Malignant glioblastomatous transformation of a low-grade glioma in a child

Background  The term of low-grade glioma addresses a favorable clinical outcome with indolent histological features in general consideration; however, recent studies underline the inconsistency, which originates from the accumulation of different histologic subtypes in this terminology. The malignant transformation of a low-grade glioma is unusual but presents a poor prognosis. Case history  We report a case of a 12-year-old boy, who was referred for complaints of recurrent seizures. His physical examination was unremarkable, but it was learned that a peripheral mass lesion located on the left posterior parietal lobe—which had been thought to be a low-grade glioma—had been detected on a magnetic resonance imaging 2 years ago at a different hospital. The patient was then treated with valproate and carbamazepine for the seizures and advised to be followed up without any additional diagnostic and therapeutic studies for his suspected low-grade glioma. A recent magnetic resonance imaging study showed enlargements of the mass and surrounding edema with additional necrosis. Surgical excision of the tumor was performed. After the diagnosis of glioblastoma multiforme, the patient received radiation therapy and chemotherapy with a good clinical recovery without any evidence of residue or recurrence at 12-month follow-up. Conclusion  The first line treatment modality in the management of low-grade glioma—especially in suitable patients—is clearly surgery. The gross total resection guarantees the distinguishing of the histological types of the low-grade gliomas and reflects the biologic behavior of these tumors. Observation without surgery must be reserved for selected unoperable cases.     

Surgery for gliomas

PURPOSE OF REVIEW: To assess selected papers on surgery for glioma for their impact on clinical practice. RECENT FINDINGS: Recent developments in surgical neuro-oncology for gliomas have centred around technological advances that enable the fusion of preoperative structural and functional imaging datasets, the use of intraoperative magnetic resonance imaging scanning, and awake craniotomy and cortical stimulation as means to maximize glioma resection, minimize postoperative morbidity, and improve survival times. Correlations of preoperative functional imaging information with operative awake neurophysiological findings are good, but the problem of brain shift during resective surgery remains problematical and is a cogent reason for using intraoperative magnetic resonance imaging. Two reviews showed little evidence to support the concept that 'aggressive' resection of both high and low-grade gliomas significantly prolongs the life of patients. Attempting radical excision of these tumours can have unfortunate consequences in eloquent brain regions, particularly as functional studies confirm brain activity within the limits of many gliomas. SUMMARY: Despite amazing technical advances in the investigation, assessment and surgical management of patients with glioma, the lack of an evidence basis for 'aggressive' resective management continues to pose dilemmas for surgeons.     

Radiological and clinical outcome following stereotactic biopsy and radiotherapy for low-grade insular astrocytomas

Thirty patients with low-grade (WHO Grade II) astrocytomas involving the insula, who had undergone stereotactic biopsy followed by radiotherapy, were followed up to evaluate the outcome with regard to control of seizures, memory and language function, Karnofsky Performance scale and regression in tumor volume. Patients were followed up for a mean of 27.8 months, during which time they showed improvement in all the factors that were studied. A statistically significant change was, however, seen only in the reduction in tumor size, probably due to the small sample size and the short duration of follow-up. Stereotactic biopsy followed by radiation therapy provides a good short-term outcome in patients with low-grade insular astrocytomas.     

Symptomatic cranial fibrous dysplasias: clinico-radiological analysis in a series of eight operative cases with follow-up results.

