Radical excision of intramedullary spinal cord tumors: surgical morbidity and long-term follow-up evaluation in 164 children and young adults

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.

1995 ◽  
Vol 82 (4) ◽  
pp. 536-547 ◽  
Author(s):  
Ian F. Pollack ◽  
Diana Claassen ◽  
Qasim Al-Shboul ◽  
Janine E. Janosky ◽  
Melvin Deutsch

✓ Low-grade gliomas constitute the largest group of cerebral hemispheric tumors in the pediatric population. Although complete tumor resection is generally the goal in the management of these lesions, this can prove difficult to achieve because tumor margins may blend into the surrounding brain. This raises several important questions on the long-term behavior of the residual tumor and the role of adjuvant therapy in the management of these lesions. To examine these issues, the authors reviewed their experience in 71 children with low-grade cerebral hemispheric gliomas who were treated at their institution between 1956 and 1991 and assessed the relationship between clinical, radiographic, pathological, and treatment-related factors and outcome. Only seven patients in the series died, one from perioperative complications, five from progressive disease, and one (a child with neurofibromatosis) from a second neoplasm. For the 70 patients who survived the perioperative period, overall actuarial survivals at 5, 10, and 20 years were 95%, 93%, and 85%, respectively; progression-free status was maintained in 88%, 79%, and 76%, respectively. On univariate analysis, the factor that was most strongly associated with both overall and progression-free survival was the extent of tumor resection (p = 0.013 and p = 0.015, respectively). A relationship between extent of resection and progression-free survival was present both in patients with pilocytic astrocytomas (p = 0.041) and those with nonpilocytic tumors (p = 0.037). Histopathological diagnosis was also associated with overall survival on univariate analysis; poorer results were seen in the patients with nonpilocytic astrocytoma compared to those with other low-grade gliomas, such as pilocytic astrocytoma, mixed glioma, and oligodendroglioma (p = 0.021). The use of radiotherapy was not associated with a significant improvement in overall survival (p = 0.6). All three patients who ultimately developed histologically confirmed anaplastic changes in the vicinity of the original tumor had received prior radiotherapy, 20, 46, and 137 months, respectively, before the detection of malignant progression. In addition, children who received radiotherapy had a significantly higher incidence of late cognitive and endocrine dysfunction than the nonirradiated patients (p < 0.01 and 0.05, respectively). The authors conclude that children with low-grade gliomas of the cerebral hemispheres have an excellent overall prognosis. Complete tumor resection provides the best opportunity for long-term progression-free survival. However, even with incomplete tumor excision, long-term progression-free survival is common. The findings in this study do not support the routine use of postoperative radiotherapy after an initial incomplete tumor resection: although irradiation appears to increase the likelihood of long-term progression-free survival, overall survival is not improved significantly, and long-term morbidity may be increased.


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.


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.


2003 ◽  
Vol 99 (5) ◽  
pp. 854-862 ◽  
Author(s):  
Tom B. Johannesen ◽  
Frøydis Langmark ◽  
Knut Lote

Object. The goal of this study was to document and compare long-term survival during the periods 1970 through 1981 and 1982 through 1993 in all adult patients in Norway with histologically verified supratentorial low-grade gliomas (LGGs). Methods. Nine hundred ninety-three patients 15 to 69 years of age were found to have a primary supratentorial diffuse astrocytoma, oligodendroglioma, oligoastrocytoma, or pilocytic astrocytoma. Survival time was analyzed in all patients and, in a subset of 451 patients, the influence of new imaging methods on the time from symptom onset to imaging diagnosis was estimated. Overall median survival was 6.4 years (95% confidence interval [CI] 5.7–7.1 years). Survival times for patients in whom a diagnosis was made between 1970 and 1981 (397 patients) and between 1982 and 1993 (596 patients) were 4.1 years (95% CI 3.3–4.9 years) and 9.2 years (95% CI 7.9–10.6 years), respectively (p < 0.0001). Survival also improved in the later period within each histological subgroup. In patients in whom a biopsy was performed the median length of survival was 6.4 years (95% CI 3.1–9.7 years); in patients treated with subtotal tumor resection it was 6.8 years (95% CI 5.8–7.7 years); and in those treated with gross-total tumor resection it was 7.6 years (95% CI 5.5–9.7 years), a nonsignificant difference (p = 0.59). A considerable age-dependent variation in overall survival was demonstrated. The availability of computerized tomography (CT) scanning and/or magnetic resonance (MR) imaging as a diagnostic tool reduced the median period of symptoms prior to diagnosis by 6 months. Conclusions. Long-term overall survival significantly improved, but age-related differences in prognosis persisted. The increased sensitivity of the diagnostic method due to the availability of CT scanning and/or MR imaging may partly, but not entirely, account for the observed magnitude of improvement in overall survival. Thus local tumor treatment improved during the study period.


