Intramedullary spinal cord tumors in children under the age of 3 years

1996 ◽  
Vol 85 (6) ◽  
pp. 1036-1043 ◽  
Author(s):  
Shlomo Constantini ◽  
John Houten ◽  
Douglas C. Miller ◽  
Diana Freed ◽  
Memet M. Ozek ◽  
...  

✓ Over a 13-year period extending from 1980 to 1993, 27 children less than 3 years of age underwent operation for removal of an intramedullary spinal cord tumor (IMSCT). The majority (18 of 27) of children had undergone surgery before being referred to New York University (NYU) Medical Center. The most common reasons for radiological investigation were pain (42%), motor regression (36%), gait abnormalities (27%), torticollis (27%), and progressive kyphoscoliosis (24%). Forty procedures were performed in 27 children. Nine children underwent two operations and two children underwent three procedures. A gross-total resection was achieved in 72% of the procedures. There was no surgical mortality. A comparison of the preoperative and 3-month postoperative functional grades for the first NYU procedure (NYU-1) yielded the following findings: 20 patients' conditions remained the same, five patients improved, and two patients deteriorated. The functional outcomes of a second operation (NYU-2) were similar. The majority of the children (24 of 27, 89%) had histologically determined low-grade lesions. There were 12 patients with low-grade astrocytomas (Grades I-III), eight with gangliogliomas, two with ganglioglioneurocytomas, one with a glioneurofibroma, and one child with a mixed astro/oligodendroglioma. Two children had anaplastic astrocytomas (Grades II–III) and one child had a glioblastoma multiforme. In a median follow-up review of 76 months, two patients died and two patients were lost to follow up. The 3- and 5-year progression-free survival (PFS) rates were 81.7% (standard error of the mean (SEM) 0.083) and 76.2% (SEM 0.094), respectively. Eight of 24 patients suffered a recurrence within a mean time of 45.4 ± 28.9 months. All were treated with surgery (NYU-2). Lesions recurred in three of 12 children with low-grade astrocytomas, two of eight children with gangliogliomas, one child with an anaplastic astrocytoma, one child with a ganglioglioneurocytoma, and one child with a glioblastoma multiforme. At follow-up review, most of these children were doing well. Sixteen are in functional Grades I or II and 18 children attend a normal school system. The authors conclude that surgery for the removal of IMSCTs in children less than 3 years of age can be performed radically and safely. The postoperative functional performance is determined by the degree of the preoperative deficit. It is, therefore, of utmost importance to diagnose and treat these children as early as possible. Spinal cord tumors should be recognized as potentially excisable lesions on their initial presentation and when they recur. The optimum treatment for malignant lesions is still to be determined.

2005 ◽  
Vol 2 (3) ◽  
pp. 249-255 ◽  
Author(s):  
Raj K. Shrivastava ◽  
Fred J. Epstein ◽  
Noel I. Perin ◽  
Kalmon D. Post ◽  
George I. Jallo

Object. Intramedullary spinal cord tumors (IMSCTs) in the older-age adult population pose complex management issues regarding the extent of resection and functional outcome, especially in terms of quality of life. Historically, IMSCTs in the older adult population were treated with irradiation alone because it was assumed that functional recovery would be poor. The authors examined their IMSCT database and report the first large series of IMSCTs in patients older than 50 years of age. Methods. In this retrospective clinical and chart review there were 30 cases meeting inclusion criteria drawn from databases at three different institutions. A modified McCormick Scale was used to assess functional levels in all 30 patients pre- and postoperatively. The mean age of patients in this cohort was 59.8 years (range 50–78 years), and the mean follow- up period was 10.6 years (range 2–16 years). Ependymoma was the most common tumor (83%), and 55% were located in the thoracic spine. The most common presenting symptom was sensory dysesthesia, with rare motor loss. The prodromal period to treatment was 19.4 months. Based on the McCormick Scale score at last follow-up examination 67% of patients were clinically functionally the same, 9% were worse, and 24% were improved after surgery. There were two deaths due tumor progression (both malignant tumors) and one recurrence (anaplastic astrocytoma). All three patients in whom malignant astrocytomas were diagnosed underwent postoperative radiation therapy. Conclusions. In the population of patients older than age 50 years, thoracic ependymomas are the most common IMSCTs that present characteristically with sensory symptoms. The longer prodromal period in the older adult population may reflect the fact that their diagnosis and workup is inadequate. There was no significant increase in the length of stay in the neurosurgical ward. The authors recommend motor evoked potential-guided aggressive microsurgical resection, because the long-term outcome of benign lesions is excellent (good functional recovery and no tumor recurrence).