Cranial fibrous dysplasias are rare and comprise less than one percent of all primary bone lesions. These painless progressively expanding destructive bone swellings produce cosmetic deformities (commonest), sino-orbital and auditive complications (less common), peripheral compressive cranial neuropathies (uncommon) and compressive central neurological manifestations (rarest). Until recently some of the primary dysplastic skull base lesions were either treated expectantly or excised incompletely. In a significant percentage of cases, these lesions were considered inaccessible. However, with the development of modern neuro-imaging (CT and MRI) and the applications of current skull base approaches, the more aggressive and radical surgical procedures are being undertaken, with a significant improvement in overall outcome. We present a series of 8 cases of fibrous dysplasia in the age group of 10-33 years with a mean age of 19.75 years. Four cases each had monostotic type and polyostotic type. All patients presented with mild to moderate headaches, and seven cases with some form of cosmetic deformity. In 4 patients there was a unilateral proptosis, while only two had visual deterioration. One patient had anosmia and one hearing impairment. In one case fibrous dysplasia was hemicranial, and in another case the disease was pancranial. Neuro-imaging studies have well delineated the extent of the lesions. In two patients with unilateral fronto-orbital fibrous dysplasia, the removal of dysplastic bone, autoclaving and remodeling and replacement were performed. In two patients of sphenoidal wing fibrous dysplasia a total excision was possible. In the remaining four patients, a partial excision was performed. Overall outcome was good. There was no mortality. One patient had recurrent post-operative extradural haematomas. The authors present the results of an analysis of eight surgically treated cases of symptomatic cranial fibrous dysplasias along with their long-term outcome and the lessons learnt from this series.     

Focal neuronal migration disorders and intractable partial epilepsy: results of surgical treatment.

Twenty-six patients with focal or lateralized neuronal migration disorders and intractable partial epilepsy were treated surgically. Twenty-four had reliable follow-up ranging from 1 to 15 years (mean, 5.0). Pathologically, they fell into two categories: focal cortical dysplasia (12 patients) and forme fruste of tuberous sclerosis (8 patients). In the remaining 4 patients, the material was inadequate for histological analysis. Outcome regarding seizure control was assessed according to a classification most sensitive to variations in frequency of major attacks. Ten (42%) of the 24 patients achieved good or excellent outcome, 6 (25%) had a worthwhile decrease in seizure frequency, and 8 (33%) had only discrete improvement. The variable most strongly correlated with surgical outcome was the amount of lesion removed. Seventy-seven percent of patients in whom a complete excision or excision of 50% or more of the lesion was accomplished achieved excellent or good surgical outcome. Conversely, no patient with less than 50% of the lesion removed attained the same result. There was no correlation between other clinical, radiological, or electrographic variables and outcome regarding seizure control. Specifically there was no significant correlation between the amount of excision of the epileptogenic area as judged by scalp electroencephalography and electrocorticography studies, and surgical outcome. In patients with neuronal migration disorders and intractable partial epilepsy, removal of the structural abnormality takes precedence over removal of epileptogenic tissue as the main surgical strategy to achieve seizure control.     

Surgical outcome in occipital lobe epilepsy: Implications for pathophysiology

Medically refractory occipital lobe epilepsies are increasingly treated with surgery, but outcome and its relationship to etiology, pathological substrate, occipital lobe location, surgical approach, and electroclinical features have not been systematically investigated in a substantial group of patients. Thirty-five patients who underwent surgery for intractable occipital lobe seizures were retrospectively evaluated. Outcome and occipital lobe location were analyzed with respect to surgical procedure, pathology, clinical seizure characteristics, seizure onset and termination locations, and localization of interictal spikes. Most patients had developmental abnormalities (14) or tumors (13, all gliomas). Developmental abnormalities consisted of focal cortical dysplasia (5), heterotopia (2), hamartoma (3), cortical duplication (1), polymicrogyria (1), Sturge-Weber syndrome (1), and tuberous sclerosis(1). There was 1 patient with a vascular abnormality, 1 with chronic inflammatory changes, 4 with gliosis, 1 with cerebral ossification, and 1 with normal pathology. Developmental abnormalities had significantly worse outcome (45% excellent/good) than tumors (85% excellent/good). In the developmental group, low-grade focal cortical dysplasias had better outcome than heterotopia and hamartoma regardless of type of surgical procedure. Pathological groups did not significantly differ with respect to location within the occipital lobe (overall medial [50%] or lateral [38%]); clinical seizure characteristics referable to specific lobe (occipital [14%], temporal [34%], frontal [23%], more than one type [29%]); electroencephalographic localization (to occipital [17%], temporal [27%], or other/multifocal locations [56%]); or intracranial ictal onset or termination location. Electroclinical variables were also unrelated to the occipital lobe location of abnormality. Surgical outcome was not predicted by surgical approach (lesion excision with margins or lobectomy). The main pathological substrates of uncontrolled occipital lobe epilepsy are gliomas and developmental abnormalities. Whereas resection of occipital lobe tumors associated with chronic epilepsy produces nearly uniform seizure control, outcome after resection of occipital lobe developmental abnormalities is less uniform.     

