Cervicomedullary Tumors Research Articles

5-aminolevulinic acid-guided surgery for focal pediatric brainstem gliomas: A preliminary study.

Background: There is a growing body of literature supporting the use of 5-aminolevulinic acid (5-ALA) in the pediatric population, however, its use is still considered “off label” in this setting. In this retrospective study, we report our experience using 5-ALA in pediatric patients with focal brainstem gliomas (BSGs).Methods: Patients younger than 16 years presenting with a newly diagnosed BSG that was focal in nature were considered suitable for treatment with 5-ALA-assisted surgery. Exclusion criteria included MRI features suggestive of a diffuse intrinsic pontine glioma. A single dose of 5-ALA was administered preoperatively. Intraoperative fluorescence was recorded as “solid,” “vague,” or “none.” The effectiveness of the fluorescence was graded as “helpful” or “unhelpful.”Results: Eight patients underwent 5-ALA-assisted surgery. There were four tumors located in the pons, two midbrain tumors, and two cervicomedullary tumors. Histological analysis demonstrated three diffuse astrocytomas, three pilocytic astrocytomas, and two anaplastic astrocytomas. Solid fluorescence was found in three of the eight cases, vague fluorescence was found in two cases, and no fluorescence was found in three cases. Fluorescence was useful in 3 (37%) cases. No patients experienced any complications attributable to the administration of the 5-ALA.Conclusion: With a total fluorescence rate of 62.5% but a subjectively assessed “usefulness” rate of only 37.5%, the role of 5-ALA in BSG surgery is limited. Given the toxicological safety, however, of the agent, caution is perhaps needed before dismissing the use of 5-ALA entirely.

Microsurgery for Cervicomedullary Tumor: 2-Dimensional Operative Video.

The treatment of brainstem tumors are challenging for the neurosurgeons due to the delicate nature of this region. These tumors present a bimodal incidence, with one peak occurring during childhood, where up to 18% of all tumors manifest, while the other occurs during the fourth decade of life. The classic clinical presentation for brainstem tumors is the involvement of cranial nerves. About 90% of the cases have glial etiology, allowing the radiological classification of these tumors into 4 different types: type 1, diffuse; type II, focal, limited, and slow-growing tumors; type III, exophytic tumors, mostly low-grade gliomas, and type IV, cervicomedullary tumors, which may commonly present as an exophytic lesion. We present a 52-yr-old patient with a type IV tumor. The patient reported a history of high cervical pain, which started about 2 mo after admission, and was associated with lower right limb paresthesia. Examination of the cervical and encephalic regions using magnetic resonance imaging revealed a tumor located in the posterior medulla oblongata medial line, near the fourth oblique ventricle. Resection of the lesion was performed using a median suboccipital approach, with subsequent entrance to the oblong medulla via the median posterior sulcus. The lesion was successfully resected. Following surgery, the patient maintained the initial evocated potentials and previous paresthesia in the lower right limb. Pathologic examination revealed that the tumor was a grade II ependymoma. Herein, we present a video demonstrating the microsurgical steps that were required to perform the cervicomedullary tumor resection. The patient signed the Institutional Consent Form, which allows the use of his/her images and videos for any medical publications in conferences and/or scientific articles.Watch now at https://academic.oup.com/ons/article-lookup/doi/10.1093/ons/opy024 {"href":"Single Video Player","role":"media-player-id","content-type":"play-in-place","position":"float","orientation":"portrait","label":"Microsurgery for Cervicomedullary Tumor: 2-Dimensional Operative Video","caption":"","object-id":[{"pub-id-type":"doi","id":""},{"pub-id-type":"other","content-type":"media-stream-id","id":"1_x4kowbwi"},{"pub-id-type":"other","content-type":"media-source","id":"Kaltura"}]} Disclosure The authors have no personal, financial, or institutional interest in any of the drugs, materials, or devices described in this article.

