Striking advances in our knowledge of the history and symptoms of compression of the cord have been and are still being made. The whole subject to date has been put in most clear and readable form in Elsberg's 1) book, based on one hundred cases which he personally studied and operated on. The localization of tumors of the spinal cord is easier than that of tumors of the brain, because a growth cannot attain any considerable size in the spinal canal without compressing the cord and giving definite motor, sensory and reflex signs. A careful history and repeated neurological examinations will accurately localize the great majority. Many patients have intensification of symptoms and signs after a spinal puncture, manifested by increased paralysis, or by a higher and more distinct level of sensory disturbance. The examination, therefore, should always he repeated after withdrawal of spinal fluid.
The greatest difficulty comes in attempting to differentiate very early compression from degenerative changes in the cord which simulate tumor. Dynamic and chemical studies of the spinal fluid (Queckenstedt's and Aycr's tests), are of great assistance in the detection of early partial blocks in the spinal subarachnoid spaces. A new method of diagnosis and localization was introduced in 1921 by Sicard, who showed that lipiodol introduced through a needle at the cisterna magna would be arrested at the upper margin of the tumor, where it could be demonstrated by the roentgen ray. This method unquestionably gives a perfect visual localization in the presence of a block ; but in such cases the localization can almost always be made without its use. It is irritative, and it is not absorbed in less than three years. But, more important, the lipiodol is not always arrested by a tumor. Babinski 2) and Guillain 3) have reported cases in which, after negative lipiodol tests, operations have disclosed tumors. Moreover, lipiodol sometimes shows a false arrest which has led to negative explorations. De Martel 4) has cited four such cases, operated on by him, in two of which the lipiodol localization was made by Sicard. Though the method is still subjudice, it has not lived up to the hopes which were aroused that it would, like cerebral pneumogratns in tumors of the brain, localize the few tumors of the spinal cord whose level could not be determined by other methods 5).
Astrocytomas are the most common intramedullary spinal cord tumor in pediatric and adolescent patients and the incidence decreases with age. There are very few cases of spinal pilocytic astrocytomas (World Health Organization grade 1) reported after the fourth decade 16).
They can be classified according to many ways:
Adult spinal cord tumor.
Cervical spinal cord tumor
Thoracic spinal cord tumor
glial neoplasms : 90 - 95% of all intramedullary tumours
Spinal cord ependymoma : 60% of all glial spinal cord tumours
Spinal cord astrocytoma : 33% of all glial spinal cord tumours
Spinal cord ganglioglioma : 1% of all glial spinal cord tumours
highly vascular lesions
other rare lesions :
primary lymphoma of the spinal cord
solitary fibrous tumour
intramedullary benign masses
spinal canal epidermoid cyst
Intramedullary spinal neoplasms are more common in patients with neurofibromatosis:
ependymomas occur more often in patients with NF2
astrocytomas occur more often in patients with NF1.
Approximately 70% of intramedullary tumours are associated with cysts.
Two types of cysts are recognised :
tumoural (or intratumoural) cysts
contained within the tumour itself
typically demonstrate peripheral enhancement
may result from necrosis, fluid secretion, or degeneration of the neoplasm
need to be resected along with the solid portion of the tumour because there is a high likelihood of neoplastic cells within the cyst wall
occurs in association with the following proportion of tumours:
spinal ganglioglioma : in 46%
spinal ependymoma : in 22%
spinal astrocytoma : in 21%
spinal haemangioblastoma : in 2 - 4%
non-tumoural (or reactive) cysts
occur rostral or caudal to the solid portion of the tumour
occur due to dilatation of the central canal
do not enhance
present in 60% of all intramedullary spinal tumours may resolve once the neoplasm is resected
Syringomyelia occurs in approximately 50% of all intramedullary tumours but is most frequently associated with spinal cord hemangioblastomas.
The clinical presentation of primary spinal cord tumors is determined in part by the location of the tumor, and in nearly all clinical instances pain is the predominant presenting symptom. Motor disturbance is the next most common symptom, followed by sensory loss.
The clinical signs and symptoms of these tumors are due to mass effect and neurological dysfunction 17).