Anaplastic astroblastoma of childhood: aggressive behavior

Objective  Astroblastoma is an unusual brain tumor in childhood. Tumor usually arises from cerebral hemispheres. This large tumor is well-demarcated, lobulated, solid or cystic, and therefore, may resemble glioblastoma. Histopathologically, there are two types of astroblastoma: well-differentiated (low-grade) or anaplastic (high-grade). In low-grade astroblastoma, treatment of choice is complete excision. This type of tumor usually does not recur. However, anaplastic tumors can recur despite surgery, radiation, and chemotherapy and may be problematic for clinician. Case Report  A 7-year-old female patient presented with an acute onset of vomiting and seizure. Magnetic resonance imaging study revealed a large mass in the left parieto-occipital region. She underwent total excision of the tumor. Histopathologically, the tumor was an anaplastic astroblastoma. Her adjuvant treatment was planned to consist of radiation therapy and cisplatin-based chemotherapy. However, the tumor recurred early in the course, and she died 18 months after diagnosis. Conclusion  High-grade astroblastomas behave like glioblastoma, as emphasized in this case report. Local control of this type of tumor seems difficult despite surgery, radiation therapy, and cisplatin-based chemotherapy.     

Supratentorial cavernous haemangiomas and epilepsy: a review of the literature and case series

OBJECTIVES: To characterise the clinical features and response to treatment of supratentorial cavernomas associated with epilepsy. METHODS: A systematic review of the literature was carried out and a retrospective case series of patients with cavernoma diagnosed by MRI and/or histology was compiled. Patient selection biases in the literature review were reduced as far as possible by selection of unbiased publications. RESULTS: In the literature, cavernomas were relatively less common in the frontal lobes. There were multiple cavernomas in 23% of cases. The main clinical manifestations were seizures (79%) and haemorrhage (16%). The annual haemorrhage rate was 0.7%. The outcome after excision was good with improvement in seizures in 92% of patients. In the case series the surgical outcome was less favourable, reflecting inclusion of a higher proportion of patients with intractable epilepsy. In both the literature review and the case series, outcome was poorer in cases with a longer duration of seizures at the time of surgery. CONCLUSIONS: The good surgical results, particularly in cases treated earlier, and the significant cumulative haemorrhage rate, suggest that excision is the optimum treatment. However, these factors have not been examined prospectively and, despite the availability of several retrospective studies, the optimum treatment, particularly for non-intractable cases, will only be determined by a prospective study.     

Effect of age on treatment decisions in low-grade glioma.

The proper treatment of low-grade glioma is unclear and major uncertainties include the timing of therapy, the need for extensive surgery, or the application of radiotherapy. Although prospective trials are in progress, it may be years before results become available, as the five-year survival of low-grade glioma is around 45% or more. Age is an important prognostic factor in malignant glioma, but its implications for decisions on treatment have not yet been addressed in guidelines. This review examines the interaction between age and the results of applied treatment, based on data from published series. The available evidence suggests that, in younger patients, whether treatment is started early or late does not seem to affect long-term survival substantially. For patients under 35 years of age, more radical surgery appears to be beneficial, while radiation does not seem to improve the outcome. For patients who are 35 years and older, surgery and radiotherapy seems to produce better survival rates. The age of the patients should therefore be considered when decisions on the treatment of supratentorial, non-pilocytic, low-grade gliomas. For patients under 35 years of age who have either epilepsy or a surgically inaccessible tumour, it is advisable to defer treatment. The tumour should be largely excised, if possible. Following any surgery, radiotherapy should be withheld in this age group. For patients over 35 years of age, early treatment, including biopsy or surgery followed by radiotherapy, should not be delayed. Because of more prolonged survival, and to prevent neurotoxicity, radiation fields should be limited to the tumour bed and not include the whole of the brain. Future trials need to establish whether age is a crucial factor in deciding the timing and extent of treatment in patients with low-grade glioma.     

