Optic glioma in children: a retrospective analysis of 101 cases.

AIM: To evaluate the clinical characteristics and long-term outcome of pediatric patients with optic glioma. PATIENTS AND METHODS: A total of 101 patients with optic glioma newly diagnosed between 1975 and 2008 were evaluated retrospectively. COPP (cyclophosphamide, vincristine, procarbazine, prednisolone) and cisplatin plus etoposide were the most commonly used chemotherapy regimens. Radiotherapy was administered in patients with progressive or unresponsive disease. RESULTS: The median age at the time of diagnosis was 6 years, and the male/female ratio was 1.15. The most common referral complaint was strabismus. The most common site of optic glioma was the hypothalamic-chiasmatic region (31.7%). Fifty-three patients (52.5%) had neurofibromatosis type 1 (NF-1). Treatment consisted of surgery, radiotherapy, and chemotherapy. Forty-nine patients (48.5%) underwent surgery, which was predominantly subtotal resection, radiotherapy was administered to 39.4%, and 30 patients received chemotherapy. The 5-year progression-free survival (PFS) and overall survival (OS) rates were 65.8% and 88.4%, respectively, and the 10-year PFS and OS were 54.2% and 83.4%, respectively, with an 8-year median follow-up. OS was significantly lower in patients with hypothalamo-chiasmatic involvement and significantly higher in patients with NF-1. The 5- and 10-year PFS rates were significantly higher in patients 10 years or older at diagnosis (P=0.0001) and in patients with intraorbital involvement (P=0.032). Eighteen patients (17.8%) died of disease. CONCLUSIONS: Patients with NF-l and those older than 10 years have a better prognosis, whereas patients younger than 3 years and those with hypothalamic-chiasmatic optic glioma have a worse outcome. Further studies are needed to find appropriate treatment strategies.

Complications of endoscopic surgery of the pituitary adenomas: analysis of 570 patients and review of the literature.

Endoscopic transsphenoidal surgery is emerging as a minimally invasive and maximally effective procedure for pituitary adenomas. In this report we analyzed the complications in 624 procedures of endonasal transsphenoidal endoscopic surgery in the treatment of 570 patients with pituitary adenomas. The leading author (MB) operated pituitary adenomas via pure endoscopic endonasal transsphenoidal surgery between January 2006 and August 2011 at the Hacettepe University, Department of Neurosurgery in Ankara. Complications were assessed in 624 surgical procedures under five groups; rhinological, CSF leaks, infection, vascular and endocrinologic complications. We observed a total of 76 complications (12.1%). Rhinological complications occurred in 8 patients (1.3%): 4 epistaxis (0.6%) and 4 hyposmia (0.6%). Postoperative CSF leaks occurred in 8 patients (1.3%), and infectious complications occurred in 8 patients: 3 cases of sphenoidal sinusitis (0.4%), 5 cases of meningitis (0.8%). Only 1 case of internal carotid aneurysm rupture during the opening of sellar floor (0.16%) was observed. Endocrinologic complications occurred in 51 (8.1%) patients: Anterior pituitary deficiency in 12 (1.9%), transient diabetes insipidus (DI) in 29 (4.6%), permanent DI in 3 (0.4%) and inappropriate antidiuretic hormone secretion syndrome occurred in 7 (1.1%). There was no mortality directly related to the surgical procedure. The complication rates observed in our study suggests that the endoscopic pituitary surgery is at least as safe as microscopic transphenoidal surgery. These rates were obtained with due experience and well-coordinated teamwork. To further improve these rates, new technological developments will be helpful.

Is diffusion-weighted imaging useful in grading and differentiating histopathological subtypes of meningiomas?

PURPOSE: Meningiomas are mostly benign, however atypical or malignant subtypes with more aggressive clinical course and higher recurrence rates can also be seen. The purpose of this study was to determine whether histopathological subtypes of meningiomas could be assessed preoperatively using apparent diffusion coefficient (ADC) values. MATERIALS AND METHODS: Conventional magnetic resonance (MR) and diffusion-weighted (DW) imaging of 177 adult patients with pathologically proven meningiomas were retrospectively evaluated. Tumor size and the degree of associated edema were noted. The signal intensity of the lesions on DW imaging was evaluated and graded. Mean ADC values were obtained as the mean of measurements from three regions of interests within the mass. ADC ratios of meningioma/contralateral normal appearing subcortical parietal white matter were also calculated. RESULTS: The histopathological analysis revealed 135 benign, 37 atypical and 5 malignant lesions. With classification according to the subtype, the mean ADC values and ratios of benign meningiomas were as 0.99±0.12×10(-3) mm(2)/s and 1.22±0.07, respectively. ADC values for atypical and malignant groups were both 0.84±0.1×10(-3) mm(2)/s. The ADC ratios were 1.05±0.1 and 0.96±0.2 for atypical and malignant subtypes, respectively. There was no statistically significant difference between the mean ADC ratios of the three subtypes (ANOVA test; P≥0.05). Gender, age of the patients and tumor size showed no statistically significant difference between the different histological groups. CONCLUSION: DW MR imaging was not found to have any additional value in determining histological behaviour nor in differentiating histopathological subtypes of meningiomas.

