Chordomas of the craniocervical junction: follow-up review and prognostic factors

2001 ◽  
Vol 95 (6) ◽  
pp. 933-943 ◽  
Author(s):  
Benedicto O. Colli ◽  
Ossama Al-Mefty

Object. Chordomas are rare tumors that arise from the remnants of the notochord. Because of their deep location, local infiltrative nature, and involvement of surrounding bone, treatment of chordomas is a challenge. In this study the authors analyze the data and prognostic factors obtained during the follow-up period (range 1–150 months, median 38 months) in 53 patients with craniocervical junction chordomas and 10 patients with chondrosarcomas. Methods. Several surgical approaches were used, and some tumor excisions required staged procedures. Survival was calculated according the Kaplan—Meier method. Statistical analysis was performed using Fisher exact, log rank and Kruskal—Wallis tests. Radical/subtotal resection was achieved in 77.8% of the patients. The mortality rate during the follow-up period was 14.3%. In patients harboring chondrosarcoma better 5-year recurrence-free survival (RFS) rates were demonstrated than in those with chordoma (100% and 50.7%, respectively). Histological patterns (typical or chondroid chordomas) and patient age at onset of symptoms had no effect on the RFS rates. Radical/subtotal resections were associated with better RFS rates than partial resection. Adjuvant proton-beam therapy was shown to increase the RFS rates compared with conventional radiotherapy (90.9% and 19.4%, respectively, at 4 years posttreatment). Karyotypically abnormal tumors were associated with the worst RFS rates compared with karyotypically normal lesions (44.5% and 90.3%, respectively, at 3 years). Cases of cranial nerve palsy followed by those with cerebrospinal fluid leakages were the most frequent postoperative complications. Permanent postoperative neurological deficit was observed in 28.6% of the patients. Conclusions. A better prognosis was observed in patients with chondrosarcoma compared with those harboring chordoma. Histological pattern and patient age at symptom onset were not factors that influenced prognosis in cases of chordoma. Extensive resection and possibly adjuvant proton-beam therapy provided better prognoses for these patients.

2001 ◽  
Vol 10 (3) ◽  
pp. 1-11 ◽  
Author(s):  
Benedicto O. Colli ◽  
Ossama Al-Mefty

Object Chordomas are rare tumors that arise from the remnants of the notochord. Because of their deep location, local infiltrative nature, and involvement of surrounding bone, treatment of chordoma is a challenge. The authors analyze the data and prognostic factors obtained during the follow-up period (range 1–150 months, median 38 months) in 53 patients with craniocervical junction chordoma and 10 patients with chondrosarcoma. Methods Several surgical approaches were used, and some tumor excisions required staged procedures. Survival was calculated according the Kaplan–Meier method. Statistical analysis was performed using the Fisher exact and Kruskal–Wallis tests. Radical/subtotal resection was achieved in 77.8% of the patients. The mortality rate during the follow-up period was 14.3%. In patients harboring chondrosarcoma better 5-year recurrence-free survival (RFS) rates were demonstrated than in those with chordoma (100% and 50.7%, respectively). Histological patterns (typical or chondroid chordoma) and patient age at onset of symptoms had no effect on the RFS rates. Radical/subtotal resections were associated with better RFS rates than partial resection. Adjuvant proton-beam radiotherapy was shown to increase the RFS rates compared with conventional radiotherapy (90.9% and 19.4%, respectively at 4 years posttreatment). Karyotypically abnormal tumors were associated with worst RFS rates as compared with karyotypically normal lesions (44.5% and 90.3%, respectively at 3 years). Cases of cranial nerve palsy, followed by CSF leakages were the most frequent postoperative complication. Permanent postoperative neurological deficit was observed in 28.6% of the patients. Conclusions A better prognosis was observed in patients with chondrosarcoma compared with those harboring chordoma. Histological pattern and the patient's age at symptom onset were not factors that influenced prognosis in cases of chordoma. Extensive resection and possibly adjuvant proton-beam radiotherapy provided better prognosis for these patients.


2003 ◽  
Vol 99 (2) ◽  
pp. 254-263 ◽  
Author(s):  
Fred G. Barker ◽  
William E. Butler ◽  
Sue Lyons ◽  
Ethan Cascio ◽  
Christopher S. Ogilvy ◽  
...  

