Approaches to Incidental Intradural Tumors of the Spine in the Pediatric Population. 2023

Andrew M Hersh, and Daniel Lubelski, and Nicholas Theodore, and Daniel M Sciubba, and George Jallo, and Nir Shimony
Department of Neurosurgery, Johns Hopkins School of Medicine, Baltimore, Maryland, USA, ahersh2@jhmi.edu.

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. CONCLUSIONS 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. CONCLUSIONS 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.

UI MeSH Term Description Entries
D009442 Neurilemmoma A neoplasm that arises from SCHWANN CELLS of the cranial, peripheral, and autonomic nerves. Clinically, these tumors may present as a cranial neuropathy, abdominal or soft tissue mass, intracranial lesion, or with spinal cord compression. Histologically, these tumors are encapsulated, highly vascular, and composed of a homogenous pattern of biphasic fusiform-shaped cells that may have a palisaded appearance. (From DeVita Jr et al., Cancer: Principles and Practice of Oncology, 5th ed, pp964-5) Neurinoma,Schwannoma,Schwannomatosis, Plexiform,Neurilemoma,Neurilemmomas,Neurilemomas,Neurinomas,Plexiform Schwannomatoses,Plexiform Schwannomatosis,Schwannomas
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D012600 Scoliosis An appreciable lateral deviation in the normally straight vertical line of the spine. (Dorland, 27th ed) Scolioses
D013120 Spinal Cord Neoplasms Benign and malignant neoplasms which occur within the substance of the spinal cord (intramedullary neoplasms) or in the space between the dura and spinal cord (intradural extramedullary neoplasms). The majority of intramedullary spinal tumors are primary CNS neoplasms including ASTROCYTOMA; EPENDYMOMA; and LIPOMA. Intramedullary neoplasms are often associated with SYRINGOMYELIA. The most frequent histologic types of intradural-extramedullary tumors are MENINGIOMA and NEUROFIBROMA. Intradural-Extramedullary Spinal Cord Neoplasms,Intramedullary Spinal Cord Neoplasms,Intramedullary Spinal Cord Neoplasms, Primary,Neoplasms, Spinal Cord,Primary Intramedullary Spinal Cord Neoplasms,Primary Spinal Cord Neoplasms, Intramedullary,Spinal Cord Neoplasms, Benign,Spinal Cord Neoplasms, Intradural-Extramedullary,Spinal Cord Neoplasms, Intramedullary,Spinal Cord Neoplasms, Malignant,Spinal Cord Neoplasms, Primary Intramedullary,Tumors, Spinal Cord,Intradural Extramedullary Spinal Cord Neoplasms,Neoplasm, Spinal Cord,Spinal Cord Neoplasm,Spinal Cord Neoplasms, Intradural Extramedullary,Spinal Cord Tumor,Spinal Cord Tumors,Tumor, Spinal Cord
D013131 Spine The spinal or vertebral column. Spinal Column,Vertebrae,Vertebral Column,Vertebra,Column, Spinal,Column, Vertebral,Columns, Spinal,Columns, Vertebral,Spinal Columns,Vertebral Columns
D017253 Neurofibromatoses A group of disorders characterized by an autosomal dominant pattern of inheritance with high rates of spontaneous mutation and multiple neurofibromas or neurilemmomas. NEUROFIBROMATOSIS 1 (generalized neurofibromatosis) accounts for approximately 95% of cases, although multiple additional subtypes (e.g., NEUROFIBROMATOSIS 2, neurofibromatosis 3, etc.) have been described. (From Neurochirurgie 1998 Nov;44(4):267-72) Neurofibromatosis,Neurofibromatosis Type 3,Multiple Neurofibromas,Neurofibromatosis 3,Neurofibromatosis Syndrome,Multiple Neurofibroma,Neurofibroma, Multiple,Neurofibromas, Multiple,Neurofibromatosis 3s,Neurofibromatosis Syndromes,Neurofibromatosis Type 3s,Syndrome, Neurofibromatosis,Syndromes, Neurofibromatosis,Type 3, Neurofibromatosis

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