Temporal lobectomy in early childhood: the need for long-term follow-up

We retrospectively identified 15 children ages 12 years and under with anticonvulsant resistant epilepsy who underwent a temporal lobectomy at Children's Hospital, Boston, between 1978 and 1993. Our aim was to study the long-term seizure outcome. Data pertaining to preoperative evaluation, electroencephalography (EEG), neuroimaging, surgery, seizure outcome, and postoperative complications were reviewed. Only patients followed for more than 12 months were included. The average duration of follow-up was 57 months. At the last visit, 47% (7 of 15) of the children were seizure free or only had auras: another 33% (5 of 15) had > 90% reduction in seizure frequency. Three patients had < 90% seizure reduction. Four cases were initially seizure free but had subsequent recurrence between 11 and 28 months after the epilepsy surgery. Factors associated with a good outcome include exclusively focal EEG discharges or an imaging suggestive of a low-grade tumor; factors associated with a poor outcome include generalized EEG discharges and a normal magnetic resonance image. Temporal lobectomy is useful in the treatment of early childhood drug-resistant partial epilepsy, but long-term follow-up is necessary as late seizure recurrence may occur up to 28 months after surgery.     

Successful early-stage corticosteroid treatment in a case of granulomatous angiitis of the central nervous system]

A 74-year-old woman presented with progressive mental deterioration following a low-grade fever and headache. Upon admission, she appeared lethargic, could not obey simple commands and was disoriented to time, place and person. She had low-grade fever and mild neck stiffness. The cerebrospinal fluid had an elevated protein content of 496 mg/dl, contrast-enhanced MRI revealed diffuse leptomeningeal enhancement, particularly in the occipital area, and a cerebral angiogram showed diffuse segmental narrowing of multiple intracranial arteries, especially in the distal portion of the right middle cerebral artery. A clinical diagnosis of granulomatous angiitis of the central nervous systems (GANS) was made, and corticosteroid therapy was initiated. The patient improved gradually, and corticosteroid therapy was tapered to the maintenance dose (prednisolone 0.4 mg/kg daily). An open brain biopsy showed multiple vessels containing granulomatous inflammation with giant cells. GANS is one of the most challenging neurologic disorders to diagnose because of its relative rarity and the lack of specificity of clinical signs and efficient, non-invasive, diagnostic tests. In this case, we were able to begin corticosteroid therapy in the early stage of the disease (before brain biopsy), and it yielded a good outcome.     

Spinal drop metastasis from grade I skull base chondrosarcoma

Chondrosarcoma of the skull base is a rare tumour with a good prognosis following surgical resection. We describe a patient with low-grade chondrosarcoma of the skull base with intradural extramedullary spinal metastases. A 31-year-old female with grade 1 chondrosarcoma involving the cavernous sinus, sphenoid wing and clivus presented at age 19. The tumour was subtotally excised at initial surgery and over the following 4 years, 3 subsequent resections were undertaken for tumour progression followed by proton beam radiotherapy to the residual tumour. The patient re-presented with cervical radiculopathy 7 years later. MRI showed multiple, intradural extramedullary spinal drop metastases. Following surgical excision of the symptomatic lesion, histological diagnosis was confirmed as a mixed hyaline/myxoid grade 1 chondrosarcoma. Patients with skull base chondrosarcoma with intradural extension should have whole spine imaging as part of long-term monitoring to exclude drop metastases, particularly after intradural surgery.     