Object. The use of radiosurgery for the treatment of cerebral arteriovenous malformations (AVMs) and other lesions demands an accurate understanding of the risk of radiation-related complications. Some commonly used formulas for predicting risk are based on extrapolation from small numbers of animal experiments, pilot human treatment series, and theoretical radiobiological considerations. The authors studied the incidence of complications after AVM radiosurgery in relation to dose, volume, and other factors in a large patient series. Methods. A retrospective review was conducted in 1329 patients with AVM treated by Dr. Raymond Kjellberg at the Harvard Cyclotron Laboratory (HCL) between 1965 and 1993. Dose and volume were obtained from HCL records, and information about patient follow up was derived from concurrent clinical records, questionnaires, and contact with referring physicians. Multivariate logistic regression with bootstrapped confidence intervals was used. Follow up was available in 1250 patients (94%); the median follow-up duration was 6.5 years. The median radiation dose was 10.5 Gy and the median treatment volume was 33.7 cm3. Twenty-three percent of treated lesions were smaller than 10 cm3. Fifty-one permanent radiation-related deficits occurred (4.1%). Of 1043 patients treated with a dose predicted by the Kjellberg isoeffective centile curve to have a less than 1% complication risk, 1.8% suffered radiation-related complications. Actual complication rates were 4.7% for 128 patients treated at Kjellberg risk centile doses of 1 to 1.8%, and 34% for 61 patients treated at risk centile doses of 2 to 2.5%. The fitted logistic model showed that complication risk was related to treatment dose and volume, thalamic or brainstem location, and patient age. Conclusions. The Kjellberg isoeffective risk centile curve significantly underpredicted actual risks of permanent complications after proton beam radiosurgery for AVMs. Actual risks were best predicted using a model that accounted for treatment dose and volume, lesion location, and patient age.


1994 ◽  
Vol 80 (5) ◽  
pp. 877-883 ◽  
Author(s):  
Isabelle Salmon ◽  
Olivier Dewitte ◽  
Jean-Lambert Pasteels ◽  
Jacqueline Flament-Durand ◽  
Jacques Brotchi ◽  
...  

✓ High-grade astrocytic tumors constitute the most serious as well as the most common group of primary brain tumors. Although several prognostic factors have been proposed, little is known about the prognostic value of deoxyribonucleic acid (DNA) ploidy in adult astrocytic tumors. In a series of 146 adult patients, aged 16 to 82 years, the individual prognostic values of six variables were studied, namely: tumor histopathological grade, treatment, patient age, extent of tumor, ploidy level, and DNA histogram type. Cox's proportional hazard model was then applied to the data to ascertain which factors might independently determine patient survival. Univariate analyses revealed that histopathological grade, age, and DNA histogram type were very powerful prognostic factors. The statistical significance of the influence of adjuvant radiotherapy and chemotherapy was at a borderline level, and the two remaining variables (tumor extent and ploidy level) had no prognostic relevance. Multivariate analyses showed that age, histopathological grade, and DNA histogram type were independent, statistically significant prognostic factors. A prognostic score was calculated from Cox's polynomial function in which those factors were introduced. The best score corresponded to a patient aged 16 years with a hypertriploid low-grade astrocytoma, while the worst score corresponded to a patient aged 82 years with a diploid high-grade astrocytoma. The worst score:best score ratio revealed a risk 71 times higher for a bad prognosis. It is concluded that patient age, histopathological grade, and DNA histogram type are very powerful prognostic factors for adult astrocytic tumors. A prognostic score including those factors could be used to characterize astrocytic tumor aggressiveness presurgically on fine-needle aspirates, and to monitor the patient's postsurgical evolution to define the appropriate therapy.


