Pediatric Neurosurgery Department, CHU de Brabois, Nancy, France.
Pediatric Visceral Surgery Department, CHU de Brabois, Nancy, France.
Childs Nerv Syst. 2024 Dec;40(12):4301-4307. doi: 10.1007/s00381-024-06603-5. Epub 2024 Sep 7.
"Spinal ganglioneuroma" is a rare entity of neuroblastic tumors, frequent among children, that has been sparsely reported in articles involving both the pediatric and adult populations. These tumors mostly arise from the sympathetic ganglia located in the paravertebral region, near the intervertebral foramina of the spine. This makes their extension into the spinal canal possible but quite rare, in a dumbbell fashion, producing radicular or medullary symptoms. We provide an extensive review of the pediatric cases found in the literature; while reporting a challenging case we have recently got to treat at the CHRU de Brabois in Nancy, France.
We searched PubMed's database for articles and abstracts related to "spinal ganglioneuromas," from inception until June 1, 2024. We combined every case among patients under 18 years of age and divided them between the different spinal areas. We excluded those that originated from outside of the spine. We particularly focused our attention on the thoraco-lumbar region, which involved our case report, since the surgical management is completely different from other regions of the spine.
As per June 1, 2024, 21 patients aged between 3 and 17 years were identified with "spinal ganglioneuromas," with a predominance of females (81%). The thoracic region of the spine seems to be the area of predilection for these tumors (42.85%), although some have been found in the cervical (9.5%), cervico-thoracic (4.76%), thoraco-lumbar (19.04%), lumbar (19.04%), and sacral (4.76%) regions.
Our specific case included long-date abdominal pain and recent progressive paraparesis as the main symptoms, with radiological manifestations of renal atrophy and hydronephrosis, due to the compression and complete displacement of the kidney, as well as scoliosis. To our knowledge, this combination of symptoms has never been seen with spinal ganglioneuromas before in the pediatric population, given the gigantic size of the tumor, and its significant extension to both the spinal canal and the retroperitoneal space, which is quite uncommon.
Spinal ganglioneuromas are very rare, benign lesions, that should be included in the differential diagnosis of dumbbell tumors that can cause in the pediatric population, alongside neurological symptoms in the limbs and back pain, thoracic, abdominal, and even urinary symptoms.
“脊柱节神经母细胞瘤”是一种罕见的神经母细胞瘤，常见于儿童，在涉及儿科和成人的文献中也有报道。这些肿瘤主要起源于位于脊柱椎间孔附近的椎旁交感神经节。这使得它们有可能向椎管内延伸，但很少见，呈哑铃状，产生神经根或髓内症状。我们对文献中发现的儿科病例进行了广泛的回顾；同时报告了一个我们最近在法国南锡 CHRU de Brabois 治疗的具有挑战性的病例。
我们在 PubMed 数据库中搜索了从创建到 2024 年 6 月 1 日与“脊柱节神经母细胞瘤”相关的文章和摘要。我们将 18 岁以下的患者的每个病例都合并在一起，并将其分为不同的脊柱区域。我们排除了那些起源于脊柱外的病例。我们特别关注涉及我们病例报告的胸腰椎区域，因为其手术管理与脊柱的其他区域完全不同。
截至 2024 年 6 月 1 日，共确定了 21 名年龄在 3 至 17 岁之间的“脊柱节神经母细胞瘤”患者，其中女性居多（81%）。脊柱胸段似乎是这些肿瘤的好发部位（42.85%），尽管有些肿瘤位于颈椎（9.5%）、颈胸段（4.76%）、胸腰椎段（19.04%）、腰椎段（19.04%）和骶骨段（4.76%）。
我们的具体病例主要症状为长期腹痛和近期进行性截瘫，影像学表现为肾脏萎缩和肾积水，由于肾脏受压和完全移位，以及脊柱侧凸。据我们所知，这种症状组合在儿科人群中以前从未与脊柱节神经母细胞瘤有关，因为肿瘤巨大，并且向椎管和腹膜后间隙显著延伸，这是相当罕见的。
脊柱节神经母细胞瘤是非常罕见的良性病变，在儿科人群中，应将其纳入可引起肢体神经症状和背痛的哑铃状肿瘤的鉴别诊断中，此外还可能出现胸、腹甚至泌尿道症状。