Intramedullary spinal cord tumors and hydrocephalus: an analysis of the results of surgical treatment in 541 patients

The study purpose was to explore, based on large clinical material, the occurrence of hydrocephalus combined with intramedullary tumors, possible pathogenetic mechanisms of its development, effect of tumor resection on the course of hydrocephalus, and need and timing of shunting surgery. We present and analyze the data of the largest individual series of patients of all age groups operated on for IMTs of the spinal cord: 541 patients; 586 operations; age from 2 months to 72 years. Our findings confirm a potential pathogenetic relationship between IMT and HC. The overall occurrence rate of HC in IMT patients was 6.3%. In patients with benign tumors (WHO Grade 1-2; 449 patients), HC developed in 25 (5.6%) cases; in patients with malignant tumors (WHO Grade 3-4; 84 patients), HC developed in 7 (8.3%) cases. A statistically significant prevalence of cervico-medullary tumors was found in HC patients: 19 (59.4%) cases. According to our data, dissemination of the tumor process is a potential factor of HC development.

Cervicomedullary tumors in children.

Cervicomedullary tumors (CMTs) represent a heterogeneous group of intrinsic neoplasms that are typically low grade and generally carry a good prognosis. This single-institution study was undertaken to document the outcomes and current treatment philosophy for these challenging neoplasms. The charts of all pediatric patients with CMTs who received treatment at St. Jude Children's Research Hospital between January 1988 and May 2013 were retrospectively reviewed. Demographic, surgical, clinical, radiological, pathological, and survival data were collected. Treatment-free survival and overall survival were estimated, and predictors of recurrence were analyzed. Thirty-one children (16 boys, 15 girls) with at least 12 months of follow-up data were identified. The median age at diagnosis was 6 years (range 7 months-17 years) and the median follow-up was 4.3 years. Low-grade tumors (Grade I or II) were present in 26 (84%) patients. Thirty patients underwent either a biopsy alone or resection, with the majority of patients undergoing biopsy only (n = 12, 39%) or subtotal resection (n = 14, 45%). Only 4 patients were treated solely with resection; 21 patients received radiotherapy alone or in combination with other treatments. Recurrent tumor developed in 14 children (45%) and 4 died as a result of their malignancy. A high-grade pathological type was the only independent variable that predicted recurrence. The 5- and 10-year treatment-free survival estimates are 64.7% and 45.3%, respectively. The 5- and 10-year overall survival estimate is 86.7%. Children with CMTs typically have low-grade neoplasms and consequently long-term survival, but high risk of recurrence. Therapy should be directed at achieving local tumor control while preserving and even restoring neurological function.

Clinico-radiological profile and nuances in the management of cervicomedullary junction intramedullary tumors.

Background:Cervicomedullary junction (CMJ) intramedullary tumors comprise of tumors that often pose a surgical challenge even in the present era. Though classified under brainstem glioma CMJ tumors are well amenable for surgical resection and have a good outcome. Various factors are involved in the outcome of these patients following surgery and a proper pre-operative assessment is often required to reduce the morbidity and mortality.Materials and Methods:Patients admitted in the Department of Neurosurgery with a diagnosis of CMJ intramedullary tumors from January 2001 to January 2010 were included in the study. Patients were analyzed retrospectively regarding their symptomatology, clinical findings, radiology and outcome after surgery. All patients underwent pre-operative magnetic resonance imaging (MRI) and post-operatively all were managed in the neurosurgery intensive care unit for days to weeks or as dictated by the clinical condition of the patient.Results:A total of 32 patients were included in the present study. The number of males was 21 (65.6%) and females were 11 (34.4%) respectively. The mean age of presentation was 22.97 ± 9.8 years and the mean duration of pre-operative symptoms was 13.3 ± 12.9 months. The tumor had extension from the CMJ into the cervical region in 17 (53.1%) and into the medullary region in 14 (43.8%) patients. Tumor decompression was done in 9 (28.1%) patients and gross near total excision done in 23 (71.87%) patients.Conclusions:Cervicomedullary tumors are a subset of tumors quite distinct from the usual brainstem tumors. Patients having predominant cervical involvement present early and have less post-operative deficits. Those with predominantly more medullary involvement present late, hence have a much more morbid outcome. Though closely related to vital neural structures, surgery forms the mainstay of treatment. Adequate pre-operative planning and preparation of the patient along with intense post-operative monitoring and ventilatory assistance as and when required helps in a good surgical outcome.