1994 ◽  
Vol 81 (1) ◽  
pp. 31-36 ◽  
Author(s):  
Isabelle M. Germano ◽  
Nicole Poulin ◽  
André Olivier

✓ The indications for and the risks and outcome of reoperation for medically refractory temporal lobe epilepsy have not been well documented. A retrospective review is presented of 40 patients who underwent reoperation on the temporal lobe for recurrent seizures. The mean patient age at the first operation was 22 ± 7 years (± standard deviation). Electrocorticography during the first operation showed interictal epileptic abnormalities from surface electrodes in 97% of the cases and from depth electrodes in the mesiotemporal structures in 38%. The seizures recurred with the same pattern within 6 months after the first operation in 60% of patients and within 2 years in 90%. Postoperative neuroimaging studies showed residual mesiotemporal structures in all cases. The mean time between the two operations was 5.5 ± 5 years and the mean patient age at the second operation was 28 ± 8 years. The second operation involved focal resection of the mesiotemporal structures in 30 cases. The mean postoperative follow-up period was 4.8 ± 2.7 years (range 2 to 11 years). After the second operation, 63% of the patients were seizure-free or had rare seizures (one or two per year). There were no permanent neurological complications. Patients who did not benefit from reoperation had electroencephalographic abnormalities in multiple brain areas. Reoperation for temporal lobe epilepsy effectively controls seizures in the majority of patients, and the procedure is safe if rigorous technical rules are observed. More complete resection of mesiotemporal structures during the first operation, even in the absence of intraoperative electrographic abnormalities, could prevent the need for reoperation in defined cases.


2000 ◽  
Vol 92 (3) ◽  
pp. 384-389 ◽  
Author(s):  
Cynthia S. Kubu ◽  
Gloria M. Grace ◽  
Andrew G. Parrent

Object. The authors studied the neuropsychological correlates of stereotactically guided lesioning of the right and left posteroventral globus pallidus internus (GPi) in a prospective series of patients suffering from Parkinson's disease (PD).Methods. Eighteen patients with PD who underwent stereotactically guided lesioning of the GPi (left side in 10 patients and right side in eight) completed neuropsychological evaluations before and after surgery. Patients served as their own controls. Multiple two-by-two repeated-measures analyses of variance were used to assess neuropsychological changes as a function of the side in which lesioning was performed (lesioning on the left side compared with that on the right) and surgery (presurgery compared with postsurgery). Relationships between cognitive variables and patient age at disease onset, age at surgery, and disease duration were examined using a linear regression model.The most striking findings were evident from results of a phonemic word fluency test in which patients in whom a left-sided pallidotomy had been performed achieved a mean performance score that was lower than the score of patients in whom a right-sided pallidotomy had been performed; this score declined even more as a result of surgery. Change in performance on the word fluency test across pre- and postoperative assessments was also related to patient age at onset of PD in those who had undergone left-sided pallidotomy, with patients of an older age at disease onset showing the greatest decline in performance.Conclusions. These preliminary findings indicate that the side on which surgery was performed and patient age at onset of PD are important in the prediction of postoperative cognitive outcome. The findings also indicate that stereotactically guided lesioning of the GPi presents a relatively mild cognitive risk.


2003 ◽  
Vol 98 (4) ◽  
pp. 807-811 ◽  
Author(s):  
Anan Bedavanija ◽  
Jürgen Brieger ◽  
Hans-Anton Lehr ◽  
Jan Maurer ◽  
Wolf J. Mann

Object. Acoustic neuroma is the most frequent benign tumor of the cerebellopontine angle, and surgery is still the most common form of treatment. To gain better insight into the dysregulated mechanisms causing growth of acoustic neuroma, the authors studied the proliferative activity of 34 consecutive samples by analyzing immunohistochemical staining with Ki-67 and proliferating cell nuclear antigen (PCNA), and apoptosis based on the terminal deoxynucleotidyl transferase—mediated deoxyuridine triphosphate nick-end labeling. Data from these analyses were correlated with clinical parameters (that is, tumor size, duration of symptoms, and patient age). Methods. Apoptotic cells were found in none of the tumors. Proliferation measured on staining with Ki-67 and PCNA correlated with tumor size, but not with patient age or duration of symptoms. The authors demonstrated that tumors 18 mm or smaller in diameter have lower proliferation indices and growth rates, compared with tumors larger than 18 mm with high proliferative indices and growth rates. Additionally, they observed that these more aggressive, larger tumors occur mostly in patients younger than 50 years of age. Conclusions. Patients with tumors larger than 18 mm in diameter and who are younger than 50 years of age sustain an enhanced risk for fast-growing tumors because of these lesions' enhanced proliferative activity. For these patients the authors recommend active therapy.