The lack of any specific clinical characteristic often precludes early diagnosis of the lesion and results in most patients being diagnosed with advanced neurological impairments such as paralysis and bladder incontinence 18).
van der Hoeven et al. from Hagaziekenhuis, The Hague, and Leiden The Netherlands, present the case of a woman who developed severe nightly thoracic pain during pregnancy without neurological deficits upon examination. Spontaneously after childbirth, the pain was markedly reduced. Further investigation showed that her pain was caused by an intramedullary ependymoma in the cervicothoracic spinal cord. Gross total resection was accomplished, and the patient has been free of pain ever since. With this case, the authors want to draw attention to a rare, but possibly very disabling, cause of increasing nightly thoracic pain during pregnancy. Spontaneous improvement after childbirth could erroneously cause postponement of further investigation 19).
Diagnosis of a primary spinal cord tumor requires a high index of suspicion based on clinical signs and symptoms, in addition to spine-directed magnetic resonance imaging 20).
MRI is the gold standard for diagnosis and assessment of intramedullary tumors. Nevertheless, sometimes MRI may not accurately differentiate between different types of intramedullary tumors, in particular if they are associated with syringes or intra- and peritumoral cysts. This could subsequently affect surgical strategies. Intraoperative ultrasound (ioUS) has become in the last few years a very useful tool for use during neurosurgical procedures. Various ioUS modalities such as B-mode and Doppler have been applied during neurosurgical procedures. On the other hand, the use of contrast-enhanced ultrasound (CEUS) is not yet well defined and standardized in this field. We report a case of a young patient harboring a cervicothoracic intramedullary tumor, for which the preoperative neuroradiological diagnosis was in favor of a diffuse astrocytoma with nodular components whereas ioUS demonstrated 3 distinct intramedullary nodules. CEUS showed highly vascularized lesions, compatible with hemangioblastomas. These findings, particularly those obtained with CEUS, allowed better definition of the lesions for diagnosis, enhanced understanding of the physiopathological aspects, and permitted the localization of all 3 nodules, thus limiting spinal cord manipulation and allowing complete resection of the lesions, with an uneventful postoperative neurological course. This is the first report of the use of intraoperative CEUS in a case of intramedullary hemangioblastoma 21).
Seropositive Neuromyelitis Optica imitating an Intramedullary Cervical Spinal Cord Tumor 22)
Tumor histology is the most important predictor of neurological outcome after surgical resection because it predicts resectability and recurrence 23).
Although resection did not significantly improve QOL, it is likely necessary to arrest QOL deterioration. Patients with better preoperative neurological status or ependymoma experienced QOL improvement, while postoperative complications negatively impacted long-term QOL 24).
The prognosis for pediatric IMSCTs is favorable with sustained functional improvement expected in a significant proportion of patients on long-term follow-up. Long-term survival at 10 years (75%) and 20 years (64%) is associated with aggressive resection. Gross-total resection was also associated with improved 5-year progression-free survival (86%). Hence, the treatment benefits of GTR are sustained on extended follow-up 25).
In a small cohort of children who had undergone surgery for IMSCTs with a mean follow-up of 4.2 years, quality of life scores according to the PedsQL 4.0 instrument were not different from those in a normal sample population 26).
