Foreign body reaction to hemostatic materials mimicking recurrent brain tumor. Report of three cases.

Chemical agents routinely used in neurosurgery to achieve intraoperative hemostasis can cause a foreign body reaction, which appears on magnetic resonance (MR) images to be indistinguishable from recurrent tumor. Clinical and/or imaging evidence of progression of disease early after surgical resection or during aggressive treatment may actually be distinct features of granuloma in these circumstances. A series of three cases was retrospectively analyzed for clinical, imaging, surgical, and pathological findings, and the consequences they held for further disease management. All patients were boys (3, 3, and 6 years of age, respectively) and all harbored primitive neuroectodermal tumors. Two tumors were located in the posterior fossa and one was located in the right parietal lobe. Two boys exhibited clinical symptoms, which were unexpected under the circumstances and prompted new imaging studies. One patient was asymptomatic and imaging was performed at planned routine time intervals. The MR images revealed circumscribed, streaky enhancement in the resection cavity that was suggestive of recurrent disease. This occurred 2 to 7 months after the first surgery. At repeated surgery, the resected material had the macroscopic appearance of gelatin sponge in one case and firm scar tissue in the other cases. Histological analysis revealed foreign body granulomas in the resected material, with Gelfoam or Surgicel as the underlying cause. No recurrent tumor was found and the second surgery resulted in imaging-confirmed complete resection in all three patients. Because recurrent disease was absent, the patients continued to participate in their original treatment protocols. All patients remain free from disease 34, 32, and 19 months after the first operation, respectively. During or after treatment for a central nervous system neoplasm, if unexpected clinical or imaging evidence of recurrence is found, a second-look operation may be necessary to determine the true nature of the findings. If the resection yields recurrent tumor, additional appropriate oncological treatment is warranted, but if a foreign body reaction is found, potentially harmful therapy can be withheld or postponed.

Surgical management of isolated hemangioblastomas of the spinal cord.

OBJECTIVE: Intramedullary hemangioblastomas are rare tumors, accounting for just 3% of all intraspinal neoplasms. The purpose of this study is to define the occurrence of isolated intramedullary hemangioblastomas and to analyze the role of the radiological studies and surgery for these lesions. METHODS: The charts of 19 consecutive patients operated on for isolated spinal intramedullary hemangioblastoma were reviewed. Preoperatively, all patients underwent magnetic resonance imaging and nine underwent spinal angiography. For all patients, the surgical approach was via posterior laminectomy. RESULTS: Our study sample comprised 6 women and 13 men, with an average age of 31.5 years (range, 16-75 yr). The mean prodrome was 20.8 months. Pain was the most common complaint. In all cases, the neoplasms were associated with a syrinx or edema. Gross total resection was achieved in all patients. At last follow-up examination (mean, 50.1 mo), 13 patients (68%) had improved and 6 patients (32%) had stabilized as compared with their preoperative clinical status. CONCLUSION: Isolated intramedullary hemangioblastomas typically have an indolent clinical course. These tumors have characteristic imaging properties on magnetic resonance imaging and angiography. Surgical removal of these lesions results in excellent long-term functional outcome.

Intraspinal clear cell meningioma: diagnosis and management: report of two cases.

OBJECTIVE AND IMPORTANCE: Intraspinal clear cell meningioma is a rare morphological variant of meningioma. Only 13 case reports are found in the literature; therefore, no management strategy has been defined for this tumor type. This article describes two patients, reviews the literature, and proposes a treatment plan for clear cell meningioma. CLINICAL PRESENTATION: Two female patients, 22 months and 8 years of age, respectively, presented with localized neck and leg pain that limited their ability to walk. Magnetic resonance imaging revealed intradural tumors, a cervical intramedullary neoplasm in the younger patient, and a cauda equina tumor in the older child. INTERVENTION: Both patients underwent radical resection of their intradural tumor. Both tumors, however, recurred shortly (5 and 6 mo) after the initial operation. During the second operation, a radical removal was performed on each patient. Both patients received adjuvant radiotherapy. In addition, the younger patient developed posterior fossa metastasis 20 months after intraspinal surgery. CONCLUSION: Intraspinal clear cell meningiomas are very uncommon tumors. The clinical course in our two patients supports the reported 40% recurrence rate within 15 months. These tumors also can disseminate within the central nervous system. We recommend serial imaging studies every 3 months. For recurrent tumors, we recommend localized radiation therapy after reoperation.