2001 ◽  
Vol 95 (4) ◽  
pp. 651-659 ◽  
Author(s):  
Kenji Tada ◽  
Shoji Shiraishi ◽  
Takanori Kamiryo ◽  
Hideo Nakamura ◽  
Hirofumi Hirano ◽  
...  

Object. The most frequent genetic abnormality in human malignant gliomas is loss of heterozygosity (LOH) on chromosome 10. Candidate genes on chromosome 10 that are associated with the prognosis of patients with anaplastic astrocytoma (AA) and glioblastoma (GBM) were evaluated. Methods. The authors used 12 fluorescent microsatellite markers on both arms of chromosome 10 to study LOH in 108 primary astrocytic tumors. The LOH on chromosome 10 was observed in 11 (32%) of 34 AAs and 34 (56%) of 61 GBMs. No LOH was detected in 13 low-grade gliomas. Loss of heterozygosity was not detected in any AA in the seven patients younger than 35 years, but it was discovered in 41% of the patients older than 35 years. The prognostic significance of LOH at each locus was evaluated in 89 patients older than 15 years; 33 (37%) had supratentorial AAs and 56 (63%) had supratentorial GBMs. The Cox proportional hazards model, adjusted for patient age at surgery, the preoperative Karnofsky Performance Scale score, and the extent of surgical resection revealed that LOH on marker D10S209 near the FGFR2 and DMBT1 genes was significantly associated with shorter survival in patients with AA. The LOH on markers D10S215 and D10S541, which contain the PTEN/MMAC1 gene between them, was significantly associated with shorter survival in patients with GBM. Conclusions. In the present study it is found that LOH on chromosome 10 is an age-dependent event for patients with AAs and that LOH on marker D10S209 near the FGFR2 and DMBT1 loci is a significantly unfavorable prognostic factor. It is also reported that LOH on the PTEN/MMAC1 gene is a significantly unfavorable prognostic factor in patients with GBM.


1998 ◽  
Vol 89 (5) ◽  
pp. 755-761 ◽  
Author(s):  
Hiroyuki Kinouchi ◽  
Kazuo Mizoi ◽  
Akira Takahashi ◽  
Yoshihide Nagamine ◽  
Keiji Koshu ◽  
...  

Object. A retrospective analysis was conducted of 10 patients (three women and seven men) who were treated for spinal dural arteriovenous shunts (AVSs) located at the craniocervical junction. This analysis was performed to evaluate the characteristics of this unusual location in contrast with those of the more common thoracic and lumbar AVSs. Methods. Seven patients presented with subarachnoid hemorrhage (SAH) and one with slowly progressive quadriparesis and dyspnea due to myelopathy. The other two cases were detected incidentally and included a transverse—sigmoid dural AVS and a cerebellar arteriovenous malformation. Angiographic studies revealed that the spinal dural AVSs at the C-1 and/or C-2 levels were fed by the dural branches of the radicular arteries that coursed from the vertebral artery and drained into the medullary veins. Venous drainage was caudally directed in the patient with myelopathy. In contrast, the shunt flow drained mainly into the intracranial venous system in patients with SAH. Furthermore, in four of these patients a varix was found on the draining vein. In all patients, the draining vein was interrupted surgically at the point at which this vessel entered the intradural space, using intraoperative digital subtraction angiography to monitor flow. The postoperative course was uneventful in all patients and no recurrence was confirmed on follow-up angiographic studies obtained in seven patients at 6 months after discharge. Conclusions. If computerized tomography scanning shows SAH predominantly in the posterior fossa and no abnormalities are found on intracranial four-vessel angiographic study, proximal vertebral angiography should be performed to detect dural AVS at the craniocervical junction. The results of surgical intervention for this disease are quite satisfactory.


