Angiocentric glioma: Report of a rare case presenting with psychosis (original) (raw)
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Angiocentric glioma: a rare intractable epilepsy-related tumour in children
Folia Neuropathologica, 2014
Angiocentric glioma is a low-grade tumour that occurs in children and young adults with a long-standing epilepsy. The typical histopathological features of this tumour is the presence of spindle-shaped cells, radially oriented around the cortical blood vessels. We present two teenage cases of the angiocentric variant of glioma: 1) a 15-year-old girl with a chronic and intractable partial epilepsy with cystic tumour located in the right temporal lobe and 2) a 14-year-old boy with intractable seizures and an extensive cortical lesion in the left parieto-occipital area. In both cases, the total tumours excision was performed. The histopathological findings revealed a characteristic angiocentric pattern that was composed of elongated cells arranging in pseudorosette-like structures around blood vessels. Moreover, schwannoma-like areas and subpial neoplastic infiltration with palisading of tumour cells at the brain surface were seen. The neoplastic cells displayed immunoreactivity for GFAP, S-100 protein and vimentin. A slight "dot-like" EMA staining, suggesting ependymal differentiation, was detected. The clinical and pathological picture allowed to establish the diagnosis of angiocentric gliomas. The patients were discharged home in a good condition and without seizures. During the 4-year follow-up, the tumour recurrence and seizures were not observed. The appropriate diagnosis of this peculiar type of usually low-grade glial tumour is important for adequate and successful treatment. The differential diagnosis requires the exclusion of other tumours with an angiocentric pattern, i.e. ependymoma, astroblastoma, which are associated with more aggressive biology.
A b s t r a c t Angiocentric glioma (AG) is a newly-classified, very rare, WHO grade I central nervous system (CNS) lesion, occurring usually in children and young adults. Only 52 patients with AG have been reported so far, making it one of the rarest neuropathological entities. Hereby we present two new cases of AG in young subjects with detailed neuropatholog-ical investigations and a neuroradiological picture along with a brief summary of all already published literature reports of this tumor. Histopathological examination of the resected tissue from both cases revealed similar changes characteristic of AG. The tumors were composed of spindle-like, elongated cells, forming characteristic pseudorosettes around vessels and diffusively infiltrating surrounding tissue, trapping neurons between tumor cells. Noticeably, some neoplastic cells encrusting vessels extended far beyond the main tumor mass. Hypothetically, this may be responsible for the recurrence of the tumor even in the case of apparently total excision. In immunohistochemistry, AG cells were glial fibril-lary acidic protein (GFAP) and vimentin positive, also exhibiting a strikingly significant epithelial membrane antigen (EMA) dot-like staining pattern. In one of the cases, electron microscopy revealed ependymal differentiation features such as microvilli and cilia. Taken together, all these data strongly confirm a dual astroglial-ependymal nature of the tumor. Follow up corroborates benign character of this neoplasm. Both AGs reported here were immunonegative for the product of the mutated IDH-1 gene what, according to our best knowledge, has never been reported so far. It may suggest that in their pathogenesis AGs differ from grade II astrocytomas, which in most cases harbor a mutation of IDH-1. Noteworthy, neuroimaging in our cases was relatively characteristic but not conclusive, therefore biopsy (at least) is mandatory. A newly proposed so called " A-B-C " classification of long-term epilepsy-associated tumors (LEATs) places AG in a category named ANET. The authors shortly review the A-B-C classification of LEATs.
Neurologia medico-chirurgica, 2011
A 26-year-old man presented with a case of angiocentric glioma manifesting as medically refractory epilepsy. Magnetic resonance imaging revealed a hyperintense lesion in the right superior frontal gyrus on T(2)-weighted imaging, with cortical hyperintense rim on T(1)-weighted images and minimum contrast enhancement. Video-electroencephalography (EEG) monitoring characterized his seizures as originating from the right frontal lobe. Long-term EEG recording from implanted subdural electrodes disclosed epileptic activities extending beyond the margin of the radiological lesion. Extended cortical resection of the superior frontal gyrus including the tumor and the surrounding epileptic cortices was performed. Postoperatively, he became seizure-free with antiepileptic medication during a 12-month follow-up period. Histological examination of the surgical specimen showed the characteristic findings of angiocentric glioma. Associated cortical dyslamination consistent with cortical dysplasia ...
