Spinal cord tumors account for 1–10% of all pediatric nervous system tumors. Only 2.5–4% of pediatric spine tumors consists of schwannomas. They are 70–80% intradural in location. Shaped like a dumbbell mass, both intradural and extradural placement are seen in 15% [2]. However, the literature informs that schwannomas are most common in the cervical segment of the spine (63%), less frequently (26%) in the thoracic segment [6].
In a case report and literature review study published in 2021, it was reported that there were 6 cases of giant extradural schwannomas in the pediatric age group without accompanying fibromatosis [7–12]. No new case report has been published from 2021 to the present.
Spinal schwannomas and neurofibromatosis are two closely related diseases. Neurofibromatosis is known to occur in the brain, spinal cord and peripheral nerves. It is a multiple tumor-related genetic disease. These tumors originate from Schwann cells. The most common type is NF1, also spinal neurofibromas are more common in this type. In a series of 21 pediatric spinal schwannoma cases, it was found that 6 patients were associated with neurofibromatosis [2]. Additionally, in 1998, a study was conducted with 128 patients who present the recurrence rate of NF2 spinal schwannoma cases after resection was reported to be higher than in NF1 cases [13]. In our case, there was no finding of neurofibromatosis detected in the patient's scans that performed after the diagnosis of schwannoma.
Schwannomas are generally benign tumors with slow growth pattern. Upon reaching critical mass, they may present with different clinical presentations depending on their anatomic location and spinal level. The most common presentations of spinal schwannomas are: non-radicular pain (65.6%), radiculopathy (28.1%), sensory deficit or paresthesia (37.5%), motor weakness (18.8%), bowel or bladder dysfunction (3.1%) [14]. However, unlike the adult age group, the presenting symptoms in the pediatric patient group are progressive motor deficits (86%), localized pain (67%) and blade disturbances (29%) [2]. These presenting complaints are generally non-traumatic originated and are rarely post-traumatic related. However, pediatric patient in our case report presented post-traumatic sciatica, motor deficits and walking difficulties.
The recommended treatment for giant spinal schwannomas is total excision. Surgical approach choice depending on the location and size of the mass is ımportant at this stage. In pediatric patients, instrumentation should be avoided as much as possible. To prevent the development of postoperative kyphosis, laminoplasty is recommended over laminectomy [9–10]. In the our case, we chose to perform laminoplasty over laminectomy. Our patient is mobilized with a thoracolumbar orthosis on postoperative day 1. There was no evidence of kyphosis or significant angle changes seen in X-ray on the 3rd month of outpatient clinic.