Brain stem tumors in children and adolescents: single institutional experience

Pediatric brain stem tumors (BsT) are a heterogeneous group of diseases. Our aim was to analyze our experience to find out prognostic factors. A retrospective study with BsT patients was performed. Imaging characteristics, extension of surgery, pathology, and adjuvant therapy were analyzed and correlated with overall survival (OS) and progression-free survival (PFS) as outcome measures. Since 1980 to 2010, we analyzed 65 BsT patients, 41 of them girls (63%), median age of 8 years (range 13.9 months to 17.6 years). Twenty-two patients (33.8%) had diffuse intrinsic pontine gliomas (DIPG) and 43 (66.2%) presented with focal BsT. Histology was available in 42 patients; the most frequent is low-grade glioma in 24/42 patients (57%). DIPG's histology (obtained usually at necropsy) confirmed five high-grade gliomas. After median follow-up of 49.3 months (0.5-175 months), 20/22 DIPG patients have died (90.9%), while 27/43 with focal tumors were alive (62.8%). Variables related to outcome were histology (better for low-grade glioma (LGG) OS p < 0.001), surgery (better if operated OS p < 0.001), and adjuvant therapy (worse if given, PFS p = 0.001, OS p = 0.024). The outcome for DIPG was dismal, median OS/EFS of 14.2/9.4 months, significantly worse than focal BsT (p = 0.000), while OS/EFS was 122.8/87.2 months for focal intrinsic, 88.2/47.1 months for exophytic, and 124.4/54 months for cervico-medullary tumors: no differences were found among them, except the histology (OS p < 0.001 for low-grade vs high-grade tumors). BsT in children comprised two different groups: diffuse (DIPG) and focal gliomas. The DIPGs continue having a dismal prognosis, needing new approaches, while focal tumors including LGG have better prognosis.

Neurofibromatosis-2 and spinal cord ependymomas: Report of two cases and review of the literature

The incidence of ependymoma in patients with neurofibromatosis-2 (NF-2) is low and information regarding treatment and prognosis is lacking. We present two cases of cervicomedullary tumors in patients with NF-2 from our institution, and we provide a review of the literature in order to summarize the known clinical information about this rare occurrence. Patient #1 had histological confirmation of ependymoma and was treated with subtotal resection followed by observation and has had no evidence of progression for 11 months. Patient #2 has been observed for 4 1/2 years without treatment for a cervicomedullary tumor, which appears to be an ependymoma by imaging. Although it has increased in size very slowly, there have been no clinical symptoms. Among the additional 21 cases of NF-2 and ependymoma from the literature, the most common location is the cervical spine (70%), and the median age at diagnosis is 15 years. Surgical resection was performed in 85% of the cases and subtotal resection in 64% of cases. Fifteen patients (75%) were reported alive at the time of the published reports, with survival ranging from 0.1 to 10 years, and the 8-year survival estimated as 51%. Survival was related to the location of the tumor. We conclude from our two cases and review of the existing literature that NF-2 associated spinal ependymomas have an indolent course and typically can be observed or treated by surgical excision alone.

Clinical, radiologic and pathologic features and outcome following surgery for cervicomedullary gliomas in children