Amyloid goiter and hypopituitarism in a patient with systemic amyloidosis.

Systemic amyloidosis may infiltrate the thyroid or other endocrine glands but rarely causes endocrine dysfunction. We describe a 45 years old female patient with diffusely enlarged goiter and hypopituitarism secondary to amyloid infiltration of the thyroid gland and possibly pituitary gland, respectively. She was on chronic haemodialysis for 3 years due to systemic amyloidosis. While she was being prepared for thyroidectomy, adrenal failure developed. Her anterior pituitary hormone levels were low and magnetic resonance imaging of the hypophysis showed low signal intensity in right part of the adenohypophysis. She improved with corticosteroid replacement therapy and underwent subtotal thyroidectomy without any complication. Histopathologically, amyloid deposition was demonstrated in the thyroid gland. To our knowledge, this is the first case with amyloid goiter and hypopituitarism secondary to systemic amyloidosis. Amyloid infiltration should be considered in a systemic amyloidosis patient presenting with rapidly enlarged thyroid gland and signs of hypopituitarism.

Parenchymal lymphoma of the brain on initial MR imaging: a comparative study between primary and secondary brain lymphoma.

BACKGROUND AND PURPOSE: Parenchymal lymphomatous brain masses have not been investigated considering if they are primary or as a part of systemic lymphoma (secondary) on imaging studies previously. We aimed to determine characteristics of the secondary parenchymal lymphomatous involvement of the brain and to find if there is any radiologic feature to help discrimination of untreated primary and secondary central nervous system lymphoma on patients' initial magnetic resonance imaging. MATERIALS AND METHODS: We evaluated MR images of 18 patients with the diagnosis of primary (n=12) and secondary central nervous system lymphoma (n=6). We considered the number, localization, enhancement pattern, signal characteristics, diffusion properties, presence of hemorrhage and presence of butterfly pattern on MR imaging at initial presentation. RESULTS: Secondary central nervous system lymphomas predominantly presented as multiple (n=4, 66.7%) lesions. Homogenous nodular enhancement and supratentorial white matter involvement were present in all patients with butterfly pattern and infiltrative/perivenular enhancement in half (n=3) of the patients. Deep gray matter (n=1, 16.7%) and infratentorial involvement (n=1, 16.7%) were scarce and no ring enhancement was observed. There was no statistically significant difference in any of the investigated MR features between the two groups. CONCLUSION: Statistical analyses revealed no significant distinctive radiologic characteristics between primary and secondary lymphoma of the brain parenchyma.

Spinal low-grade neoplasm with leptomeningeal dissemination mimicking tuberculous meningitis in a child.

Spinal seeding of primary malignant intracranial tumors via CSF is common. However, this is rare in low-grade glial tumors. Cranial leptomeningeal metastasis of primary spinal cord low-grade gliomas at diagnosis or relapse is extremely rare. Leptomeningeal metastasis of spinal cord low-grade tumors may mimic tuberculous meningitis in children. A patient with primary spinal cord low-grade neoplasm mimicked tuberculous meningitis is presented. The patient successfully treated with chemoradiotherapy. At the end of 19-month follow-up, diffuse leptomeningeal infiltration and a dural mass compatible with relapse developed. Chemoradiotherapy was started.

Prognostic significance of tumor volume in nasopharyngeal carcinoma.

