Diagnosis Of Brainstem Glioma Research Articles

Correlation between MRI findings and histological diagnosis of brainstem glioma

Objective To compare the MRI characteristics with histopathological findings of brainstem glioma and identify the characteristics associated with the diagnosis of pathologies. Methods From January 2002 to January 2012, 150 patients was diagnosed as brainstem gliomas by MRI and received operations in Beijing Tiantan Hospital. All the MRI and histopathological findings of these patients were respectively reviewed. Results One hundred and eight patients were low-grade glioma (72.0%), and 35 high-grade (23.3%). The accuracy of the MRI diagnosis for brainstem glioma was 95.3%. The data analysis of the MRI findings revealed that in not only the intrinsic (P=0.005) but also the exophytic (P=0.001) brainstem glioma patients, having a focal lesion was a significant factor associated with a more favorable histopathological diagnosis. In the intrinsic diffuse type, tumors without enhancement had the better pathologies (P=0.009). There was no significant difference of the histopathological diagnosis between the adults and children in not only the intrinsic (P=0.624) but also the exophytic (P=0.771) brainstem glioma. Conclusions The results of this study could help us to estimate the pathology with data obtained from MRI and help to obviate the need for invasive biopsy. The pathology of tumors with a focal appearance was better than their diffuse counterparts. For the intrinsic diffuse type, tumors with nonenhancement might have a better pathology. The different growth pattern and invasiveness of brainstem glioma between children and adult could be due to the different host cellular environments between the two age groups. Key words: Brain stem neoplasms; Glioma; Magnetic resonance imaging; Pathology

Two cases of posterior reversible encephalopathy syndrome resembling brainstem glioma

A brainstem glioma is often the initially suspected diagnosis hen a diffuse abnormal signal is found localized in the brainstem n magnetic resonance imaging (MRI). An appropriate differential iagnosis is needed based on clinical presentation, neuroimagng results and pathological finding by surgical biopsy, in order o make a rapid and accurate diagnosis of this pathological conition, which can have a fatal clinical course. Two patients were nitially given a diagnosis of brainstem glioma, but subsequently iven a diagnosis of posterior reversible encephalopathy synrome (PRES) due to their distinctive clinical presentation and euroimaging results and could be avoided dangerous surgical iopsy. Case 1. The patient was a 54-year-old woman with no hisory of hypertension. She began to experience insomnia a month efore admission due to work stress. She went to a local clinic or a physical examination and a head MRI scan due to the suden onset of dysarthria and right mild hemiparesis. In addition to acunar infarction of the left corona radiata, she was highly susected a diagnosis of brainstem glioma, because the differential iagnosis of the physician was limited. Her blood pressure was

A Case of Posterior Reversible Encephalopathy Syndrome Presenting with Isolated Diplopia

Background: Posterior Reversible Encephalopathy Syndrome (PRES) is a clinical entity characterized by a unique pattern of vasogenic brain edema mainly caused by eclampsia, immune suppressing drugs, or severe hypertension. The disease typically affects parieto-occipital lobe as the nomenclature indicates. Here we report a man with isolated concomitant strabismus, who was initially suspected as having glioma but was finally diagnosed with PRES. Methods: A 43-year-old man complained of horizontal double vision for a week. He had no other neurological symptom, including headache or confusion. He had a history of hypertension but had no medication. On examination, he showed 10 to 12 prism diopter of exotropia in all gaze directions without any restriction of eye movement. The sudden onset of the symptom and the history of hypertension urged us to take head MRI, which showed poorly-demarcated T2-high intensity area in extensive brainstem and bilateral cerebellum. The image suggested a plausible diagnosis of brainstem glioma. Results: He was admitted for planning brain biopsy. Since his systolic blood pressure was over 240 mmHg, he was to undergo blood pressure control before the biopsy. As the pressure decreased to around 180 mmHg, his symptom improved gradually. The lesion diminished in 2 weeks. Finally, he was diagnosed with PRES due to hypertension judged from the clinical course. Conclusions: PRES can affect any locations in central nervous system including brainstem as shown in the present case. And of note, the patients with brainstem variant of PRES may present only minimal symptoms such as diplopia in the present case. Differentiation from tumors or infarction is very important to avoid unnecessary and invasive interventions. One useful characteristic is the clinical radiologic dissociation. Once brainstem variant of PRES is diagnosed, to monitor and control blood pressure is important because it is often caused by severe secondary hypertension.