2002 ◽  
Vol 96 (1) ◽  
pp. 64-70 ◽  
Author(s):  
Bryce Weir ◽  
Lew Disney ◽  
Theodore Karrison

Object. The authors explore the risk of rupture in aneurysms categorized by size. Methods. A computerized database of 945 patients with aneurysms treated between 1967 and 1987 was retrospectively established. All available clinical and radiological studies were abstracted. Because of the recent interest in the size of intracranial aneurysms in relation to their likelihood of rupture, the database was searched with respect to this parameter. In 390 patients representing 41% of all cases, aneurysms were measured by neuroradiologists at the time of diagnosis. In 78% of the 945 patients there was only one aneurysm, and of the 507 aneurysms that were measured, 60% were solitary. Of all patients, 86% had ruptured aneurysms. The average age of all patients was 47 years, and for those with ruptured aneurysms it was 46 years. Of the ruptured aneurysms, 77% were 10 mm or smaller, compared with 85% of the unruptured aneurysms. It was found that 40.3% of the ruptured aneurysms were on the anterior cerebral artery or anterior communicating artery, compared with 13% of the unruptured aneurysms. None of the cavernous internal carotid artery (ICA) aneurysms were ruptured and 65% of the ophthalmic artery (OphA) aneurysms were. Of the unruptured aneurysms, 15% were located in the cavernous ICA or the OphA. Of the ruptured aneurysms, 29% were on the middle cerebral artery, compared with 36% of the unruptured aneurysms. The mean size of ruptured and unruptured aneurysms showed no statistically significant increase with patient age, although the difference in size between the ruptured and unruptured aneurysms decreased with increasing age. The mean size of all ruptured aneurysms (10.8 mm) was significantly larger than the mean size of all unruptured aneurysms (7.8 mm, p < 0.001); the median sizes were 10 mm and 5 mm, respectively. The size of ruptured aneurysms in patients who died in the hospital was significantly larger than those in the patients who survived (12 mm compared with 9.9 mm, p = 0.004). Symptomatic unruptured aneurysms were significantly larger than incidental unruptured aneurysms (14.6 mm compared with 6.9 mm, p = 0.032), which were, in turn, larger than aneurysms that were unruptured and part of a multiple aneurysm constellation. Both ruptured and unruptured aneurysms were larger in male than in female patients, but not significantly. Conclusions. Site and patient age, as well as lesion size, may affect the chance of rupture.


1993 ◽  
Vol 79 (6) ◽  
pp. 845-852 ◽  
Author(s):  
Alain Pierre-Kahn ◽  
Jean-François Hirsch ◽  
Mathieu Vinchon ◽  
Christine Payan ◽  
Christian Sainte-Rose ◽  
...  

A study was made of 75 children treated between 1970 and 1990, with partial, subtotal, or total removal of three intrinsic and 72 exophytic or surface brain-stem tumors. In all cases, the goal of surgery was to remove as much tumor as possible. Extent of removal was defined according to data obtained from postoperative computerized tomography or magnetic resonance imaging, and was considered partial when only a small amount of tumor was removed, subtotal when a few cubic millimeters of tumor was left, and total when no residual tumor was seen on postoperative radiological investigations. An ultrasonic aspirator was used for the 43 most recent operations. Among tumor removals without the aspirator, 24 (75%) were partial, eight (25%) subtotal, and none total; with the use of the aspirator, the number of partial removals decreased to 44.5% while that of subtotal and total removals increased to 32% and 23.5%, respectively. There were 69 gliomas (92%) and 47 benign tumors (62.6%). Forty-nine patients were irradiated postoperatively, and 14 of the 23 patients whose benign tumors were removed totally or subtotally did not undergo irradiation. This study showed that: 1) the overall prognosis of patients with malignant tumors was poor and was not improved by surgery; 2) the survival rate of those with benign tumors was significantly (p < 0.01) lower after partial removal than after total or subtotal removal (52% and 94%, respectively, at 5 years); 3) comparison of means and proportions (Student's and chi-squared tests) between benign and malignant tumors showed a significant difference relating to patient age (p < 0.03), peritumoral hypodensity (p < 0.001), and preoperative duration of symptoms (p < 0.001); 4) stepwise logistic regression analysis confirmed that two of these three variables were related to malignancy: namely, patient age at surgery (p < 0.03) and presence of peritumoral hypodensity (p < 0.001); and 5) routine postoperative irradiation was contraindicated after total or subtotal removal of benign tumors.


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