Treatment of Chiari I malformation in patients with and without syringomyelia: a consecutive series of 66 cases.

OBJECT: The authors describe the results of performing a standard posterior craniovertebral decompression and placement, if indicated, of a syringosubarachnoid shunt for the treatment of patients with Chiari I malformation with and without syringomyelia. METHODS: This is a retrospectively analyzed consecutive series of 66 patients (mean patient age 15 years, range 1-53 years). The uniform posterior craniovertebral decompression consisted of a small suboccipital craniectomy, a C-1 laminectomy, microsurgical reduction of the cerebellar tonsils, and dural closure with a synthetic dural graft to increase the cerebrospinal fluid space at the craniocervical junction. The presence of a large syrinx, with significant thinning of the spinal cord tissue and obliteration of the spinal subarachnoid space, particularly when combined with syrinx-related symptoms, was an indication for the placement of a syringosubarachnoid shunt. In 32 patients Chiari I malformation alone was present, and 34 in patients it was present in combination with syringomyelia. Clinical findings included pain, neurological deficits, and spinal deformity. The presence of syringomyelia was significantly associated with the presence of scoliosis (odds ratio 74.4 [95% confidence interval 8.894-622.4]). All patients underwent a posterior craniovertebral decompression procedure. In 22 of the 34 patients with syringomyelia a syringosubarachnoid shunt was also placed. The mean follow-up period was 24 months (range 3-95 months). Excellent outcome was achieved in 54 patients (82%) and good outcome in 12 (18%). In no patient were symptoms unchanged or worse at follow-up examination, including four patients who initially required a second operation for persistent syringomyelia. Pain was more likely to resolve than sensory and motor deficits after decompressive surgery. Radiological examination revealed normalization of tonsillar position in all patients. The syrinx had disappeared in 15 cases, was decreased in size in 17, and remained unchanged in two. CONCLUSIONS: Posterior craniovertebral decompression and selective placement of a syringosubarachnoid shunt in patients with Chiari I malformation and syringomyelia is an effective and safe treatment. Primary placement of a shunt in the presence of a sufficiently large syrinx appears to be beneficial. The question of if and when to place a shunt, however, requires further, preferably prospective, investigation.

Infrasellar craniopharyngiomas: case report and review of the literature.

Although craniopharyngiomas account for a large percentage of pediatric intracranial tumors, there is a bimodal age distribution. Most of these neoplasms are suprasellar or sellar in location. In this report we describe an unusual case of an infrasellar craniopharyngioma in a child. Only four previous cases of infrasellar craniopharyngiomas with no sellar involvement have been described. Infrasellar craniopharyngiomas are part of the continuum representing intracranial craniopharyngiomas and ameloblastomas of the jaw. A transnasal endoscopic biopsy was performed with a preliminary diagnosis of craniopharyngioma. The patient then underwent a radical resection of the infrasellar tumor via a subfrontal transbasal approach. This case illustrates a rare and unusual location for a craniopharyngioma. Craniopharyngiomas should be considered in the differential diagnosis of infrasellar neoplasms. Infrasellar craniopharyngiomas compromise part of the spectrum of tumors originating from enamel-forming neural crest cells.

Prognostic value of motor evoked potentials elicited by multipulse magnetic stimulation in a surgically induced transitory lesion of the supplementary motor area: a case report.