1990 ◽  
Vol 72 (4) ◽  
pp. 523-532 ◽  
Author(s):  
Paul C. McCormick ◽  
Roland Torres ◽  
Kalmon D. Post ◽  
Bennett M. Stein

✓ A consecutive series of 23 patients underwent operative removal of an intramedullary spinal cord ependymoma between January, 1976, and September, 1988. Thirteen women and 10 men between the age of 19 and 70 years experienced symptoms for a mean of 34 months preceding initial diagnosis. Eight patients had undergone treatment prior to tumor recurrence and referral. Mild neurological deficits were present in 22 patients on initial examination. The location of the tumors was predominantly cervical or cervicothoracic. Radiological evaluation revealed a wide spinal cord in all cases. Magnetic resonance (MR) imaging was the single most important radiological procedure. At operation, a complete removal was achieved in all patients. No patient received postoperative radiation therapy. Histological examination revealed a benign ependymoma in all cases. The follow-up period ranged from 6 to 159 months (mean 62 months) with seven patients followed for a minimum of 10 years after surgery. Fourteen patients underwent postoperative MR imaging at intervals ranging from 8 months to 10 years postoperatively. No patient has been lost to follow-up review and there were no deaths. No patient showed definite clinical or radiological evidence of tumor recurrence during the follow-up period. Recent neurological evaluation revealed functional improvement from initial preoperative clinical status in eight patients, no significant change in 12 patients, and deterioration in three patients. The data support the belief that long-term disease-free control of intramedullary spinal ependymomas with acceptable morbidity may be achieved utilizing microsurgical removal alone.


1985 ◽  
Vol 63 (4) ◽  
pp. 492-499 ◽  
Author(s):  
Paul R. Cooper ◽  
Fred Epstein

✓ The management of patients with intramedullary spinal cord tumors is controversial. In the past, these tumors have often been treated with biopsy or subtotal removal followed by irradiation — a therapy that is usually associated with early tumor recurrence and progressive neurological impairment. In an attempt to improve on the outcome of patients with intramedullary tumors, the authors performed radical resection in most of the 29 adult patients who had surgery for these tumors within the past 30 months. The mean duration of symptoms was 9½ years, and all patients presented because of progressive neurological deficit. Patients were evaluated with metrizamide myelography-computerized tomography scanning and intraoperative ultrasound imaging to define the site of the tumor and cystic components. There were 14 ependymomas, 11 astrocytomas, two lipomas, and one case each of intramedullary fibrosis and astrogliosis. Solid tumor spanned a mean of five spinal cord segments and 16 tumors were associated with cysts. Twenty tumors were in the cervical and/or cervicothoracic regions. Total removal was achieved in 14 patients and “99% removal” in seven others. In 21 of 29 patients (72%), the neurological condition was stabilized or improved as a result of the operation. Postoperative deterioration occurred for the most part in patients who could not walk or who had minimal motor function at the time of operation, and these patients are no longer considered as operative candidates. Radical resection of intramedullary tumors can be achieved, with stabilization or improvement of neurological deficit in the majority of patients.