OBJECTIVE: To investigate the prognostic significance of primary and residual tumor volume in nasopharyngeal carcinoma. METHODS: 56 patients were included in the study. Diameters of tumors were measured from CT and MR film hardcopies. Diameter-based measurements were computed as an ellipsoid (V=4/3·π·d1·d2·d3) to calculate diameter-based volume. It was investigated whether primary tumor volume provided prognostic information about local regional recurrence free survival (LRRFS), disease free survival (DFS), distant metastasis free survival (DMFS) and overall survival (OS) by monovariant and multivariant analysis. Kaplan-Meier survival analysis method and log-rank test were used to estimate survival analysis (95% confidence interval). Cox regression test was used for two variant and multivariant survival analysis. Statistical Package for Social Sciences (SSSP) 10.0 for Windows programme was used for data analysis. RESULTS: In the multivariate analysis, in the patients with tumor volume more than 60ml, local regional recurrence more frequently developed. The relationship between tumor volume and local regional recurrence was found significant (p=0.053). In the monovariate analysis, primary tumor volume was found to be a significant predictive value on DFS and DMFS. In the patients with tumor volume below 20ml, DFS was 60%, whereas in the patients with tumor volume above 60ml, DFS was 0% (p=0.007). The prevalence were 68% and 0% in the patients group that had tumor volume below 30ml and above 60ml respectively. DMSF ratios in the patients with primary tumor volume below 20ml and above 60ml were 86.67% and 33.3% respectively. The residual tumor volume (RTV) at first control after treatment was found to be a significant prognostic factor on LRRFS (p=0.03). CONCLUSION: The foundation of new T staging systems that consists of PTV that was found as an independent prognostic factor alone in multivariate statistical analysis may precede better prediction of prognosis and more appropriate treatment of patients having different prognostic factors. RTV in the first control after treatment was a significant prognostic factor on LRRFS.

Pineal cyst apoplexy: report of an unusual case managed conservatively.

Pineal cyst apoplexy is a very rare entity with previously reported symptoms of severe frontal or occipital headache, gaze paresis and visual field defects, nausea or vomiting, syncope, ataxia, hearing loss and sudden death. The treatment options for symptomatic pineal cysts are observation, shunting, aspiration via stereotactic guidance or endoscopy, third ventriculostomy, ventriculocysternostomy, and/or surgical resection by craniotomy and microsurgery. Here, the authors report an unusual case of a 28-year-old male patient with pineal cyst apoplexy, presenting with headache, insomnia, and sexual dysfunction symptoms who is being managed conservatively and observed for two years by an academic tertiary care unit.

Imaging features of CNS involvement in AIDS.

Patients with acquired immunodeficiency syndrome (AIDS) are the subjects of a large part of routine neuroradiological work in the Western world currently. The World Health Organization announced that Turkish authorities had reported a cumulative total of 2544 HIV cases from 1985 to 2006, of whom 623 had developed AIDS and 140 had died. It is estimated that approximately one-third of AIDS patients develop neurological complications. The spectrum of diseases affecting the central nervous system (CNS) in AIDS patients comprises predominantly opportunistic infections and primary CNS lymphoma. Although to a lesser degree when compared with Western countries, the incidence of AIDS and related neurological diseases are on the rise also in Turkey. Therefore radiologists should recognize HIV-associated problems and their imaging features. In this review, we focus on imaging features of more common CNS diseases in HIV-seropositive patients. This essay has been prepared using radiological studies of the patients who had been managed in our hospital which is a tertiary care center with a highly motivated medical team for this peculiar disease in the years between 2002 and 2008.

Structural abnormalities of the brain other than molar tooth sign in Joubert syndrome-related disorders.

PURPOSE: We performed a retrospective study in which we investigated malformations other than brainstem and vermian dysgenesis in Joubert syndrome-related disorders (JSRD). We investigated the frequency and type of structural abnormalities that coexist with the molar tooth sign (MTS) in JSRD. MATERIALS AND METHODS: We searched our archive for the years 2002-2008 in order to find patients with the diagnosis of JSRD. Cranial magnetic resonance imaging studies of 20 patients with the diagnosis of JSRD were reviewed by two neuroradiologists. RESULTS: In addition to known anomalies including callosal dysgenesis, heterotopia, polymicrogyria, atretic encephalocele, hypomyelination, and nonobstructive dilatation of lateral ventricles; malformations that have not been previously reported were determined, including cerebellar folial disorganization, hippocampal malformation, temporal lob hypoplasia, ambient cistern lipoma, and parenchymal cyst. CONCLUSION: Structural abnormalities associated with the MTS are not rare, and the additional imaging findings may help explain the neurological presentation in these patients.

The effect of clozapine on regional cerebral blood flow and brain metabolite ratios in schizophrenia: relationship with treatment response.