Surgery involving the supplementary motor area (SMA) places the patient at risk of transient motor deficit. To predict outcome in patients with early postoperative hypokinesis would be relevant to both the patient and the surgical team. A 15 year old girl with a large left thalamic tumour removed through a left transcallosal approach is described. Despite intraoperatively preserved muscle motor evoked potentials (mMEPs) from all limbs, elicited by multipulse electrical stimulation, she awoke with a right hemiplegia and mutism. On the first postoperative day, neurophysiological evaluation using a multipulse magnetic stimulation technique, with a train of four magnetic stimuli, confirmed the presence of mMEPs from the hemiplegic right limbs. Slight spontaneous motor activity of the right limbs and initial speech were seen later on the same day with dramatic improvement over subsequent days. It is concluded that multiple rather than single magnetic stimulation techniques may be needed to elicit mMEPs for an early postoperative differential diagnosis of SMA damage versus injury to the primary motor cortex or the corticospinal tract.

Primitive neuroectodermal tumors of the brainstem: investigation of seven cases.

OBJECTIVE: We discuss the clinical aspects, pathology, and molecular genetics of 7 patients with primitive neuroectodermal tumors (PNETs) arising in the brainstem that were treated at our institution from 1986 through 1995. Most neuro-oncologists avoid performing biopsies in children with pontine tumors. This article raises the question as to whether biopsies should be performed, because treatment recommendations might differ if a PNET was diagnosed rather than a pontine glioma. PATIENTS AND METHODS: We reviewed the clinical neuro-oncology database and the files of the Division of Neuropathology at New York University Medical Center from 1986 through 1995 and identified 7 histologically confirmed PNETs arising in the brainstem among 146 pediatric brainstem tumors. The clinical, neuroradiological, and neuropathological data were reviewed. Postmortem examinations were performed in 2 cases. Formalin-fixed, paraffin-embedded tumor tissues were also available in 6 of 7 patients that were tested for p53 gene mutations using single-strand conformation polymorphism analysis. We also tested 9 cerebellar PNETs, 9 brainstem gliomas, and 3 normal brains for p53 gene mutations as controls. RESULTS: All 7 patients presented with focal cranial nerve deficits, and 2 were also hemiparetic. The median age at diagnosis was 2.7 (1-8 years). Magnetic resonance imaging (MRI) characteristics included a focal intrinsic exophytic nonenhancing brainstem lesion that had low T1-weighted and high T2-weighted signals. Hydrocephalus was present in 5 patients at diagnosis, 3 of whom had leptomeningeal dissemination. Meningeal dissemination occurred later in the course of the disease in 3 other patients. Five children required shunts at diagnosis and another 2 at recurrence. Despite therapy, all 7 PNET patients died within 17 months of diagnosis with a mean survival of 8 (4-17) months. No mutation in the p53 gene was detected. CONCLUSIONS: Brainstem PNETs tend to arise at a younger age than brainstem gliomas and medulloblastomas. The MRI pattern suggests a localized rather than a diffuse intrinsic nonenhancing brainstem tumor. Like other PNETs, brainstem PNETs have a high predilection to disseminate within the central nervous system. The absence of p53 mutations is similar to other PNETs. Despite their origin close to the cerebellum, brainstem PNETs exhibit a more aggressive behavior and result in worse clinical outcomes than do cerebellar PNETs.

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

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.

Sympathetic storms in a child with a midbrain glioma: a variant of diencephalic seizures.

The authors report the unusual case of a 7-year-old child, one of the youngest reported to date, who developed repeated episodes of sympathetic hyperactivity after surgical resection of a midbrain glioma. These paroxysmal events were similar to previously described diencephalic seizures. However, there was no evidence of epileptogenic activity on electroencephalography, and radiologic imaging did not reveal hydrocephalus or intraparenchymal hemorrhage. In this report, clinical features are described of this patient, along with the novel use of clonidine--a sympathetic blocking agent--in his treatment, published reports are reviewed on diencephalic seizures, and steps are recommended in the treatment of a patient who presents in this manner. The authors believe that diencephalic seizures can present with a spectrum of autonomic features, and treatment should be tailored with the appropriate pharmacologic blockade.

Nonneoplastic intramedullary spinal cord lesions mimicking tumors.