1993 ◽  
Vol 79 (2) ◽  
pp. 204-209 ◽  
Author(s):  
Fred J. Epstein ◽  
Jean-Pierre Farmer ◽  
Diana Freed

✓ Thirty-eight patients underwent surgery for an intramedullary spinal cord ependymoma. In 37 patients, postoperative magnetic resonance imaging confirmed that the tumor was totally removed. The morbidity of surgery was directly related to the preoperative neurological condition. Patients who were normal or nearly normal preoperatively were rarely worse after surgery, and those who had significant disability preoperatively were at greatest risk of being more impaired after surgery. There has been no tumor recurrence in any patient after a mean follow-up period of 24 months, and radiation therapy has not been employed as a surgical adjunct.


2000 ◽  
Vol 93 (2) ◽  
pp. 183-193 ◽  
Author(s):  
Shlomi Constantini ◽  
Douglas C. Miller ◽  
Jeffrey C. Allen ◽  
Lucy B. Rorke ◽  
Diana Freed ◽  
...  

Object. The majority of intramedullary spinal cord tumors (IMSCT) in children and young adults are low-grade gliomas. Radical resection of similar tumors in the cerebral hemisphere or cerebellum is usually curative; however, the conventional management for IMSCTs remains partial resection followed by radiotherapy because of the concern for surgical morbidity. Nevertheless, radical resection of IMSCTs without routine adjuvant treatment has been the rule at our institution since 1980. In an attempt to resolve this controversy, the long-term morbidity and survival in a large series of children have been retrospectively reviewed. Methods. The database records and current status of 164 patients 21 years of age and younger in whom an IMSCT was resected were reviewed. A gross-total resection (> 95%) was achieved in 76.8% of the surgical procedures. Subtotal resections (80–95%) were performed in 20.1%. The majority of patients (79.3%) had histologically low-grade lesions. There were no deaths due to surgery. When comparing the preoperative and 3-month postoperative functional grades, 60.4% stayed the same, 15.8% improved, and 23.8% deteriorated. Only 13 patients deteriorated by more than one functional grade. Patients with either no deficits or only mild deficits before surgery were rarely injured by the procedure, reinforcing the importance of early diagnosis and treatment. The major determinant of long-term patient survival was histological composition of the tumor. The 5-year progression-free survival rate was 78% for patients with low-grade gliomas and 30% for those with high-grade gliomas. Patients in whom an IMSCT was only partially resected (< 80%) fared significantly worse. Conclusions. The long-term survival and quality of life for patients with low-grade gliomas treated by radical resection alone is comparable or superior to minimal resection and radiotherapy. The optimum therapy for patients with high-grade gliomas is yet to be determined.


1971 ◽  
Vol 34 (5) ◽  
pp. 693-696 ◽  
Author(s):  
Harvey Gass ◽  
Paul A. Goodman ◽  
John Miller ◽  
Jacob L. Chason

✓ An intramedullary spinal cord tumor of unusual pathology is reported which has been called a “sclerosing astrocytoma” because of the predominance of a sclerotic collagenous nodule in its center. A successful removal was accomplished with virtually complete neurological recovery maintained now for 3 years.


1994 ◽  
Vol 81 (2) ◽  
pp. 288-293 ◽  
Author(s):  
Stephen B. Tatter ◽  
Lawrence F. Borges ◽  
David N. Louis

✓ Central neurocytoma is a neuronal neoplasm that occurs supratentorially in the lateral or third ventricles. The authors report the clinical, neuroradiological, and neuropathological features of two neurocytomas arising in the spinal cord of two men, aged 65 and 49 years. The patients presented with progressive neurological deficits referable to the cervical spinal cord. Magnetic resonance imaging revealed isodense intramedullary spinal cord tumors at the C3–4 level. Both tumors were initially misdiagnosed as gliomas. In Case 1 the correct diagnosis was made after electron microscopy revealed neuronal features. Immunostaining in Case 2 revealed that tumor cells were positive for synaptophysin and negative for glial fibrillary acidic protein, strongly indicating a neuronal tumor. It is suggested that this spinal cord neoplasm be included under the designation “central neurocytoma.”