The objective of this study was to identify clinically relevant predictors of progression-free survival and functional outcomes in patients who underwent surgery for intramedullary spinal cord tumors (ISCTs). METHODS An institutional spinal tumor registry and billing records were reviewed to identify adult patients who underwent resection of ISCTs between 1993 and 2014. Extensive data were collected from patient charts and operative notes, including demographic information, extent of resection, tumor pathology, and functional and oncological outcomes. Survival analysis was used to determine important predictors of progression-free survival. Logistic regression analysis was used to evaluate the association between an “optimal” functional outcome on the Frankel or McCormick scale at 1-year follow-up and various clinical and surgical characteristics. RESULTS The consecutive case series consisted of 63 patients (50.79% female) who underwent resection of ISCTs. The mean age of patients was 41.92 ± 14.36 years (range 17.60-75.40 years). Complete microsurgical resection, defined as no evidence of tumor on initial postoperative imaging, was achieved in 34 cases (54.84%) of the 62 patients for whom this information was available. On univariate analysis, the most significant predictor of progression-free survival was tumor histology (p = 0.0027). Patients with Grade I/II astrocytomas were more likely to have tumor progression than patients with WHO Grade II ependymomas (HR 8.03, 95% CI 2.07-31.11, p = 0.0026) and myxopapillary ependymomas (HR 8.01, 95% CI 1.44-44.34, p = 0.017). Furthermore, patients who underwent radical or subtotal resection were more likely to have tumor progression than those who underwent complete resection (HR 3.46, 95% CI 1.23-9.73, p = 0.018). Multivariate analysis revealed that tumor pathology was the only significant predictor of tumor progression. On univariate analysis, the most significant predictors of an “optimal” outcome on the Frankel scale were age (OR 0.94, 95% CI 0.89-0.98, p = 0.0062), preoperative Frankel grade (OR 4.84, 95% CI 1.33-17.63, p = 0.017), McCormick scale (OR 0.22, 95% CI 0.084-0.57, p = 0.0018), and region of spinal cord (cervical vs conus: OR 0.067, 95% CI 0.012-0.38, p = 0.0023; and thoracic vs conus: OR 0.015: 95% CI 0.001-0.20, p = 0.0013). Age, tumor pathology, and region were also important predictors of 1-year McCormick scores. CONCLUSIONS Extent of tumor resection and histopathology are significant predictors of progression-free survival following resection of ISCTs. Important predictors of functional outcomes include tumor histology, region of spinal cord in which the tumor is present, age, and preoperative functional status 27).
The consecutive case series consisted of 63 patients (50.79% female) who underwent resection. The mean age of patients was 41.92 ± 14.36 years (range 17.60-75.40 years). Complete microsurgical resection, defined as no evidence of tumor on initial postoperative imaging, was achieved in 34 cases (54.84%) of the 62 patients for whom this information was available. On univariate analysis, the most significant predictor of progression-free survival was tumor histology (p = 0.0027). Patients with Grade I/II astrocytomas were more likely to have tumor progression than patients with WHO Grade II ependymomas (HR 8.03, 95% CI 2.07-31.11, p = 0.0026) and spinal myxopapillary ependymomas (HR 8.01, 95% CI 1.44-44.34, p = 0.017). Furthermore, patients who underwent radical or subtotal resection were more likely to have tumor progression than those who underwent complete resection (HR 3.46, 95% CI 1.23-9.73, p = 0.018). Multivariate analysis revealed that tumor pathology was the only significant predictor of tumor progression. On univariate analysis, the most significant predictors of an “optimal” outcome on the Frankel scale were age (OR 0.94, 95% CI 0.89-0.98, p = 0.0062), preoperative Frankel grade (OR 4.84, 95% CI 1.33-17.63, p = 0.017), McCormick score (OR 0.22, 95% CI 0.084-0.57, p = 0.0018), and region of spinal cord (cervical vs conus: OR 0.067, 95% CI 0.012-0.38, p = 0.0023; and thoracic vs conus: OR 0.015: 95% CI 0.001-0.20, p = 0.0013). Age, tumor pathology, and region were also important predictors of 1-year McCormick scores. CONCLUSIONS Extent of tumor resection and histopathology are significant predictors of progression-free survival following resection of ISCTs. Important predictors of functional outcomes include tumor histology, region of spinal cord in which the tumor is present, age, and preoperative functional status 28).
Case records of 37 patients with low-grade intramedullary spinal cord tumors (IMSCTs) were identified, with a mean follow-up duration of 12.3 ± 1.4 years (range 0.5-37.2 years). Low grade astrocytomas were the most prevalent histological subtype (n = 22, 59%). Gross total resection (GTR) was achieved in 38% of patients (n = 14). Fusion surgery was required in 62% of patients with pre- or postoperative deformity (10 of 16). On presentation, functional improvement was observed in 87% and 46% of patients in McCormick scale I and II, respectively, and in 100%, 100%, and 75% in Grades III, IV, and V, respectively. Kaplan-Meier PFS rates were 63% at 5 years, 57% at 10 years, and 44% at 20 years. OS rates were 92% at 5 years, 80% at 10 years, and 65% at 20 years. On multivariate analysis, shunt placement (hazard ratio [HR] 0.33, p = 0.01) correlated with disease progression. There was a trend toward improved 5-year PFS in patients who received adjuvant chemotherapy and radiation therapy (RT; 55%) compared with those who did not (36%). Patients who underwent subtotal resection (STR) were most likely to undergo adjuvant therapy (HR 7.86, p = 0.02).