OBJECTIVE: We report a group of nine patients with atypical, nonneoplastic intramedullary spinal cord lesions. By retrospectively reviewing these patients, we hoped to elucidate characteristics that would identify these patients as harboring nonneoplastic lesions before surgical intervention. METHODS: We reviewed the histological findings of 212 patients undergoing surgery for intramedullary spinal cord tumors between 1989 and 1994. We identified nine patients with nonneoplastic lesions (4%); case histories and radiographs were reviewed. RESULTS: All patients were evaluated preoperatively using magnetic resonance imaging. The extent of enhancement with gadolinium varied from homogeneous enhancement to no enhancement. All lesions showed marked T2 changes. There was a lack of significant spinal cord expansion associated with the lesions in all cases. All patients underwent surgery. The histology of the surgical specimens showed demyelinating lesions in four patients, sarcoidosis in two patients, amyloid angiopathy in two patients, and a mass of nonneoplastic inflammatory cells of unknown origin in one patient. CONCLUSION: Although it was difficult to antecedently distinguish these lesions from neoplastic spinal cord tumors by case history and physical examination, the most consistent clue was absent or minimal spinal cord expansion on the preoperative magnetic resonance images.

Headache and Chiari I malformation in the pediatric population.

There has been disagreement regarding surgical intervention in treating pediatric patients with Chiari I malformation with headache as sole complaint. Therefore, we retrospectively reviewed our experience over a 6-year period, with patients less than 5 years of age (mean = 34.8 months) with radiographically confirmed Chiari I malformation. We identified 7 patients who presented with headaches as their only complaint. The headaches varied in location and severity. All patients were treated with posterior fossa decompression and syringosubarachnoid shunt when indicated. At follow-up, all patients were noted to have rapid clinical improvement (mean = 11.6 weeks) and remain asymptomatic. Our data suggest that patients less than 5 years of age with Chiari I malformation benefit from surgical decompression when presenting with a chief complaint of headache.

Intraparenchymal clear cell meningioma of the brainstem in a 2-year-old child. Case report and literature review.

We report an unusual case of an intraparenchymal clear cell meningioma of the brainstem, occurring in a 22-month-old girl. She presented with bulbar dysfunction and a right hemiparesis due to an intrinsic tumor of the medulla, which was confirmed by radiologic imaging to be focal and with an exophytic component. At surgery, a partial resection was achieved and no dural attachment was found. Pathologic examination revealed a clear cell meningioma. In reviewing the literature, there have been fewer than 20 reported cases of clear cell meningioma, none of which were intraparenchymal, involved the brainstem or occurred in such a young patient. The pathologic findings, treatment options and present understanding of this tumor are discussed.

MR of spinal cord ganglioglioma.

PURPOSE: Our purpose was to describe the MR imaging features in a series of spinal intramedullary gangliogliomas and to compare these findings with the MR features of intramedullary astrocytomas and ependymomas. METHODS: A retrospective analysis was performed of 76 MR examinations in 27 patients with histologically proved spinal ganglioglioma; these were then compared with imaging findings in a representative sample of histologically proved spinal cord astrocytomas and ependymomas. RESULTS: Statistically significant observations regarding spinal gangliogliomas included young age of the patients (mean, 12 years), long tumor length, presence of tumoral cyst, presence of bone erosion and scoliosis, absence of edema, presence of mixed signal intensity on T1-weighted images, and presence of patchy enhancement and cord surface enhancement. A trend (not statistically significant) was noted for holocord involvement and lack of magnetic susceptibility. CONCLUSION: Spinal ganglioglioma can be strongly suspected if MR images reflect the above criteria; however, the ultimate diagnosis still depends on radical resection and appropriate histopathologic investigation.

Intramedullary spinal cord ependymomas in children: treatment, results and follow-up.

A retrospective review of 20 pediatric patients with intramedullary spinal cord ependymomas, all of whom underwent operative resection between 1985 and 1996, was undertaken to determine surgical results, long-term follow-up and tumor recurrence. Twelve children operated on in the same period with filum or cauda equina ependymomas were not included in this study. Nine children had had previous treatment before referral. Gross total resection was achieved in 14 patients and subtotal in 6. None of these had a post-operative radiation therapy. The median follow-up period was 67 months (range 25-177 months). All children were clinically evaluated before and after operation and at the last follow-up. The clinical grade at the last follow-up showed improvement in 8 patients (40%), was unchanged in 10 (50%) and deteriorated in 2 (10%). Three patients had a recurrence, 2 at the primary site (2 and 3 years after our surgery) and 1 at a distant site (3 years after). The actuarial 5- and 10-year survival rates were both 90%; 5- and 10-year progression-free survival rates were 93 and 70%, respectively. We conclude that a complete removal can be achieved in almost all cases of intramedullary spinal cord ependymomas in children, and that the long survival rates justify avoiding post-operative radiation therapy.