1995 ◽  
Vol 83 (3) ◽  
pp. 480-485 ◽  
Author(s):  
Giuseppe Cinalli ◽  
Christian Sainte-Rose ◽  
Arielle Lellouch-Tubiana ◽  
Guy Sebag ◽  
Dominique Renier ◽  
...  

✓ Over the past 15 years, eight children affected by intramedullary low-grade gliomas associated with hydrocephalus were treated at l'Hôpital des Enfants Malades. In all cases the diagnosis of hydrocephalus was made prior to that of the spinal tumor. Neuroradiological examination of all patients revealed contrast enhancement of the intracranial subarachnoid spaces. In six cases this was progressive, suggesting subarachnoid spread of the tumor, which was confirmed in two cases by histological examination. The authors analyzed 38 cases of intramedullary low-grade glioma associated with hydrocephalus that were reported in the literature. Fifteen of the cases had intracranial leptomeningeal seeding. Several hypotheses have been proposed to explain this unusual association, such as 1) increase in cerebrospinal fluid (CSF) viscosity because of elevated fluid protein content; 2) obliteration of the cisterna magna due to a rostral extension of the tumor; and 3) blockage of the spinal subarachnoid pathways of CSF resorption. Two other theories seem of particular interest. Bamford and Labadie suggested that the abnormal presence of fibrinogen in the CSF and its transformation into fibrin at the level of the basal cisterns and Pacchioni's granulation may alter CSF hydrodynamics. This mechanism alone is sufficient to induce hydrocephalus of the communicating type. In addition, as suggested by Maurice-Williams and Lucey, the resulting leptomeningeal fibrosis might predispose secondary implantation of neoplastic elements in the subarachnoid spaces of the intracranial compartment.


2005 ◽  
Vol 2 (3) ◽  
pp. 289-297 ◽  
Author(s):  
Markus Wenger ◽  
Nicola Sapio ◽  
Thomas-Marc Markwalder

Object. The authors assessed the late outcome of patients with Meyerding Grade I and II isthmic spondylolisthesis (IS) who underwent posterior instrumentation and posterolateral fusion (PLF). Decompression and posterior internal fixation with PLF is the classic surgical treatment for painful low-grade IS. Nevertheless, outcome data are scarce and of limited value mainly because they represent small numbers of patients, short follow-up periods, or both. Methods. The authors obtained data in the cases of 132 consecutive adult patients (mean age 40.6 years, range 15.2–69.9 years) with IS who underwent treatment between 1984 and 2003. Assessment involved analysis of responses to mailed questionnaires, clinical charts and, in cases in which unsatisfactory results were suspected, results of clinical reevaluations. Spondylolisthesis was present at L3–4 in three patients, L4–5 in 14, L3–4 in one, L3–5 in one, L5—S1 in 113, and S1–2 in one. Signs and symptoms included back and leg pain (65.3%), leg pain alone (26.3%), back pain alone (8.4%), and neurological dysfunction (18%). At a mean follow-up duration of 9.9 years (range 0.5–19.4 years), favorable results were reported for back and leg pain in 91.7 and 87.1% of patients, respectively. The mean visual analog scale scores were 2.13 for back and 1.59 for leg pain. Eighty-four patients resumed full- or part-time work, and 56.8% were capable of performing housework more easily. In 45.5% of the patients analgesic medications were not required, and 43.9% required them sporadically. The majority (63.6%) of patients reported they would undergo surgery again and recommended it to others. Thirteen (9.9%) suffered adjacent-segment morbidity, and in seven (5.3%) pseudarthrosis was documented. There were two deep and one superficial infections (2.3%). Conclusions. Posterior instrumentation and PLF, with possible neurodecompression, yielded favorable long-term results in this retrospective study of 132 patients with low-grade IS.


1995 ◽  
Vol 83 (4) ◽  
pp. 583-589 ◽  
Author(s):  
Leslie N. Sutton ◽  
Patricia T. Molloy ◽  
Heidi Sernyak ◽  
Joel Goldwein ◽  
Peter L. Phillips ◽  
...  