Diagnosis and surgical management of intraspinal synovial cysts: report of 19 cases

OBJECTIVE: Synovial cysts of the vertebral facet joints are a source of nerve root compression. Different surgical procedures are in use, but no consensus has been formed so far as to which method should be used in synovial cysts. To clarify the role of surgical management, the efficacy of operative procedures and factors influencing the outcome in our own series of 19 patients treated between 1994 and 1998 were analysed. METHODS: Nineteen patients with a mean age of 65 years underwent surgery for medically intractable radicular pain or neurological deficits caused by synovial cysts. The patients' records were retrospectively analysed for neurological deficits, cysts diameter, operative approach, segmental hypermobility, and clinical outcome; CT and MRI were analysed for additional degenerative changes. RESULTS: In 17 patients an excellent result and in two patients a good postoperative result was achieved. Twelve patients were found to have hypermobility of the facet joints and six had spondylolisthesis. There was no correlation between cyst diameter, operative approach, and outcome. No intraoperative or postoperative complications occurred. CONCLUSIONS: Age and hypermobility may play a part in the aetiology of facet joint synovial cysts. As all operative strategies showed equally good clinical outcome, total excision via a small flavectomy as the least invasive approach should be considered therapy of choice in patients with cysts causing neurological deficits.     

中颅窝底硬膜外血肿的分型和诊治分析（附21例报告）

目的对21例中颅窝底硬膜外血肿进行分型,分析其临床及CT特点、手术时机和手术要点。方法对21例患者的临床资料进行回顾性分析。结果重型和中型组手术治疗,轻型组先保守治疗,4例中转手术。6月后按GOS评估,重型组恢复良好3例,中残2例,重残2例,死亡1例;中型组中残1例,良好4例;轻型组死亡1例,良好7例。结论中颅窝底硬膜外血肿有特殊的临床特点和CT表现;重型即刻手术,中、轻型手术指征要放宽,选择合理的手术方案,抓住手术要点,有利于改善预后。     

Pediatric intramedullary spinal cord tumors Critical review of the literature

The objective of this review was to analyze the literature on the management of intramedullary spinal cord tumors to determine whether enough information was available for treatment guidelines to be established. Using standard computerized search techniques, databases containing medical literature were queried for keywords related to intramedullary spinal cord tumors, beginning in 1966. Of the 445 articles published in English and with potential relevance, only 75 articles were included in the final analyses. Based on the strength of their recommendations for the treatment of this controversial condition, articles were divided into class I, class II and class III data. There were no class I studies related to any aspect of the treatment of intramedullary spinal cord tumors. Based on this critical review of literature, gross total removal of an ependymoma confirmed by immediate postoperative magnetic resonance imaging and adjunctive treatment for high-grade tumors using radiotherapy, with or without chemotherapy, can be recommended as standards of therapy. With the strength of a guideline, radiotherapy should be withheld after gross total removal of intramedullary ependymomas and radical resection of low-grade intramedullary astrocytomas. The use of intraoperative ultrasonography and evoked potentials, important surgical adjuncts, can also be considered guidelines. The radical resection of intramedullary low-grade astrocytomas is an option. Received: 15 October 1998     

Intractable Epilepsy and Structural Lesions of the Brain: Mapping, Resection Strategies, and Seizure Outcome

Forty-seven patients with structural brain lesions on neuroimaging studies and partial epilepsy intractable to medical therapy were studied. Prolonged noninvasive interictal and ictal EEG recording was performed, followed by more focused mapping using chronically implanted subdural electrode plates. Surgical procedures included lesion biopsy, maximal lesion excision, and/or resection of zones of epileptogenesis depending on accessibility and involvement of speech or other functional areas. The epileptogenic zone involved exclusively the region adjacent to the structural lesion in 11 patients. It extended beyond the lesion in 18 patients. Eighteen other patients had remote noncontiguous zones of epileptogenesis. Postoperative control of epilepsy was accomplished in 17 of 18 patients (94%) with complete lesion excision regardless of extent of seizure focus excision. Postoperative control of epilepsy was accomplished in 5 of 6 patients (83%) with incomplete lesion excision but complete seizure focus excision and in 12 of 23 patients (52%) with incomplete lesion excision and incomplete focus excision. The extent of lesion resection was strongly associated with surgical outcome either in itself (p less than 0.003), or in combination with focus excision. Focus resection was marginally associated with surgical outcome as a dichotomous variable (p = 0.048) and showed a trend toward significance (p = 0.07) only as a three-level outcome variable. We conclude that structural lesions are associated with zones of epileptogenesis in neighboring and remote areas of the brain. Maximum resection of the lesion offers the best chance at controlling intractable epilepsy; however, seizure control is achieved in many patients by carefully planned subtotal resection of lesions or foci.(ABSTRACT TRUNCATED AT 250 WORDS)     

