Cervical
30 %
Cervicothoracic
25 %
Thoracic
29 %
Conus
15 %
Table 18.2
Intramedullary tumors’ major characteristics
Tumor type | Incidence | Presentation | MRI appearance | Treatment | Outcome |
---|---|---|---|---|---|
Ependymoma | Most common intramedullary spinal cord tumor | Mixed sensorimotor syndrome is most common. Syringomyelic syndrome may be present | Hyperintense on T2 and hypo- or isointense on T1 with heterogeneous contrast enhancement | Surgery is the most effective treatment. Radiotherapy is reserved for recurrent or malignant tumors | Local control rate 90–100 % after complete resection |
Astrocytoma | Second most common intramedullary spinal cord tumor | Mixed sensorimotor syndrome is most common. Syringomyelic syndrome may be present | Fusiform expansion with irregular margins. Often with a cystic component, associated edema, or syrinx. Hypo- to isointense on T1, hyperintense on T2, with variable contrast enhancement | Maximal safe surgical resection followed by observation or radiotherapy. Total resection is accomplished infrequently. Radiotherapy only indicated for clinical or radiographic progression, not for pilocytic astrocytoma | Grade is the most important prognostic factor. Five-year survival exceeds 70 % |
Hemangioblastoma | Rare, although common in patients with VHL | Usually sensory, especially slowly worsening proprioception. Rarely, patients present with acute hemorrhage | Homogenously enhancing, hypervascular nodule with associated cyst or syrinx. Angiography shows enlarged feeding arteries, intense nodular stains, and early draining veins | Maximal safe surgical resection followed by observation or external beam radiotherapy. There is no role for radiotherapy and experience with VEGF antagonists is limited, although promising. Stereotactic radiosurgery is an option for recurrent or unresectable tumors | Local control rate is almost 100 % after complete resection |
MRI (with and without contrast) plays an essential role in the diagnosis of primary spinal cord tumors. Currently, no other imaging modality can be utilized alone to establish a diagnosis. Plain X-rays may show scalloping in rare cases of long-standing intramedullary tumors, and CT myelography may show spinal cord enlargement in cases of intramedullary tumors. However, the internal structure of the spinal cord and tumor as well as the tumor/cord interface cannot be visualized with those modalities.
The presence of mass effect in the form of spinal cord segmental enlargement, cyst formation, contrast enhancement, and peritumoral edema favors the diagnosis of a neoplastic process. Tumor mimicking diseases are multiple sclerosis, transverse myelitis, spinal cord ischemia, cavernous malformation, sarcoid, and CNS angiitis [1] and should be considered as part of the differential diagnosis. Acute demyelination, for example, in multiple sclerosis and transverse myelitis, may be associated with spinal cord edema and segmental enlargement resembling tumor. Additional studies such as brain MRI, CSF analysis, and angiography should be performed in suspected cases to establish the correct diagnosis. In rare cases when diagnosis cannot be established on the basis of clinico-radiological data, patient observation with follow-up imaging in 2–3 months is a reasonable option. The absence of lesion progression, diminished enhancement, and edema on interval MRI favor nonneoplastic process.
Microsurgery is the cornerstone of spinal cord tumor treatment. It has been shown that tumor type and grade are the most important factors affecting outcome (Table 18.3). Surgery provides tissue sampling and consequently, a pathological diagnosis with corresponding prognosis as well as cytoreduction. In the majority of well-defined (circumscribed) low-grade tumors, resective surgery can be curative. In instances of infiltrative tumors, maximal safe resection or biopsy contributes to management by providing diagnosis and defining further treatment. In addition to standard contrast MRI, diffusion tension tractography is a useful tool that can show the passage of fibers around or though the tumor and therefore can be used as part of the preoperative planning [8]. Functional MRI (fMRI), widely used for preoperative planning in eloquent brain areas, has not been utilized for spinal cord tumors, though application for other pathologies such as traumatic injury or multiple sclerosis has yielded encouraging results [4].