Surgical resection is generally recommended for cervicomedullary tumors, but morbidity of resection may be significant. This study sought to identify MRI characteristics that might predict morbidity and extent of resection. A retrospective review was performed of MRI findings, histopathology, extent, and morbidity of resection in cervicomedullary gliomas undergoing resection during 1985-2008. Of 78 brainstem tumors, nine cervicomedullary tumors undergoing resection were identified: two pilocytic astrocytomas, two gangliogliomas, and five grade II astrocytomas. Mean age was 6.3 years (range 1.7-11.2 years). Initial treatment was surgery in seven: biopsy (1), <25% resection (4), and 25-50% resections (2). Bulbar worsening occurred in five of six patients with interposed areas of non-enhancement versus one of three patients without interposed non-enhancing tissue (P = 0.014). Additionally, bulbar worsening occurred in five of five patients with a poorly defined tumor/brainstem interface and abnormal low T1 signal extending beyond obvious tumor into the brainstem versus one of four with a well-defined tumor margin (P = 0.008). Following chemo- or radiotherapy, the definition of the brainstem/tumor interface improved. In four patients undergoing surgery after chemo/radiotherapy, more extensive resections were achieved without neurologic worsening: >80% in three and 30% in one. A less aggressive initial surgical approach, supplemented by postoperative chemotherapy, designed to preserve brainstem function, is proposed for patients with interposed non-enhancing tissue continuous with normal cervical cord or medulla and/or a poorly defined ventral tumor/brainstem interface with abnormal low T1 signal extending beyond obvious tumor into the brainstem.

A phase I trial of etanidazole and hyperfractionated radiotherapy in children with diffuse brainstem glioma

Purpose To determine the toxicity and maximum tolerated dose of etanidazole administered concurrently with hyperfractionated radiation therapy (HRT) for children with brainstem glioma. Methods and materials Eighteen patients with brainstem glioma were treated with etanidazole and HRT on a dose escalation protocol (Phase I trial) between 1990 and 1996. All patients had MRI confirmation of diffuse pontine glioma and signs/symptoms of cranial nerve deficit, ataxia, or long tract signs of <6 months’ duration. Cervicomedullary tumors were excluded. Patients (median age: 8.5 years; 11 males, 7 females) received HRT to the tumor volume plus a 2-cm margin with parallel-opposed 6–15-MV photons. The total dose was 66 Gy in 44 fractions (1.5 Gy b.i.d., with at least 6 h between fractions) for the first 3 patients and 63 Gy in 42 fractions for the subsequent 15 patients. Etanidazole was administered as a rapid i.v. infusion 30 min before the morning fraction of HRT. Planned doses of etanidazole were 1.8 g/m 2 × 17 doses (30.6 g/m 2) at Step 1 to a maximum of 2.4 g/m 2 × 21 doses (50.4 g/m 2) at Step 8. Dose escalation was planned with 3 patients at each of the 8 levels. Results Three patients were treated at each dose level except Level 2, on which only 1 patient was treated. The highest dose level achieved was Level 7, which delivered a total etanidazole dose of 46.2 g/m 2. Two patients were treated at this level, and both patients experienced Grade 3 toxicity in the form of a diffuse cutaneous rash. Three patients received a lower dose of 42 g/m 2 (dose Level 6) without significant toxicity, and this represents the maximum tolerated dose (MTD). There were 23 cases of Grade 1 toxicity (10 vomiting, 5 peripheral neuropathy, 2 rash, 2 constipation, 1 weight loss, 3 others), 11 cases of Grade 2 toxicity (4 vomiting, 2 skin erythema, 2 constipation, 1 arthralgia, 1 urinary retention, 1 hematologic), and 4 Grade 3 toxicities (2 rash, 1 vomiting, 1 skin desquamation). Grade 2 or 3 peripheral neuropathy was not seen at any dose level. The median survival from the start of treatment was 8.5 months (range: 3–58 months). Conclusion The MTD of etanidazole in children receiving HRT for brainstem glioma is 42 g/m 2, with cutaneous rash as the dose-limiting toxicity. This is in contrast to the adult experience, which demonstrates a 24% lower MTD of 34 g/m 2 limited by peripheral neuropathy.

A clinicopathologic reappraisal of brain stem tumor classification. Identification of pilocystic astrocytoma and fibrillary astrocytoma as distinct entities.