✓ The feasibility of radical surgery for astrocytomas of the optic chiasm/hypothalamus has been reported by several groups. Such surgery carries significant risks, however, including permanent damage to the pituitary gland, optic apparatus, hypothalamic structures, and carotid arteries. The benefits of radical surgery, both in terms of efficacy and toxicity, should, therefore, be evaluated against standard therapy, as is usually done for new chemotherapeutic protocols. To this end, a retrospective review was performed of 33 patients treated at Children's Hospital of Philadelphia between 1976 and 1991 who met criteria that would have made them eligible for radical surgery in many centers today, but were treated with either no surgery or conservative surgery (< 50% resection) or biopsy followed by adjuvant therapy with local radiation therapy (29 patients) and/or chemotherapy with actinomycin-D and vincristine (18 patients). The review encompassed all children with a globular enhancing mass of at least 2 cm in the hypothalamic/chiasmatic region, no evidence of optic nerve involvement or involvement of the optic radiations by computerized tomography or magnetic resonance imaging, and follow up of at least 3 years. All but one patient had tissue confirmation of a low-grade or pilocytic astrocytoma. Thirteen of the patients were 2 years of age or younger at diagnosis. Five individuals died: three of tumor progression, one of acute shunt malfunction, and one of intercurrent infection. The remaining 28 were alive at last follow up, a mean of 10.9 years from diagnosis. Twenty-three surviving patients have functional vision in at least one eye, 12 require no endocrine replacement, and 16 are in or have completed schooling with regular academic requirements. If radical surgery is to become standard care for children with low-grade astrocytomas of the hypothalamic/chiasmatic region, long-term survival and functional outcome will have to equal or surpass those of historical controls who were treated conservatively.


1998 ◽  
Vol 4 (4) ◽  
pp. E10 ◽  
Author(s):  
Claudia Martin ◽  
Eben Alexander ◽  
Terry Wong ◽  
Richard Schwartz ◽  
Ferenc Jolesz ◽  
...  

Radical resection of low-grade gliomas can decrease the incidence of recurrence, the time to tumor progression, and the incidence of malignant transformation. The authors present a series of 25 patients who underwent craniotomy and resection of low-grade tumor in an intraoperative magnetic resonance (MR) imager. This is an open configuration 0.5-tesla imager developed by The Brigham and Women's Hospital and General Electric, in which a patient can be placed to undergo surgery. Gross-total removal was accomplished under real-time image guidance. These intraoperative images allow definitive localization and targeting of the lesions and accommodate anatomical changes that may occur during surgery. The authors consistently found that the extent of abnormality seen on the intraoperatively obtained films of resection was larger than that apparent in the surgical field of view alone. Intraoperative imaging made accurate surgical identification of these abnormal areas and subsequent resection possible. Patients with tumors adjacent to or within motor or language cortex underwent resection while awake, with monitoring of neurological function. In these cases, an aggressive resection without increased neurological morbidity was accomplished using the image guidance in conjunction with serial testing. A 1-month postoperative MR image was obtained in all patients. These correlated with the final intraoperative images obtained after the resection was completed. Only one patient had a mild postoperative deficit that remained at the 1-month follow-up examination. As the long-term outcome in patients with low-grade gliomas has been shown to correspond to the degree of resection, surgical resection in which intraoperative MR imaging guidance is used can be an invaluable modality in the treatment of these tumors.


1995 ◽  
Vol 82 (3) ◽  
pp. 418-429 ◽  
Author(s):  
Friedrich W. Kreth ◽  
Michael Faist ◽  
Peter C. Warnke ◽  
Reinhard Roβner ◽  
Benedikt Volk ◽  
...  

✓ The treatment of patients with low-grade gliomas remains a subject of controversy, especially with respect to new treatment modalities such as interstitial radiosurgery (brachytherapy), radiosurgery, and stereotactic radiotherapy. In a retrospective analysis conducted between 1979 and 1991, the authors studied the results of interstitial radiosurgery in 455 patients with low-grade gliomas (World Health Organization (WHO) Grade I + WHO Grade II) with regard to survival time, quality of life, the risk of malignant transformation, and the risk profile of the treatment concept. Interstitial radiosurgery with iodine-125 was performed using permanent (1979–1985) or temporary implants (after 1985) with low-dose rates (≤ 10 cGy/hr) and a reference dose of 60 to 100 Gy calculated to the outer rim of the tumor. The 5- and 10-year survival rates in patients with pilocytic astrocytomas (97 patients) were 84.9% and 83%, and in patients with WHO Grade II astrocytomas (250 patients) 61% and 51%, respectively. Five-year survival rates for patients with oligoastrocytomas (60 patients), oligodendrogliomas (27 patients), and gemistocytic astrocytomas (21 patients) were 49%, 50%, and 32%, respectively. In the group with WHO Grade II gliomas, young age and a good performance status were associated with a better prognosis. Unfavorable factors were midline shift, enhancement on computerized tomography (CT) scan, and tumor recurrence after previous radiotherapy or surgery. Tumor location had no influence on the prognosis (247 patients in this series had deep-seated tumors). Malignant transformation was the major cause of death. Important risk factors for malignancy were the patient's age, tumor enhancement in CT scan, and tumor recurrence after previous surgery or radiotherapy. Perioperative mortality was 0.9% and perioperative morbidity was 1.7%. Radiogenic complications were observed in 2.7% of all patients, most often in larger tumors and after using permanent implants. The authors conclude that interstitial radiosurgery represents a specific treatment modality for selected patients with unifocal circumscribed low-grade gliomas with a diameter of less than 4 cm in any location. The efficacy of this treatment lies in the same range as the best results after surgery and radiotherapy.


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).


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