The purpose of this study was to investigate the effect of clozapine on regional cerebral blood flow (rCBF) and its relationship with response to treatment. In addition, we aimed to study the influence of clozapine on proton magnetic resonance spectroscopy ((1)H-MRS) findings in the dorsolateral prefrontal cortex (DLPFC) in a subgroup of patients. Psychopathology, neurocognitive functioning, and SPECT imaging of 22 patients were assessed at the baseline and 8 weeks after the initiation of clozapine treatment. In 10 of these patients intermediate-echo (TE: 135 ms) single-voxel (1)H-MRS was also performed at the baseline and after 8 weeks. Clozapine treatment increased the right frontal (superior and medial)/caudate perfusion ratio in the whole group, while it increased bilateral frontal (superior and medial)/caudate perfusion ratios in treatment responders. In addition, percentage changes in left and right frontal (superior and medial)/caudate perfusion ratios compared to the baseline were higher in treatment responders than in non-responders. The improvement in attention was related to the increase in percentage change in the right frontal (superior and medial)/caudate perfusion ratio, while the improvement in verbal fluency was related to the increase in percentage changes in both right and left frontal (superior and medial)/caudate perfusion ratios and to right frontal (superior and medial)/thalamus perfusion. Baseline frontal (superior and medial)/thalamus perfusion could explain 32% of the variability of percentage improvements in psychopathology. (1)H-MRS showed that the baseline PANSS general psychopathology score was inversely correlated with the baseline NAA/Cre ratio. An increased NAA/Cre ratio in DLPFC after 8 weeks of clozapine treatment was also revealed by (1)H-MRS. Our SPECT imaging results suggest the presence of an imbalance in fronto-striato-thalamic circuitry that changes with clozapine, especially in the responders, while (1)H-MRS results indicate a supportive effect of clozapine on neuronal integrity.

Intravascular lymphoma masquerading as multiembolic stroke developing after coronary artery by-pass surgery.

BACKGROUND: Intravascular lymphoma (IVL) is a very rare non-Hodgkin type lymphoproliferative disorder characterized by neoplastic growth of lymphoid cells within the lumen of capillaries, small veins, and arterioles. The neoplastic cells cannot reach the parenchyma because of the loss of adhesion molecules during malignant transformation. Multifocal vascular occlusions caused by proliferation of malignant lymphocytes in the lumen result in diffuse thrombosis and tissue infarction. The clinical symptoms of the disease are dependent on the specific organ involvement which most often includes the central nervous system and skin. Neurologic presentation includes focal sensory or motor deficits, altered sensorium, rapidly progressive dementia, seizures, ataxia, and vertigo. CASE REPORT: We report a patient with IVL whose symptoms developed on the second postoperative day of coronary artery-bypass surgery imitating a multiembolic stroke. Magnetic resonance imaging showed widespread ischemic subcortical lesions. The patient's clinical status worsened irrespective of supportive medical treatment. The diagnosis was established by autopsy. CONCLUSION: IVL may mimic ischemic stroke. IVL is not often diagnosed before death because of the intravascular growth pattern of the tumor cells and a fulminant clinical course. IVL may be considered in the differential diagnosis of ischemic stroke patients with progressive worsening despite medical management.

Intraventricular dysembryoplastic neuroepithelial tumor-like neoplasm with disseminated spinal tumor.

Dysembryoplastic neuroepithelial tumor (DNT)- like lesions arise in extracortical locations and behave in a benign fashion similar to that of cortical DNTs. They usually come to attention by symptoms and signs of increased intracranial pressure rather than focal neurological impairment. Here we report a case of 9-year-old boy with a complaint of headache and back pain. A third ventricular mass lesion with disseminated spinal tumor was detected on his magnetic resonance imaging. The presence of floating neurons in a mucinous matrix, oligodendrocyte-like cells (OLCs) aligning axonal columns and vessels, immunohistochemical profile of the neoplasm in addition to the clinical and radiological manifestations of the patient led to the diagnosis of "DNT-like neoplasm of the third ventricle".

Dynamic contrast-enhanced susceptibility-weighted perfusion imaging of intracranial tumors: a study using a 3T MR scanner.

PURPOSE: To determine whether there are statistically significant differences in cerebral blood volume (CBV) and cerebral blood flow (CBF) of brain tumors of different histopathologic types including primary and secondary benign and malignant lesions. To determine whether these measurements relate to tumor grade. MATERIALS AND METHODS: Forty-five patients with brain tumors, age 2 to 79 years, underwent dynamic contrast-enhanced susceptibility- weighted echo-planar perfusion magnetic resonance imaging (MRI) using a 3T MR scanner. The lesions were evaluated by measurements of relative cerebral blood volume (rCBV) and relative cerebral blood flow (rCBF). The Mann-Whitney U test was used to compare rCBV and rCBF measurements of tumor groups -- 13 low-grade and 13 high-grade neuroepithelial (NE) tumors, five metastases, 10 meningiomas, and four others. Peritumoral rCBV and rCBF measurements of high grade NE tumors and metastases were also compared. The relationship between rCBV and rCBF measurements was evaluated by Spearman rank correlation. RESULTS: Measurements of rCBV and rCBF were statistically significantly higher (P < 0.05) in high-grade NE tumors than in low-grade NE tumors. The difference was not statistically significant in comparing high-grade NE tumors with metastases and meningiomas. Peritumoral rCBV of high-grade NE tumors was significantly higher than peritumoral rCBV of metastases (P < 0.05). There was a strong correlation between rCBV and rCBF values. CONCLUSION: CBV and CBF measurements provided by 3T perfusion MRI can help to predict NE tumor grading preoperatively, and differentiate between primary brain tumors and metastases.

