Approaches to Incidental Intradural Tumors of the Spine in the Pediatric Population

Abstract

Background: Incidental intradural tumors of the spine in the pediatric population are rare lesions whose management remains unclear. Surgeons must balance the risks of iatrogenic deficits and complications after surgical resection against the risks from progressive growth of the tumor. Moreover, the natural history of an incidental finding can be difficult to predict. Here we review the literature on incidental intradural tumors of the spine and present considerations for their management. Summary: Growth of the tumor or changes in radiographic features are usually indications for resection. Asymptomatic lesions can be found in patients with genetic syndromes that predispose to tumor formation, such as neurofibromatosis type 1 and 2, schwannomatosis, and Von-Hippel-Lindau syndrome, and careful workup of a genetic cause is warranted in any patient presenting with multiple tumors and/or cutaneous features. Close follow-up is generally favored given the heavy tumor burden; however, some recommend pre-emptive resection to prevent permanent neurological deficits. Incidental intradural tumors can also occur in association with hydrocephalus, significant syringomyelia, and cord compression, and surgical treatment is usually warranted. Tumors may also be discovered as part of the workup for scoliosis, where they are not truly incidental to the scoliosis but rather are contributing to curve deformation. Key Messages: Thorough workup of patients for associated genetic syndromes or comorbidities should be undertaken in pediatric patients with incidental intradural tumors. Further research is needed into the natural history of these incidental lesions. Incidental tumors can often be managed conservatively with close follow-up, with surgical intervention warranted for expanding tumors or new-onset symptoms.

A comprehensive evaluation of imaging features in pediatric spinal gliomas and their value in predicting tumor grade and histology

“…In our study, when scoliosis was present, the patient also had a spinal canal widening with an underlying low-grade astrocytoma. We did not see this feature in ependymomas, although it is estimated that 20–33% of patients with intramedullary spinal cord tumors present with concomitant scoliosis including both astrocytomas and ependymomas [ 11 ]. Scoliosis results from muscular imbalance, and asymmetrical weakness due to the tumor’s effect on trunk musculature [ 11 , 32 ].…”

“…We did not see this feature in ependymomas, although it is estimated that 20–33% of patients with intramedullary spinal cord tumors present with concomitant scoliosis including both astrocytomas and ependymomas [ 11 ]. Scoliosis results from muscular imbalance, and asymmetrical weakness due to the tumor’s effect on trunk musculature [ 11 , 32 ]. Pain and spinal rigidity are also considered partly responsible for scoliosis [ 33 ].…”

“…The growth of the neoplasm leads to a gradual deformation of the spinal cord, which in turn can cause a range of symptoms, including pain, numbness, sensory deficits, motor weakness, and disturbances in bowel and bladder function [ 3 , 9 , 10 ]. Similarly, tumors with extramedullary components, depending on their location, may produce comparable symptoms [ 8 , 11 ]. In our study, the most frequently observed clinical symptoms were pain and weakness.…”

Unmasking the Mystery of A Hidden Syrinx: Case Report of Progressive Paralysis in A 20-Year-Old Male with Intact Pain and Temperature Sensations

“…9 In aymptomatic and mild cases, a conservative management plan focused on physiotherapy, avoidance of activities that cause strain on spine and close observation is usually employed. 10 In more severe symptomatic or progressive disease, the solution is surgery aimed to eliminate the syrinx and prevent further spinal cord injury. 9,10 There are two general forms of surgical treatment: restoration of normal CSF flow around the spinal cord, and directly draining the syrinx depending on the symptoms and severity of the disease.…”

“…10 In more severe symptomatic or progressive disease, the solution is surgery aimed to eliminate the syrinx and prevent further spinal cord injury. 9,10 There are two general forms of surgical treatment: restoration of normal CSF flow around the spinal cord, and directly draining the syrinx depending on the symptoms and severity of the disease. 11 Osama et al 12 reported a case of C6-C7 level syrinx presenting as neck pain, radiating to his right arm, associated with paresthesia involving Index and Middle finger and a positive spurling test with postural deviation and associated disability.…”