Brain stem tumors in children have been classified pathologically as low grade or high grade gliomas and descriptively as diffuse gliomas, intrinsic gliomas, midbrain tumors, tectal gliomas, pencil gliomas, dorsal exophytic brain stem tumors, pontine gliomas, focal medullary tumors, cervicomedullary tumors, focal gliomas, or cystic gliomas. To search for a simplified and prognostic clinicopathologic scheme for brain stem tumors, the authors reviewed a consecutive cohort of patients younger than age 21 years with tumors diagnosed from 1980 through 1997. Pathology specimens and neuroimaging were classified by masked review. Statistical and survival analysis along with Cox proportional hazards regression was performed. Seventy-six patients were identified, with initial diagnostic magnetic resonance imaging available for 51 and pathology specimens for 48 patients. Twenty cases were classified histologically as pilocytic astrocytoma (PA), 14 as fibrillary astrocytoma (FA), and 14 as other tumors or indeterminate pathology. For all tumors, characteristics significantly associated with a worse survival rate were: symptom duration less than 6 months before diagnosis (P = 0.004); abducens palsy at presentation (P < 0.0001); pontine location (P = 0.0002); and engulfment of the basilar artery (P = 0.006). Pilocytic astrocytoma was associated with location outside the ventral pons (P = 0.001) and dorsal exophytic growth (P = 0.013); Fibrillary astrocytoma was associated with symptoms less than 6 months (P = 0. 006), abducens palsy (P < 0.001), and engulfment of the basilar artery (P = 0.002). Pilocytic astrocytoma showed 5-year overall survival (OS) of 95% (standard error [SE], 5%) compared with FA 1-year OS of 23% (SE, 11%;P < 0.0001). Brain stem tumors can be succinctly and better biologically classified as diffusely infiltrative brain stem gliomas-generally FA located in the ventral pons that present with abducens palsy, often engulf the basilar artery, and carry a grim prognosis-and focal brain stem gliomas-frequently PA arising outside the ventral pons, often with dorsal exophytic growth, a long clinical prodrome, and outstanding prognosis for survival. Our findings emphasize the individuality of PA as a distinct clinicopathologic entity with an exceptional prognosis.

Cervicomedullary astrocytomas of childhood: clinical and imaging follow-up.

BACKGROUND. Cervicomedullary astrocytomas are a unique subset of brainstem tumors in children because they have a good prognosis when compared to the pontine subset of brainstem gliomas. Objective. To review the clinical and imaging findings in a series of children with cervicomedullary astrocytomas as to diagnosis and management. A retrospective review of eleven children (six females, five males, age range: 10 days-18 years; mean = 7 years) with cervicomedullary tumors was done including the clinical presentation, imaging studies (MR: eleven, CT and MR: four), surgical findings, pathological results, and follow-up clinical and imaging findings (range: 0.2-11 years; mean = 5.2 years). Symptoms and signs were delayed and protracted, often occurring over months to years (mean = 2.3 years, range 0.5-7 years). The tumors expanded the dorsal medulla and involved the upper cervical spinal cord (mean maximum tumor diameter = 4.4 cm). Only three patients had hydrocephalus. In three of four cases the tumor was not seen on CT. On MR, the majority of the tumors were T1 hypointense and T2 hyperintense. Treatment consisted of surgery only in six patients, surgery and radiation therapy in four, and surgery, chemotherapy, and radiation in one. There was recurrent local disease in four patients and on follow-up metastatic disease in the brain in one. On follow-up the majority of the patients are alive and stable (mean = 5.2 years, range 0.2-11 years). There has been one death. The majority of tumors were pilocytic astrocytomas. Cervicomedullary tumors are a unique subset of brainstem gliomas in childhood that present with a long duration of symptoms and a greater long-term survival than pontine gliomas.

Adult brainstem gliomas.

To evaluate prognostic factors and survival of adult patients with brainstem gliomas. Brainstem glioma is a disease found primarily in children, with a median survival of only 9 to 12 months. However, the prognosis and survival of adults with this disease has not been determined with precision. We conducted a retrospective analysis of patients older than 16 years at Memorial Sloan-Kettering Cancer Center with histologically proved or presumed brainstem glioma diagnosed between 1989 and 1997. We assessed the effect of gender, age at diagnosis, cranial nerve involvement, duration of symptoms, exophytic component, MRI enhancement, site of disease, treatment, and Karnofsky performance status on survival. Twenty-three patients were identified, but complete information was available in only 19 (12 males and 7 females). Patients ranged in age from 17 to 70 years (median, 40 years). Twelve patients were treated with radiotherapy at diagnosis and seven were observed, three of whom received subsequent radiotherapy. Median survival is 54 months (range, 3 to 98 months) and the 5-year survival is 45%. There was a trend for patients with a higher performance status at diagnosis to have longer survival, but this did not reach statistical significance. Other factors did not affect survival. Adults with brainstem gliomas may survive significantly longer than children, suggesting the disease may be less aggressive in adults. Furthermore, some patients with a long duration of symptoms or tectal or cervicomedullary tumors may be managed initially with observation alone.