Paediatric brainstem gliomas: Prognostic factors and management

Background Although most types of tumours involving the brainstem can be surgically removed, patients with diffuse pontine brainstem glioma do not benefit from surgical intervention. The aim of the present study is to establish whether clinical symptoms, their duration prior to diagnosis, the presence of enhancement on MRI scans and the histology of the lesion in paediatric brainstem glioma can guide the surgeon in choosing appropriate management options. Methods We retrospectively reviewed the charts and MRI scans of paediatric patients admitted to Turin’s Ospedale Regina Margherita with a diagnosis of brainstem glioma. Results Patients with a diffuse pontine tumour on MRI scan (group 1) had a mean duration of symptoms prior to diagnosis of 2.61 months, 77% had symptoms involving at least one cranial nerve at diagnosis, and no MRI scans showed enhancement with gadolinium. No patients underwent radical surgery and 84.6% died. For all other patients (group 2), the mean duration of symptoms at diagnosis was 10.58 months, cranial nerve involvement was present in only 28.5%, and the MRI scans showed enhancement in 78.6%. Radical surgery was the treatment of choice (100%). In this study, survival of patients with diffuse pontine brainstem glioma was 25% at 2 years and survival of patients with any other brainstem glioma was 90% at 2 years.

Advances Toward an Understanding of Brainstem Gliomas

The diagnosis of brainstem glioma was long considered a single entity. However, since the advent of magnetic resonance imaging in the late 1980s, neoplasms within this anatomic region are now recognized to include several tumors of varying behavior and natural history. More recent reports of brainstem tumors include diverse sites such as the cervicomedullary junction, pons, midbrain, or the tectum. Today, these tumors are broadly categorized as either diffuse intrinsic gliomas, most often in the pons, or the nondiffuse brainstem tumors originating at the tectum, focally in the midbrain, dorsal and exophytic to the brainstem, or within the cervicomedullary junction. Although we briefly discuss the nondiffuse tumors, we focus specifically on those diffuse brainstem tumors that regrettably still carry a bleak prognosis.

Intratumoral hemorrhage among children with newly diagnosed, diffuse brainstem glioma

Children with diffuse brainstem glioma (BSG) commonly undergo novel therapies because their outcome is poor with radiation therapy (RT). Although recent clinical trials using new biologic agents documented intratumoral hemorrhage (IH) among several children with BSG, to the authors' knowledge little is known regarding this phenomenon. In the current study, the authors assessed the characteristics and estimated the cumulative incidence of IH among children with BSG. All available brain imaging studies and medical records of 48 consecutive patients with newly diagnosed BSG treated at the study institution over a 10-year interval (1992-2002) were reviewed. Treatment was comprised of RT and various regimens of conventional chemotherapy; none of these patients received biologic agents. At the time of last follow-up, all patients had died of tumor progression. The authors reviewed 319 imaging studies (251 magnetic resonance imaging scans and 68 computed tomography scans). IH was present in 6.25% of patients at the time of diagnosis. The 6-month and 12-month cumulative incidence estimates of IH regardless of the associated symptoms were 15.5% +/- 5.5% and 24.4% +/- 6.5%, respectively. The same estimates for symptomatic cases were 8.9% +/- 4% and 17.8% +/- 6%, respectively. All cases of IH at the time of diagnosis and 78% of symptomatic cases that developed after diagnosis were located in necrotic areas. Although IH is uncommon at the time of diagnosis, symptomatic IH may occur among nearly 20% of children after the diagnosis of BSG. The uniform occurrence of IH among patients treated with various chemotherapeutic regimens and its association with necrotic areas suggests that tumor biology plays a significant role in this event.

Posterior fossa swelling and hydrocephalus resulting from hypertensive encephalopathy: case report and review of the literature

OBJECTIVE AND IMPORTANCE: Brain stem and cerebellar edema rarely have been described as the principal manifestation of hypertensive encephalopathy. In addition, secondary hydrocephalus has been described in only a few cases in the literature. We present an unusual case of posterior fossa swelling and hydrocephalus resulting from hypertensive encephalopathy. CLINICAL PRESENTATION: A 53-year-old man presented with increased shortness of breath, headache, and visual changes, which had been worsening for several months. Blood pressure on presentation was 253/140 mm Hg. Neuroradiological studies revealed brain stem swelling predominantly affecting the pons, with compression of the adjacent cisterns and fourth ventricle and resultant hydrocephalus. The diagnosis of brain stem glioma was briefly entertained. INTERVENTION: The patient's blood pressure was brought under control with medical management, and he was treated with dexamethasone for 48 hours. Subsequent neuroradiological studies revealed decreased posterior fossa edema as well as marked improvement in the hydrocephalus. CONCLUSION: Hypertensive encephalopathy can present principally in the posterior fossa and can give rise to obstructive hydrocephalus. Invasive treatment of the hydrocephalus is not necessarily required in this clinical setting because reduction of the blood pressure may result in rapid improvement of the hydrocephalus.