This extended follow-up duration in patients with low-grade IMSCTs beyond the first decade indicates favorable long-term OS up to 65% at 20 years. GTR improved PFS and was well tolerated with sustained functional improvement in the majority of patients. Adjuvant chemotherapy and RT improved PFS in patients who underwent STR. These results emphasize the role of resection as the primary treatment approach, with adjuvant therapy reserved for patients at risk for disease progression and those with residual tumor burden 29).
A retrospective review of 102 consecutive patients with intramedullary spinal cord tumors treated at a single institution between January 1998 and March 2009.
Ependymomas were the most common tumors with 55 (53.9%), followed by 21 astrocytomas (20.6%), 12 hemangioblastomas (11.8%), and 14 miscellaneous tumors (13.7%). Gross total resection was achieved in 50 ependymomas (90.9%), 3 astrocytomas (14.3%), 11 hemangioblastomas (91.7%), and 12 miscellaneous tumors (85.7%). At a mean follow-up of 41.8 months (range, 1-132 months), they observed recurrences in 4 ependymoma cases (7.3%), 10 astrocytoma cases (47.6%), 1 miscellaneous tumor case (7.1%), and no recurrence in hemangioblastoma cases. When analyzed by tumor location, there was no difference in neurological outcomes (P = .66). At the time of their last follow-up visit, 11 patients (20%) with an ependymoma improved, 38 (69%) remained the same, and 6 (10.9%) worsened. In patients with an astrocytoma, 1 (4.8%) improved, 10 (47.6%) remained the same, and 10 (47.6%) worsened. One patient (8.3%) with a hemangioblastoma improved and 11 (91.7%) remained the same. No patient with a hemangioblastoma worsened. In the miscellaneous tumor group, 2 (14.3%) improved, 10 (71.4%) remained the same, and 2 (14.3%) worsened. Preoperative neurological status (P = .02), tumor histology (P = .005), and extent of resection (P < .0001) were all predictive of functional neurological outcomes.
Tumor histology is the most important predictor of neurological outcome after surgical resection because it predicts resectability and recurrence 30).
53 patients (23 women and 30 men; mean age 46.3 years) were included who had undergone microsurgical resection for intramedullary spinal tumors. Schebesch et al., reviewed the patient records for tumor size, edema, intratumoral hemorrhage, consistency, midline detection, resection method, extent of resection, histopathology, and recurrence. Outcome was measured by the Karnofsky Score (KPI), the McCormick score (MCS), and the Medical Research Council Neurological Performance Score (MRC-NPS).
The most frequent diagnosis was ependymoma (37.7%), lymphoma (13.2%) and astrocytoma (11.3%). The majority of tumors were located in the thoracic spine (62.2%). Gross total resection was achieved in 73.6% and most successful in patients with ependymal histology (p<0.01). Tumor recurrence - observed in 11.3% - was significantly associated with age >65 years, astrocytic histology, higher tumor grades, and higher Ki-67 labeling. At follow-up, MCS and MRC-NPS showed significantly better results than prior to resection (p<0.001), and pain and sensory deficits had improved in 67.9% and 64.2% of patients, respectively. Microsurgical resection improved the neurological status significantly. Pain and sensory deficits showed higher improvement rates than paresis and vegetative dysfunction 31).