Neuroradiology of Brain Stem and Cervicomedullary tumours

Brain stem and cervicomedullary tumours are typical of paediatric age, 80% of them occurring in patients under 18 years of age, and comprising 10–15% of all childhood and adolescent brain tumours, as well as 20–25% of infratentorial locations.They are characteristically pontine tumours (60% of the cases), but they commonly extend to involve the medulla, midbrain and cerebellum. Although most brain stem tumours are low grade gliomas, their prognosis is extremely severe (no more than 20% of patients are alive 3 years after diagnosis and the 5-year survival rate is 5%) because both the typical infiltrating nature and the neuro-anatomical location usually make them surgically unresectable. Surgery is generally limited to biopsy, partial decompression, or excission of the exophytic components, because of the extremely severe functional sequelae of even minor resections. Thus, the mainstay of therapy has been based on irradiation alone or combined with chemotherapy, doses of 5000–5500 cGy being usually adequate...

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.

Intramedullary spinal cord tumors in neurofibromatosis.

Neurofibromatosis (NF) describes the most commonly inherited disorders affecting the nervous system. These autosomal dominant, neurocutaneous syndromes are associated with multiple tumors of the nervous system, including neurofibromas, schwannomas, meningiomas, and intracranial gliomas. Spinal cord involvement in NF is typically from extramedullary growth of spinal nerve root tumors. Intramedullary spinal cord tumors in NF have been reported as scattered, single cases in literature. However, this association has not been clearly defined as have other nervous system neoplasms that are typically linked with NF. We present a series of nine patients with NF with intramedullary spinal cord tumors who were managed at our institution from 1984 to 1994. The patients' ages ranged from 4 to 31 years. There were seven male patients and two female patients. Three patients had NF-1 (von Recklinghausen's), five patients had NF-2, and one patient had NF, type uncertain. There were three cervicomedullary tumors, two cervical tumors, three cervicothoracic tumors, and one thoracic tumor. A histological examination revealed five ependymomas, three astrocytomas, and one intramedullary schwannoma. Two patients with malignant intramedullary spinal ocrd tumors have died with progressive disease. One other patient has required a subsequent operation for recurrent tumor. The other six patients are doing well, and their prognosis is expected to be related to their systemic disease. We conclude that there is a clinical entity of intramedullary spinal cord tumors associated with NF.

Current Trends in the Management of Brainstem Tumors in Childhood

Historically, the prognosis for a child diagnosed with a brainstem tumor has been bleak. The development of magnetic resonance imaging (MRI) has allowed for detailed visualization of tumors within the brainstem. This knowledge combined with the clinical history and neurological examination allows subcategorization of such tumors and delineation of children who might benefit from surgery. Surgical candidates include children with long clinical histories, focal neurological deficits and focal, cystic, exophytic or cervico-medullary tumors on the MRI scan. All children have the potential for multiple cranial nerve abnormalities, extremity weakness and swallowing and breathing difficulties. Nursing care of patients with brainstem tumors is complex and challenging.