1988 ◽  
Vol 68 (4) ◽  
pp. 550-553 ◽  
Author(s):  
Robert A. Solomon ◽  
Bennett M. Stein

✓ In a series of 60 intramedullary spinal cord tumors, there were eight cases of hemangioblastoma. Seven of these eight patients displayed evidence of diffuse spinal cord enlargement over multiple levels at a considerable range beyond the actual location of the tumor. One patient had a large intramedullary cyst that accounted for only part of the enlargement, and one had multiple tumor nodules. Six other patients had no obvious explanation for the diffuse cord enlargement. This phenomenon of extensive spinal cord enlargement appears to be unique to this type of tumor and not related to multiple tumors or extensive cyst formation. The cord enlargement seems to be related to edema and congestion of the spinal cord, perhaps secondary to the vascular shunt present in the hemangioblastomas. The possible pathophysiology and clinical importance of this observation is discussed.


2019 ◽  
Vol 30 (5) ◽  
pp. 655-663 ◽  
Author(s):  
Wei Shi ◽  
Shan Wang ◽  
Huifang Zhang ◽  
Guoqin Wang ◽  
Yi Guo ◽  
...  

OBJECTIVELaminoplasty has been used in recent years as an alternative approach to laminectomy for preventing spinal deformity after resection of intramedullary spinal cord tumors (IMSCTs). However, controversies exist with regard to its real role in maintaining postoperative spinal alignment. The purpose of this study was to examine the incidence of progressive spinal deformity in patients who underwent laminoplasty for resection of IMSCT and identify risk factors for progressive spinal deformity.METHODSData from IMSCT patients who had undergone laminoplasty at Beijing Tsinghua Changgung Hospital between January 2014 and December 2016 were retrospectively reviewed. Univariate tests and multivariate logistic regression analysis were used to assess the statistical relationship between postoperative spinal deformity and radiographic, clinical, and surgical variables.RESULTSOne hundred five patients (mean age 37.0 ± 14.5 years) met the criteria for inclusion in the study. Gross-total resection (> 95%) was obtained in 79 cases (75.2%). Twenty-seven (25.7%) of the 105 patients were found to have spinal deformity preoperatively, and 10 (9.5%) new cases of postoperative progressive deformity were detected. The mean duration of follow-up was 27.6 months (SD 14.5 months, median 26.3 months, range 6.2–40.7 months). At last follow-up, the median functional scores of the patients who did develop progressive spinal deformity were worse than those of the patients who did not (modified McCormick Scale: 3 vs 2, and p = 0.04). In the univariate analysis, age (p = 0.01), preoperative spinal deformity (p < 0.01), extent of tumor involvement (p < 0.01), extent of abnormal tumor signal (p = 0.02), and extent of laminoplasty (p < 0.01) were identified as factors associated with postoperative progressive spinal deformity. However, in subsequent multivariate logistic regression analysis, only age ≤ 25 years and preoperative spinal deformity emerged as independent risk factors (p < 0.05), increasing the odds of postoperative progressive deformity by 4.1- and 12.4-fold, respectively (p < 0.05).CONCLUSIONSProgressive spinal deformity was identified in 25.7% patients who had undergone laminoplasty for IMSCT resection and was related to decreased functional status. Younger age (≤ 25 years) and preoperative spinal deformity increased the risk of postoperative progressive spinal deformity. The risk of postoperative deformity warrants serious reconsideration of providing concurrent fusion during IMSCT resection or close follow-up after laminoplasty.


1996 ◽  
Vol 84 (6) ◽  
pp. 1060-1061 ◽  
Author(s):  
Akira Miyauchi ◽  
Katsumi Matsumoto ◽  
Eiji Kohmura ◽  
Teruo Doi ◽  
Kazuhiko Hashimoto ◽  
...  

✓ Primary central nervous system germinoma usually presents as an extraaxial intracerebral mass. The authors report the rare occurrence of an intramedullary spinal cord germinoma at the conus medullaris in a 24-year-old man, which was treated by partial removal and radiation therapy. The tumor was highly radiosensitive and the patient remains disease free 15 months posttreatment.


Sign in / Sign up

Export Citation Format

Share Document