Motor-evoked potential monitoring for intramedullary spinal cord tumor surgery: correlation of clinical and neurophysiological data in a series of 100 consecutive procedures.

Resection of intramedullary spinal cord tumors carries a high risk for surgical damage to the motor pathways. This surgery is therefore optimal for testing the performance of intraoperative motor evoked potential (MEP) monitoring. This report attempts to provide evidence for the accurate representation of patients' pre- and postoperative motor status by combined epidural and muscle MEP monitoring during intramedullary surgery. The authors used transcranial electrical motor cortex stimulation to elicit MEPs, which were recorded from the spinal cord (with an epidural electrode) and from limb target muscles (thenar, anterior tibial) with needle electrodes. The amplitude of the epidural MEPs and the presence or absence of muscle MEPs were the parameters for MEP interpretation. A retrospective analysis was performed on data from the resection of 100 consecutive intramedullary tumors and MEP data were compared with the pre- and postoperative motor status. Intraoperative monitoring was feasible in all patients without severe preoperative motor deficits. Preoperatively paraplegic patients had no recordable MEPs. The sensitivity of muscle MEPs to detect postoperative motor deficits was 100% and its specificity was 91%. There was no instance in which a patient with stable MEPs developed a motor deficit postoperatively. Intraoperative MEPs adequately represented the motor status of patients undergoing surgery for intramedullary tumors. Because deterioration of the motor status was transient in all cases, it can be considered that impairment of the functional integrity of the motor pathways was detected before permanent deficits occurred.

The role of motor evoked potentials during surgery for intramedullary spinal cord tumors.

OBJECTIVE: This is a prospective study of the methodology and clinical applications of motor evoked potentials (MEPs) during surgery for intramedullary spinal cord tumors. METHODS: Transcranial electrical stimulation was used to activate corticospinal motoneurons, and the traveling waves of the spinal cord were recorded through catheter-electrodes placed epi- or subdurally. Intraoperative MEP monitoring was performed in 32 consecutive patients (age range, 1-50 yr) undergoing resection of intramedullary spinal cord tumors. In 19 patients, MEPs were present before myelotomy (monitorable group), and in 10 patients, MEPs were absent before myelotomy (unmonitorable group). Placement of an epidural electrode was not possible in two patients, and technical problems prevented recording in one. RESULTS: MEP amplitudes decreased intraoperatively by more than 50% of baseline in three patients, all of whom had postoperative paraplegia. Two of these patients recovered within 1 week after surgery, and one remained paraplegic. None of the patients with preserved MEP amplitude (> 50%) sustained immediate significant postoperative deterioration. Motor function was significantly deteriorated 1 week after surgery in one patient in the monitorable group and in five patients in the unmonitorable group. MEP monitorability was significantly associated with good surgical outcome for adult patients (P < 0.05), although not for pediatric patients (P > 0.6). Preoperative motor status and surgical outcome were not significantly associated for the adult (P = 0.13) or pediatric groups (P > 0.4). CONCLUSION: MEP monitorability was a better predictor of functional outcome than the patient's preoperative motor status for the adult group. Significant predictors of MEP monitorability in the adult group were preoperative motor function (P < 0.01), history of no previous treatment (surgery or irradiation) (P < 0.01), and small tumor size (P < 0.05). Weak associations with monitorable MEPs existed for low-grade tumors (P = 0.09), the presence of baseline somatosensory evoked potentials (P = 0.10), and tumor pathological abnormalities (ependymoma) (P = 0.13). No associations were determined for sex (P > 0.4), associated syrinx (P > 0.3), or tumor location (P > 0.5). In the pediatric group, none of the examined factors were associated with MEP monitorability (P > 0.3). A decline of more than 50% in MEP amplitude during tumor removal should serve as a serious warning sign to the surgeon.