Permanent I-125 brain stem implants in children.

Between 1988 and 1997, 28 children have had iodine-125 implants for CNS tumors performed in our institution. Ten had stereotactic implantation in the brain stem region, and nine had the diagnosis of brain stem glioma (8 diffuse pontine, 1 midbrain tumor). Their ages ranged from 1.8 to 12 years. All patients had histological confirmation of malignancy (7 high-grade glioma, 2 low-grade glioma, 1 PNET). Diffuse pontine glioma patients received external beam radiation (50 Gy) followed by a fractionated stereotactic boost of 3 Gyx4 fractions. After 4-6 weeks, patients were reevaluated for stereotactic interstitial I-125 therapy. The planned implant dose was 82.9 Gy to the enhancing tumor (4 cGy per h). Preliminary results indicated that no surgical complications were associated with the catheter placement. Four patients have died (7-9 months from diagnosis) and four patients remain alive (5-38 months from diagnosis, median 10 months). Two autopsies confirmed the presence of progressive glioblastoma multiforme and intralesional necrosis. In one patient who received an implant alone for midbrain LGA, necrosis without tumor was found on biopsy after 36 months. He was successfully treated with hyperbaric oxygen therapy. The implementation of permanent I-125 implants appears to have a role in the management of pediatric CNS malignancy. This study confirms the results of previous reports regarding the safety of stereotactic interstitial brachytherapy in the brain stem. Tumor control for patients with high-grade brain stem glioma remains poor even with high focal radiation doses.

Pediatric brain stem gliomas with the predominant symptom of sleep apnea

Two children complaining of sleep apnea presented with brain stem gliomas. In the early stage of their illness, neurological disorders were too subtle to be recognized as significant by the physicians or to be noted by the parents. Case 1 experienced an episode of unsteady gait and weakness in the bilateral arms, at the age of 5. When it recurred after 7 years of remission, the predominant symptom was sleep apnea. Case 2 exhibited nasality of speech as the earliest sign of his illness very early in his life, presumably 5 years before the diagnosis of brain stem glioma. A slight sleep apnea which developed afterwards did not draw attention of the physicians because no neurological signs other than paralyses of the bilateral soft palates were present. MRIs of the both cases revealed diffuse, infiltrating lesions in the pons, the medulla oblongata and the upper cervical spinal cord. Both cases shared some features: (1) diagnostic delay of several years from the first symptom; (2) the main lesion in the medulla oblongata, where important structures for respiratory control are identified; (3) infiltrative growth patterns in the MRI of the tumor, which might account for the uncommon clinical courses.

Stereotactic biopsy of brainstem mass lesions

Appropriate therapy of brainstem lesions is guided by accurate diagnosis. Because the majority of brainstem lesions are not amenable to surgical resection, stereotactic biopsy is an attractive method of obtaining pathological tissue. We reviewed the medical records of all patients who underwent stereotactic biopsy of brainstem mass lesions at our institution by the senior author (WAF) over a 10-year period ending in December 1993. Twenty-four patients ranging in age from 3 to 68 years underwent stereotactic biopsy of brainstem mass lesions at our institution by the senior author (WAF) over a 10-year period ending in December 1993. Twenty-four patients ranging in age from 3 to 68 years underwent stereotactic biopsy of mass lesions of the brainstem. Sixteen lesions were located primarily in the pons, 7 in the midbrain, and 1 in the medulla. Twenty-two of the biopsies were approached transfrontally and two were approached via the suboccipital transcerebellar route. Pathological diagnosis was made in 23 of the 24 patients. The histologic diagnosis was astrocytoma in 16 patients, metastasis in 3, lymphoma in 1, germinoma in 1, chordoma in 1, progressive multifocal leukencephalopathy in 1, and was nondiagnostic in 1. Complications included 1 case of increased hemiparesis, 1 case of obstructive hydrocephalus, and 1 death. Six patients were less than 20 years of age and in each of these patients the preoperative diagnosis was astrocytoma. In all of these patients the pathology revealed astrocytoma. In the adult patients the pathology was more varied, with 7 of the 18 patients having tumors or pathology other than astrocytoma. The data suggest that, in adult patients, brainstem lesions are of varied pathology and stereotactic biopsy can provide adequate tissue for diagnosis. The data also suggest that the diagnosis of brainstem glioma in children can often be made without submitting the patient to the risk of surgery.

