosed more recently. Median growth was 0.3 cm/year and 6 of 21 had progressive disease (PD). At median follow-up of 2.2 years (0.2-14.3), all patients were alive and for those with evaluable imaging there were 27 complete and 10 partial responses, 19 stable and 6 PD. Pathology classification changed at resection for three cases, but no GN was reclassified to NB.
GN and GNB-I have a slow growth rate and resection can be associated with significant morbidity. Watch and wait approaches should be considered for some GN and GNB-I.
神经节细胞瘤（GNs）通常表现出良好的组织学和临床特征。由于临床症状和/或理论上担心 GN 可能转化为神经母细胞瘤（NB），常进行手术治疗；然而，几项研究已经确定了 GN 手术治疗的显著发病率。
我们比较了通过手术或观察治疗的 GN 和混杂性神经节神经母细胞瘤（GNB-I）的自然病史、生物学和临床特征，以指导管理和监测。
这项回顾性研究纳入了 1990 年至 2014 年在 SickKids 医院组织学诊断为 GN（50/67）和 GNB-I（17/67）的患者。记录了临床、病理特征、肿瘤大小和治疗方法。
中位年龄和最大肿瘤直径分别为 6 岁（1.3-17.8）和 6.3cm（1.4-16.9）。67 例患者中，46 例（69%）进行了初次手术，21 例（31%）进行了观察。21 例观察患者中，4 例后来接受了手术切除。50 例接受手术治疗的患者中有 15 例出现术后并发症。影像学定义的危险因素的存在与并发症相关（P=0.005）。观察患者年龄较大（中位年龄 8.4 岁 vs. 5.3 岁），且诊断时间较晚。中位生长速度为 0.3cm/年，21 例中有 6 例出现疾病进展（PD）。中位随访 2.2 年（0.2-14.3 年）后，所有患者均存活，可评估影像学的患者中，27 例完全缓解，10 例部分缓解，19 例稳定，6 例 PD。3 例患者在接受手术切除时病理分类发生变化，但无 GN 重新分类为 NB。
GN 和 GNB-I 的生长速度较慢，手术可能会导致严重的发病率。对于某些 GN 和 GNB-I，可以考虑观察等待的方法。