Intraspinal sarcoidosis: diagnosis and management.

OBJECTIVE: Isolated intramedullary spinal cord or cauda equina involvement by sarcoidosis is quite rare. We report three patients with intraspinal sarcoidosis and absent systemic manifestations of the disease. The clinical presentation, operative management, electrophysiologic studies, pathology, laboratory investigations, and current therapy are discussed with attention to the previous literature. METHODS: Two of the three patients had a preoperative diagnosis of a cervical intramedullary spinal cord tumor. The third patient had the preoperative diagnosis of an infectious process involving the cauda equina. Magnetic resonance imaging (MRI) with gadolinium did not suggest an inflammatory process. Intraoperative somatosensory evoked potential performed in two patients exhibited normal amplitudes, but a prolonged latency in seven out of eight extremities; with normal central conduction time suggesting a peripheral or radicular involvement. All three patients underwent laminectomy and biopsy of the intraspinal pathology. RESULTS: Pathologic examination demonstrated sarcoidosis in all three patients. Intraoperative observations, intramedullary nodules, and thickening of the meninges were inconsistent with neoplasm and limited the surgical procedure to a biopsy. Frozen sections performed at two of the operations revealed an inflammatory process that confirmed the intraoperative observations. Postoperatively, the diagnostic work-up for all patients was negative for systemic manifestations. CONCLUSIONS: Isolated intraspinal sarcoidosis is a rare process. The current management for intramedullary spinal cord or cauda equina sarcoidosis is prolonged corticosteroids. The surgeon should not attempt complete resection if this granulomatous process is suspected.

Intra-axial tumors of the cervicomedullary junction: surgical results and long-term outcome.

Until recently, intra-axial brainstem tumors were traditionally regarded as surgically inaccessible lesions with a uniformly poor prognosis. However, increasing data indicate that distinct subgroups of brainstem tumors exist that are amenable to surgical intervention. To address this question, we reviewed our experience in the operative management of 39 consecutive patients, in the magnetic resonance imaging (MRI) era, with intra-axial cervicomedullary tumors, in order to determine those factors associated with long-term outcome. Thirty-nine patients (26 male, 13 female) underwent surgery by a single surgeon (F.J.E.) between 1985 and 1994. Mean age of diagnosis was 14 years (range 3 months - 60 years); mean duration of preoperative symptoms was 24 weeks (range 1-168). Twenty patients presented with lower cranial nerve dysfunction and 19 presented with motor and/or sensory dysfunction. All patients were graded according to the McCormick Scale, pre- and postoperatively, and at the time of follow-up. All patients were evaluated with MRI scanning. Twenty-three patients had either previous biopsy or subtotal resection, 13 previous radiation therapy, and 6 previous chemotherapy. The mean time to follow-up was 48 months (range 7-138). Twelve patients underwent gross total resection, 7 near total resection (>90%), 15 subtotal resection (50-90%), and 5 partial resection (< 50%). Histologically, there were 15 low-grade fibrillary astrocytomas, 9 ependymomas, 7 gangliogliomas, 3 anaplastic astrocytomas, 3 juvenile pilocytic astrocytomas, and 2 mixed gliomas. Although the vast majority of tumors were low grade histologically, a higher proportion of the patients with high-grade lesions experienced tumor progression when compared to low-grade tumors (75 vs. 30%). Overall, the 5-year progression-free and total survivals were 60 and 89%, respectively. There was 1 death within the first postoperative month. Preoperative duration of symptoms greater than 15 weeks was associated with a longer progression-free survival. There was a trend for preoperative neurologic grade to predict functional neurologic outcome at follow-up. In summary, intra-axial tumors of the cervicomedullary junction are a distinct subset of brainstem tumors, predominantly of low-grade histology, with favorable long-term progression-free and total survivals following surgical resection. A long duration of preoperative symptoms may indicate an indolent clinical course and a more favorable prognosis. Our data also indicate that early surgical intervention is warranted prior to neurologic deterioration.