Late malignant recurrence of childhood cerebellar astrocytoma

Juvenile pilocytic astrocytoma of the cerebellum has a benign course and a good prognosis. We report a case of juvenile cerebellar astrocytoma in a 6-year-old girl that underwent surgical resection of the tumor and had two recurrences, 13 and 35 years after first removal. After surgery the patient did not receive any radiation therapy. The last relapse showed histological features of an anaplastic astrocytoma. Six months later the patient died with a diffuse leptomeningeal dissemination. Late malignant transformation of a benign cerebellar astrocytoma is very rare and it is thought to be favored by postsurgical irradiation. The possible pathogenetic mechanisms of this evolution are discussed. This case and the few others reported in the literature emphasize the risk of an unpredictable outcome with the low-grade cerebellar astrocytomas of childhood.     

High angiogenic potential in an in vivo rat corneal model is associated with shorter disease-free survival in low-grade oligodendrogliomas

This study aimed to examine the association between time to tumor recurrence, angiogenic potential and tumor contrast-enhancement. Tumor samples were taken from 20 patients with low-grade oligodendroglioma and examined for their angiogenic potential using an in vivo rat corneal model of angiogenesis. Patients were evaluated for tumor contrast enhancement prior to surgical excision using MRI and they were followed for tumor recurrence. Patients who had tumors without contrast enhancement had longer disease-free survival (median time to tumor recurrence, 72 months) compared to those who had tumors with contrast enhancement (median, 42 months; p = 0.0068). Based on corneal angiogenesis assay results, a high angiogenic potential was associated with a significantly shorter disease-free survival. Our findings suggest that radiological contrast enhancement and a high angiogenic potential based on an invivo corneal angiogenesis assay were related to a shorter disease-free survival. This might have important prognostic implications in patients with low-grade oligodendrogliomas.     

Management of intramedullary spinal cord tumours: review of 68 patients.

68 consecutive patients admitted with intramedullary spinal cord tumours and operated at Vellore during a six year period from January 1990 are discussed. 41 tumours were radically resected, 11 partially excised while 14 had only a biopsy. Radiation therapy was advised post operatively to those patients for whom a partial excision or biopsy was done. There was no postoperative mortality. Two patients developed wound infection and one developed postoperative hydrocephalus. Postoperative clinical assessment between four to eight weeks after surgery showed that 25 out of 68 patients improved, 29 remained unchanged, while 14 had worsening of deficits. Immediate post operative assessment, however, was less encouraging. Evaluation of these patients was done using a functional scoring system and Karnofsky rating. The follow up period ranged from 2 weeks to 64 months after discharge from hospital with a mean of 14.6 months. The indicators of radical excision were good tumour-cord interface, cranially located tumours, presence of syringomyelia and histology of ependymoma. Two patients had recurrence of tumour.     

Glioma and multiple sclerosis: case report

We report a case of a 44-years-old woman with relapsing-remitting and secondarily progressive form of multiple sclerosis (MS) since aged 24 years, who developed an anaplastic astrocytoma. The neurological manifestations of the tumor were misinterpreted as resulting from MS. Sequential MRI examination and seizures raised the possibility of another nature of her symptoms, besides MS. Her initial good response to high doses corticosteroids led to the initial assumption her symptoms were only exclusively due to the demyelinating process. She underwent craniotomy with radical excision of the lesion. Pathological examination disclosed anaplastic astrocytoma. Other cases of coincidental MS and primary CNS tumors are reviewed, as well as their possible relation.