A total of 70 adult cases consisting of ependymoma (39), astrocytoma (11), carcinoma metastasis (8), haemangioblastoma (5), cavernoma (3) and others (4) were reviewed. Mean age was 46.8 years (range, 18-79 years), and mean follow-up was 4.5 years (range, 1-195 months). The proportion of localisation in descending order was thoracic (36%), cervical (33%), cervicothoracic (19%) and conus region (13%), with 45 gross total resections, 22 partial resections and three biopsies. Surgery-related morbidity with worsening postoperative symptoms occurred immediately in 13 patients (18.6%). The preoperative McCormick grade correlated significantly with the early postoperative grade and the grade at follow-up (χ2-test; p=0.001). None of the patients with preserved intraoperative evoked potentials exhibited significant postoperative deterioration. The degree of resection was correlated with progression-free survival (Duncan test; p=0.05). Most patients with malignant tumours, namely anaplastic ependymoma (3), astrocytoma (2) or metastatic lesions (5), underwent postoperative radiation therapy. Six patients (one anaplastic ependymoma, two anaplastic astrocytomas and three metastatic lesions) received postoperative chemotherapy.
IMSCTs should be operated on when symptoms are mild. They recommend evoked potential-guided microsurgical total resection of ependymomas and other benign lesions; partial resection or biopsy followed by adjuvant therapy should be confined to patients with high-grade astrocytomas, whereas resection or biopsy with adjuvant therapy is the best option for metastatic lesions 32).
A total of 55 patients (30 male and 25 female. The mean duration of follow-up (± SEM) was 11.4 ± 1.3 years (median 9.3 years, range 0.2-37.2 years). Astrocytomas were the most common tumor subtype (29 tumors [53%]). Gross-total resection (GTR) was achieved in 21 (38%) of the 55 patients. At the most recent follow-up, 30 patients (55%) showed neurological improvement, 17 (31%) showed neurological decline, and 8 (15%) remained neurologically stable. Patients presenting with McCormick scale I were more likely to show functional improvement by final follow-up (p = 0.01) than patients who presented with Grades II-V. Kaplan-Meier actuarial tumor progression-free survival rates at 5, 10, and 20 years were 61%, 54%, and 44%, respectively; the overall survival rates were 85% at 5 years, 74% at 10 years, and 64% at 20 years. On multivariate analysis, GTR (p = 0.04) and tumor histological grade (p = 0.02) were predictive of long-term survival; GTR was also associated with improved 5-year progression-free survival (p = 0.01).
The prognosis for pediatric IMSCTs is favorable with sustained functional improvement expected in a significant proportion of patients on long-term follow-up. Long-term survival at 10 years (75%) and 20 years (64%) is associated with aggressive resection. Gross-total resection was also associated with improved 5-year progression-free survival (86%). Hence, the treatment benefits of GTR are sustained on extended follow-up 33).
In a small cohort of children who had undergone surgery for IMSCTs with a mean follow-up of 4.2 years, quality of life scores according to the PedsQL 4.0 instrument were not different from those in a normal sample population 34).
From December 1972 to June 2003, 202 patients underwent removal of intramedullary tumors. Lesions were located in the cervical spinal cord in 61 patients (30%), at a dorsal site in 60 (29%), at a cervicodorsal site in 51 (25%), and in the medullary cone in 30 (15%). The most frequent histological tumor types were astrocytomas (86 patients, 42%) and ependymomas (68 patients, 34%).
Of the 68 ependymomas, 55 (81%) were completely removed and 13 (19%) incompletely removed. In 66% of the patients (42 patients), the presenting signs and symptoms remained unchanged at long-term follow-up; in 25% (16 patients), they improved; and in 9% (6 patients), the clinical status worsened. Of the 27 Grade I astrocytomas, 22 (81%) were completely removed and 5 (19%) incompletely removed. Functional assessment of the 23 patients available at “late” follow-up showed that 26% (6 of 23 patients) had improved, 9% (2 of 23 patients) had worsened, and 66% (15 of 23 patients) remained unchanged from preoperative status. Conversely, of the 41 Grade II astrocytomas, only 5 (12%) were completely removed, and 10% had improved. None of the 18 Grade III to IV astrocytomas could be completely removed. In 61% (11 of 18 patients), the postoperative functional status worsened.
Determinant predictors of a good outcome after surgery for intramedullary spinal cord tumors are histological type of lesion, complete removal of the lesion, and a satisfactory neurological status before surgery 35).