Predominance of pilocytic histology in dorsally exophytic brain stem tumors.

We report the magnetic resonance imaging (MRI) and clinico-histologic characterization of dorsally exophytic brain stem gliomas (DEBSGs). Between 1983 and 1991, 12 of 51 patients evaluated for the diagnosis of brain stem glioma were found to have DEBSGs emanating from the pons, pontomedullary junction or medulla. Eleven of the 12 patients had classic juvenile pilocytic astrocytomas. Unlike most other brain stem tumors, these patients were young (median 38 months, range 17-75), had a relatively long duration of symptoms (median 7 months, range 2-24) and displayed signs of increased intracranial pressure with limited cranial nerve paresis, absence of pyramidal tract findings, and near normal brain stem auditory-evoked potentials. MRI characteristically showed sharply demarcated lesions with decreased signal intensity on T1, and increased intensity on T2 sequences. Except for cystic areas, these tumors showed bright, uniform enhancement after gadolinium-DTPA. In all patients, 50-100% of the tumor volume could be resected. Three of 10 patients who received no immediate postoperative treatment eventually demonstrated disease progression, and 2 patients with subtotal resections who were treated with radiation and/or chemotherapy postoperatively remain disease-free for extended periods of time. The only death occurred in the 1 patient treated with chemotherapy who died of secondary leukemia. The overall and progression-free survival of these patients at 2 years is 100 and 67% as compared to 18 and 21%, respectively, for other concomitantly treated nonexophytic brain stem gliomas.2+ the ability to achieve significant degrees of resection.

A BRW Stereotaxic Biopsy of Brain Stem Glioma

Histopathological diagnosis of brain stem glioma should be performed for the purpose of the determination of its management and clinical course, but its surgical biopsy has been followed by high mortality and morbidity. We performed the tissue sampling for histological examination with BRW stereotaxic system under local anesthesia successfully.

5-Fluorouracil and 1-(2-chloroethyl)-3-cyclohexyl-1-nitrosourea (CCNU) followed by hydroxyurea, misonidazole, and irradiation for brain stem gliomas

Twenty-eight evaluable children with the diagnosis of brain stem glioma were treated with 5-fluorouracil and CCNU before posterior fossa irradiation (5500 rads); during irradiation, the children received hydroxyurea and misonidazole, The treatment was well tolerated, and minimal toxicity was produced. The median relapse-free survival was 32 weeks, and the median survival was 44 weeks. Analysis of covariates showed that, in patients between the ages of 2 and 19 years, survival was longest in the older children (P < 0.02). Tumor histology, sex, extent of operation (if any), Karnofsky score, and radiation dose did not correlate with survival. (Neurosurgery 14:679-681, 1984)

5-Fluorouracil and 1-(2-chloroethyl)-3-cyclohexyl-1-nitrosourea (CCNU) followed by hydroxyurea, misonidazole, and irradiation for brain stem gliomas: a pilot study of the Brain Tumor Research Center and the Childrens Cancer Group.

Twenty-eight evaluable children with the diagnosis of brain stem glioma were treated with 5-fluorouracil and CCNU before posterior fossa irradiation (5500 rads); during irradiation, the children received hydroxyurea and misonidazole. The treatment was well tolerated, and minimal toxicity was produced. The median relapse-free survival was 32 weeks, and the median survival was 44 weeks. Analysis of covariates showed that, in patients between the ages of 2 and 19 years, survival was longest in the older children (P less than 0.02). Tumor histology, sex, extent of operation (if any), Karnofsky score, and radiation dose did not correlate with survival.

Pediatric brain stem tumors: radiographic, pathological, and clinical correlations.