Surgery for epilepsy in children with dysembryoplastic neuroepithelial tumor: clinical spectrum, seizure outcome, neuroradiology, and pathology.

INTRODUCTION: Dysembryoplastic neuroepithelial tumors (DNTs) were first described by Daumas-Duport et al. in 1988 as a typically cortical tumor affecting young patients with long-standing, drug-resistant epilepsy. METHODS: We reviewed the medical records of 29 patients with DNT between 1994 and 2007 at Hacettepe University Children's Hospital retrospectively; age at the time of surgery, age at seizure onset, electroencephalography (EEG), MRI, medical treatment, surgical procedure, seizure outcome, and pathological findings were documented. RESULTS: Male to female ratio was 15/14. Age at the time of evaluation ranged 4-24 years. Twenty-seven patients (93.1%) had complex partial seizures, one (3.44%) had simple partial seizures, and one patient had generalized seizures. Preop interictal EEG showed epileptiform discharges in 24 patients, while in five patients interictal EEG before surgery showed no epileptiform discharges. Pathologically, 24 of our patients were classified as complex type and five as simple type. MRI showed temporal lesion in 20 (68.9%) patients and nine patients had extratemporal DNT. We choose the type of surgery according to lesion and the epileptojenic zone. Finally, 27 patients had Engel Class IA and two patients had Engel Class IB outcome. CONCLUSION: Complete resection of the lesion with epileptojenic zone is important for seizure-free outcome. Timing of surgery, extent of surgery, and stopping antiepileptic drugs are still important factors.

Radiological evaluation of patients with pituitary langerhans cell histiocytosis at diagnosis and at follow-up.

This study evaluated pituitary imaging findings in 13 patients with Langerhans cell histiocytosis (LCH) with diabetes insipidus. Nine patients were evaluated with pituitary magnetic resonance imaging (MRI), 3 with brain computed tomography, and 1 with brain MRI. The infundibulum was thickened in 11 (84.6%) patients, thread-like in 1 (7.7%), and normal in 1 (7.7%). Posterior pituitary intensity was absent in 10 patients (76.9%); in 4 patients, the pituitary gland was small in size, and 2 patients had atrophic pituitary. Three had a small sella. Infundibular thickening and absence of posterior pituitary intensity were the most common radiological findings. MRI imaging should be used to follow up patients with pituitary histiocytosis, and patients with LCH and diabetes insipidus should be followed for pituitary atrophy.

Hyperhomocysteinemia due to pernicious anemia leading to pulmonary thromboembolism in a heterozygous mutation carrier.

Pulmonary thromboembolism is a life-threatening condition resulting mostly from lower extremity deep-vein or pelvic-vein thrombosis. A 46-year-old woman was admitted to hospital with pain on the right side of the chest and hemoptysis. On laboratory analysis, D-dimer level was elevated. Computed tomographic pulmonary angiography revealed intravascular filling defects due to thrombi in right lower lobe pulmonary segmental arteries. Screening for thrombophilic states was normal except for heterozygous mutations of both prothrombin and methylene tetrahydrofolate reductase (MTHFR 677) genes. Homocysteine level was high, and vitamin B12 level and serum ferritin level were reduced. Serum antiparietal antibody was positive, and therefore, pernicious anemia was diagnosed along with iron-deficiency anemia. After the diagnoses were established, enoxaparin followed by warfarin was started in addition to oral vitamin B12, pyridoxine, thiamine, folic acid, and ferroglycine sulfate supplementation. At the end of 8 weeks of the replacement therapy, vitamin B12, folate, and homocysteine levels and red cell volume were found to be normal, with complete resolution of the thrombus confirmed by repeat computed tomographic pulmonary angiography. We conclude that hyperhomocysteinemia due to vitamin B12 deficiency associated with pernicious anemia might have decreased the threshold for thrombosis. In addition, the presence of heterozygous prothrombin and methylene tetrahydrofolate reductase mutations might serve as synergistic cofactors triggering pulmonary thromboembolism.