Spinal ganglioneuroma: a rare and challenging tumor in the pediatric population.

Abstract

Background: "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.

Methods: 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.

Results: 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.

Discussion: 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.

Conclusion: 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.