A retrospective analysis of the case histories of 21 pediatric patients (ages, 2.5 to 18 years) with a histologically proven diagnosis of brain stem glioma was performed to determine whether patterns of radiographic appearance could be correlated with pathology. Based on the computed tomographic or pneumoencephalographic appearance of the tumor at the time of clinical diagnosis, tumors were divided into four types: central intrinsic (Type I), central exophytic expansion into the 4th ventricle (Type II), eccentric exophytic expansion not involving the 4th ventricle (Type III), and both eccentric and central exophytic expansion (Type IV). Regardless of the radiographic classification, all patients except one, who harbored a well-differentiated astrocytoma in the area of the pons, had an anaplastic astrocytoma (n = 14) or a glioblastoma multiforme (n = 6). There was no appreciable difference in survival between patients with either tumor histology. The presence of a cystic component did not affect survival. High resolution computed tomographic scans, with reconstructed images of the posterior fossa, can predict the presence and location of brain stem tumors and associated cysts and probably the histological nature of the tumor.

Retromastoid approach for biopsy of brain stem tumors.

Abstract Brain stem tumors constitute about 10 to 20% of all pediatric brain tumors. The clinical manifestations are protean and may be produced by malignant as well as benign lesions. Although the computed tomographic (CT) scan has contributed considerably in the diagnosis of these lesions, it is not possible to differentiate these tumors from viral encephalitis of the brain stem, vascular malformations, and other benign lesions. Over the years, biopsy of brain stem lesions has been considered a low yield and high risk procedure. In one of the recent series, the incidence of nondiagnostic biopsy approached 20% and major deterioration of cranial nerve function also occurred in 20% of the cases. Traditionally, suboccipital craniectomy and biopsy of the floor of the 4th ventricle has been used. This is usually associated with aggravation of cranial nerve deficits. Review of pathological specimens indicates that brain stem gliomas most often involve the pons, with predominantly unilateral involvement, and frequently fill in part of the cerebellopontine angle. On this basis, we have recently used a retromastoid approach on five patients with brain stem tumors to obtain biopsies from the ventrolateral pons and the cerebellopontine angle. A diagnosis was made in each case without complication. Minimal morbidity might be anticipated from this approach, as all cranial nerve nuclei are far away from the biopsy site. The biopsy of brain stem lesions is extremely important because aggressive therapeutic decisions may be made only after a histological diagnosis has been made. In one of our cases, a diagnosis of medulloblastoma was made in a patient with a clinical and CT scan diagnosis of brain stem glioma; this diagnosis changed the treatment plan. The retromastoid approach for the biopsy of brain stem tumors is a safe procedure with minimal morbidity and a high diagnostic yield.

Successful Surgical Treatment of Pontine Vascular Malformation in a 3-Year Old

A 3-year-old male presented with progressive right hemiparesis. Clinical and radiological studies suggested the diagnosis of brain stem glioma. At surgery, a small vascular malformation of the pons was encountered. Although rare, vascular malformations must be considered in the differential diagnosis of brain stem masses.

Computed tomography in the diagnosis of brain stem gliomas

Twenty patients with brain stem gliomas were diagnosed by CT. The diagnosis was established on the basis of characteristic appearance, density pattern and mass effect. Differentiation from brain stem metastasis or angioma is frequently possible based on CT findings.

Role of the radionuclide brain image in the diagnosis of brainstem gliomas.

The noninvasive detection of brainstem gliomas remains difficult. Eleven of our patients with proven brainstem gliomas had radionuclide brain imaging prior to the initiation of therapy or confirmation of the diagnosis; six studies were positive. Pneumoencephalography remains the most reliable diagnostic test for brainstem glioma, and is invariably required for confirmation. Although angiography is useful in the evaluation of vasocularity it may not detect small infiltrating lesions. Radionuclide brain imaging is useful in the initial workup of patients with suspected brainstem gliomas.

The efficacy of the brain scan in diagnosis of brainstem gliomas.

Nineteen cases of brainstem glioma were reviewed to evaluate lesion detection by brain imaging and other radiological methods. 99m-Tc-pertechnetate brain imaging was definitely abnormal in only one patient. Other techniques proved to be more sensitive and specific. Angiograms were abnormal in 10 of 18 cases while pneumoencephalography demonstrated the lesion in 16 of 19 cases. Brain imaging is not a sensitive diagnostic study in patients with suspected brainstem glioma. A normal brain scan is not useful in excluding this diagnosis and further radiological evaluation is necessary.