Journal of Neuropathology and Experimental Neurology, 2005
We present 8 examples of a neoplasm with features of both astrocytoma and ependymoma that may represent a distinct clinicopathologic entity. The cerebral hemispheric tumors occurred in patients that were 3, 4, 12, 14, 15, 26, 30, and 37 years of age. All presented with seizures that, with the exception of 2, began in childhood. Magnetic resonance imaging studies showed ill-defined, T2-hyperintense, generally noncontrast-enhancing lesions that, although centered on the cortex or amygdala, extended into the underlying white matter for a short distance. Histologically, the variably infiltrative tumors were distinctively angiocentric with welldeveloped perivascular pseudorosettes in some cases. Longitudinal and/or circumferential orientations of perivascular cells were common also. The cells were uniform in their cytologic features from case to case and were bipolar in all but one case. A glial nature was inferred from immunoreactivity for GFAP, and ependymal differentiation was suggested by positivity for EMA in three cases and ultrastructural features in one. Overall, the tumors were biologically indolent except for one that recurred and ultimately proved fatal.
2014
Psychotic manifestation as an initial presentation of brain tumor is a rare manifestation of the primary disease. A 32-year-old male patient presented with auditory and visual hallucinations, delusion of persecution and profound agitation. The patient was initially suspected as a case of acute psychosis; however, on imaging work-up, a large central space-occupying lesion (SOL) in the brain was detected. Following surgical removal of the brain tumor, psychotic manifestations disappeared. The pathology revealed astrocytoma grade III. Similar presentation was observed in a 28-year-old woman who first visited the psychiatry department. Imaging revealed an SOL in the right parietal lobe. She underwent craniotomy and gross total resection of the tumor. The histopathology of the surgical specimen revealed astrocytoma grade II. Neuropsychiatric manifestations may have a large impact on the quality of life of patients with primary brain tumors and should therefore be adequately managed.
2014
Psychotic manifestation as an initial presentation of brain tumor is a rare manifestation of the primary disease. A 32-year-old male patient presented with auditory and visual hallucinations, delusion of persecution and profound agitation. The patient was initially suspected as a case of acute psychosis; however, on imaging work-up, a large central space-occupying lesion (SOL) in the brain was detected. Following surgical removal of the brain tumor, psychotic manifestations disappeared. The pathology revealed astrocytoma grade III. Similar presentation was observed in a 28-year-old woman who first visited the psychiatry department. Imaging revealed an SOL in the right parietal lobe. She underwent craniotomy and gross total resection of the tumor. The histopathology of the surgical specimen revealed astrocytoma grade II. Neuropsychiatric manifestations may have a large impact on the quality of life of patients with primary brain tumors and should therefore be adequately managed.
Angiocentric glioma: the infiltrative glioma with ependymal differentiation
Turkish Journal of Pathology, 2013
Angiocentric glioma is an epileptogenic, infiltrative, low grade glial tumor, with ependymal and astrocytic differentiation, most commonly seen in young adults and the pediatric age group. Herein we report a case of 21-year-old male patient who presented with fever and pharmaco-resistant seizures. Computed tomography revealed an iso-dense mass lesion in the gyrus rectus of the left frontal lobe. On magnetic resonance imaging the mass was hyperintense on both T1-and T2-weighted images with no contrast enhancement. Histopathological examination revealed monomorphous tumor cells diffusely infiltrating the neuropil with circumferential, radial, or longitudinal angiocentric alignment and subpial aggregation with perpendicular alignment of the cells to the pial surface. Among central nervous system tumors with ependymal differentiation, this distinct entity is the one with an infiltrating growth pattern. In spite of the infiltrating pattern, it does not seem to have a potential for aggressive behavior.
The treatment of angiocentric glioma: case report and literature review
The Permanente journal, 2013
Angiocentric glioma is a recently described tumor recognized since 2007 by the World Health Organization Classification of Tumours of the Central Nervous System. We present the only case of angiocentric glioma at our institution in the last 15 years and review the literature in an attempt to establish prognostic parameters. Our search revealed only 27 cases of angiocentric glioma in the literature. The most common presenting symptom of angiocentric glioma was seizures. Gross total resection of the lesion was curative, without need for radiation or chemotherapy.