Surgical Management of Brain Stem Tumors of Childhood and Adolescence

Intrinsic brain stem tumors are a heterogeneous group of neoplasms that may be classified according to clinical and neurodiagnostic criteria. We have described simple anatomic categories that include focal, diffuse, cystic, and cervicomedullary tumors. Although there is no surgical option for the common diffuse brain stem neoplasms, the other tumors may be operated on if the clinical and neurodiagnostic assessment suggests the possibility of a benign neoplasm. It is important to emphasize that although all tumors that were covered by intact ependyma or pia were considered intrinsic tumors in this series, most of these tumors created a "bulge" that was obvious on visual inspection of the external surface of the brain stem. In all of the tumors that were benefitted by surgery, the neoplasms tended to "bulge" from the brain stem rather than infiltrate it, which was the case with the diffuse neoplasms. It seems likely that many of the focal, cystic, and cervicomedullary tumors are similar to the dorsal exophytic brain stem tumors, which are characteristically low-grade astrocytomas that disrupt the ependymal lining and grow posteriorly into the fourth ventricle. Although surgery has been well tolerated and beneficial in many patients, it would be premature to comment on the duration of remission or the possibility of permanent cure.

Intrinsic brainstem tumors in childhood: surgical indications.

Sixty-six children with intrinsic brainstem gliomas diagnosed between 1980 and 1986 underwent radical surgical resection. Retrospective analysis permitted classification of tumors into four categories: diffuse, focal, cystic and cervicomedullary. All 27 patients with diffuse tumors had malignant neoplasms, were not benefitted by surgery and died within 12-18 months. Five of nine cystic tumors, three of five focal tumors and twenty of twenty-four cervicomedullary tumors had low grade histopathology and are alive one to six years postoperatively. The authors propose a clinical-neuroradiological criteria that accurately predict which patients with brainstem tumors are likely to benefit from radical surgical intervention.

Intrinsic brain-stem tumors of childhood: surgical indications.

This report summarizes the first author's experience with radical excision of intrinsic non-exophytic brain-stem gliomas in 34 pediatric patients. On retrospective analysis, these tumors may be classified into three subgroups: focal, diffuse, and cervicomedullary. A focal neoplasm is a circumscribed mass less than 2 cm in diameter and without associated edema. Tumors of a larger size or in which the "focal" neoplasm is associated with a large area of apparent edema are classified as diffuse. Cervicomedullary neoplasms occur at the junction of the medulla and spinal cord and involve both of the structures but do not extend rostrally into the pons. A radical tumor excision was carried out in all patients, and the only mortality and morbidity occurred in children harboring diffuse gliomas. All of the diffuse gliomas were malignant (grade III or IV astrocytomas), whereas three of the four focal astrocytomas and all of the cervicomedullary tumors were grade II astrocytomas. No patient with a diffuse astrocytoma was benefitted by surgery, while two of the focal astrocytomas and all of the cervicomedullary neoplasms either became stable or improved postoperatively. It is concluded that, although surgery may be accomplished within the substance of the brain stem with low morbidity and mortality rates, it is not indicated for malignant astrocytomas as it has no impact on the biology of the neoplasm. Therefore, while primary radical excision is recommended for cervicomedullary neoplasms, which are often benign, the more traditional radiation therapy and/or chemotherapy remain appropriate for tumors above the medulla.

Les potentiels evoques somesthesiques dans les tumeurs intra-rachidiennes

SEPs have been recorded in 15 patients with spinal cord tumors (mostly ependymomas) and in 5 patients with cord compression due to meningiomas (3 cas), neuromas (1 case) or neurosarcoma (1 cas). All the patients have been operated on so that precise informations were available on the histology and the location of the tumor. SEPs were monitored during surgery in 8 patients. The main conclusions of this study are as follows: There was a good correlation between the somatosensory troubles for touch, vibration sense and joint position sense, and SEPs abnormalities, however, SEPs may be clearly abnormal in the absence of any somatosensory deficit when the dorsal columns are compressed and not infiltrated by the tumor. In the latter situation the reverse dissociation (i.e., normal SEPs with somatosensory troubles) may be observed. The possibility of a dissociation between normal N11 and N13 cervical components and absent P14 far field components (non-cephalic reference) has been confirmed in 6 patients with cervicomedullary tumors. In 3 of them, this dissociation was found to be reversible and early SEPs returned to normal after surgery. Peroperative monitoring of scalp SEPs recorded with a non-cephalic reference electrode allowed the detection of transient SEP abnormalities related with a traction on the dorsal columns of the cord. SEP monitoring is recommended for any surgical removal of tumor that needs a posterior myelotomy.