Table 18.3
Spinal cord tumors: 5-year survival
Astrocytoma | |
Pilocytic | >90 % |
Low grade | >70 % |
High grade | 30 % |
Ependymoma | |
Low grade | 85 % |
Anaplastic | 30 % |
Extent of resection has a positive correlation with patient outcome. However, this strategy is counterbalanced with the risk of neurologic injury from aggressive surgery. Most postsurgery deficits are transient and improve with time and rehabilitation. McCormick proposed a grading system of spinal cord tumors and showed that the patient’s functional status and limited longitudinal extent of the tumor are the most favorable preoperative functional outcome factors. Delayed postoperative neurological deterioration may occur due to tumor growth (most frequent) or from operative complications such as spinal cord tethering to the dura, spinal instability, and resulting kyphotic deformity. Spinal instability may be present pre- or postoperatively and results from neurologic deficits causing an axial deformity, post-laminectomy kyphosis, radiation injury, syringomyelia, or combination of these factors. Some studies showed an advantage of laminoplasty over laminectomy for prevention of future deformity at least in the pediatric population. This effect seems to be less prevalent in the adult group, though laminoplasty facilitates surgical exposure in recurrent cases. Yasargil et al. reported his series with intraspinal AVMs and tumors approached via hemilaminectomy [10]. Though technically challenging, this approach ultimately eliminates the risk of surgery-induced instability, especially in junctional regions of the spine. Postoperative spinal cord tethering to durotomy site is not infrequent and may be the cause of significant neuropathic pain and deficit. Midline myelotomy, pial suturing after tumor resection, may decrease the likelihood of tethering and should be performed whenever feasible. Reoperation with allo- or autografts usually fails to treat the condition since the granulation tissue inevitably grows into the graft and spinal cord itself. Artificial inert materials, such as Goretex, are an excellent alternative, and in our experience prophylactic grafting at the time of first surgery reduces the risk of tethering (Fig. 18.2).
Radiotherapy is an important adjuvant therapy for the treatment of spinal tumors. Radiotherapy is primarily administered for high-grade gliomas and for recurrent or residual tumors with confirmed progression when surgical resection is deemed not possible. Radiotherapy-associated side effects are characterized as acute, early delayed, and late delayed. Acute reactions usually reflect secondary inflammatory and transient effects on nearby tissues (in-field effects) especially skin and gastrointestinal. Early delayed side effects most often manifest as transient demyelination and commonly posterior column dysfunction (i.e., Lhermitte’s sign) that can be seen on T2-weighted images. Delayed late injuries include secondary malignancies particularly in the pediatric population and patients with genetic tumor predisposition disorders.
18.2 Ependymoma
Ependymoma is the most common intradural intramedullary tumor type in adults. Myxopapillary ependymoma (WHO grade 1) is a distinct type, usually located in the lumbar cistern, and is considered as the intradural extramedullary type. Cellular ependymomas arise from the ependymal lining of the central canal and are classified as WHO grade II tumors. Anaplastic ependymomas are rare and considered as WHO grade III. The most common presentation is pain, followed by neurological deficit. Rare cases of acute neurological compromise due to tumor hemorrhage have been reported.
Histologically, ependymoma cells are characterized by round to oval nuclei containing finely dispersed chromatin with perivascular and ependymal rosettes. The association between neurofibromatosis type 2 (NF-2) and spinal ependymoma is well known. This is an autosomal dominant disease caused by mutation of merlin or schwannomin gene on chromosome 22, which is a member of the protein 4.1 family. Several studies have shown that spontaneous ependymomas also have a high rate of loss of heterozygosity (LOH) on chromosome 22 and/or merlin gene mutation [2]. Furthermore, spinal ependymomas have higher rate of merlin mutation than their cranial counterparts. Therefore, spinal cellular ependymomas (WHO grade II) are considered a distinct type of tumor mostly caused by merlin/schwannomin gene alterations. Nevertheless, the exact pathophysiological mechanism linking the gene function loss and neoplastic transformation is yet to be discovered.