The term long-term epilepsy associated tumor (LEAT) encompasses lesions identified in patients investigated for long histories (often 2 years or more) of drug-resistant epilepsy. They are generally slowly growing, low grade, cortically based tumors, more often arising in younger age groups and in many cases exhibit neuronal in addition to glial differentiation. Gangliogliomas and dysembryoplastic neuroepithelial tumors predominate in this group. LEATs are further united by cyto-architectural changes that may be present in the adjacent cortex which have some similarities to developmental focal cortical dysplasias (FCD); these are now grouped as FCD type IIIb in the updated International League Against Epilepsy (ILAE) classification. In the majority of cases, surgical treatments are beneficial from both perspectives of managing the seizures and the tumor. However, in a minority, seizures may recur, tumors may show regrowth or recurrence, and rarely undergo anaplastic progression. Predicting and identifying tumors likely to behave less favorably are key objectives of the neuropathologist. With immunohistochemistry and modern molecular pathology, it is becoming increasingly possible to refine diagnostic groups. Despite this, some LEATs remain difficult to classify, particularly tumors with "non-specific" or diffuse growth patterns. Modification of LEAT classification is inevitable with the goal of unifying terminological criteria applied between centers for accurate clinico-pathological-molecular correlative data to emerge. Finally, establishing the epileptogenic components of LEAT, either within the lesion or perilesional cortex, will elucidate the cellular mechanisms of epileptogenesis, which in turn will guide optimal surgical management of these lesions.

Long-term epilepsy-associated tumors.

Aims

Angiocentric glioma (AG) is a rare, slow-growing tumour of the central nervous system. It is often associated with refractory epilepsy and occurs most commonly in children and young adults. We herein report nine cases of AG, including four with atypical histological findings.



Methods

The clinical data and clinicopathological findings of nine cases with AG histological features were described.



Results

All nine patients had a history of refractory epilepsy with a mean history of 4.4 years and a median age of 17.6 years at surgery. The AG lesions were located in the superficial cerebrocortical region. Histological examination of these cases revealed characteristic structural features of AG, including bipolar spindle-shaped cells with an angiocentric growth pattern. However, four cases also exhibited atypical histological features: one had astroblastoma-like characteristics, two had a distinct cystic region with an onion-like structure and myxoid changes, and the other one had a region involving many abnormal neurones reminiscent to ganglioglioma. All were positive for glial fibrillary acidic protein and vimentin. Eight cases were positive for epithelial membrane antigen (EMA), with a dot-like staining pattern. A diffuse D2-40 staining was visible in these cases, with two having similar staining pattern to EMA. All cases were immunonegative for BRAF V600E and isocitrate dehydrogenase-1 R132H mutations.



Conclusions

Our results demonstrate that atypical histological features can be present in AG. A collection of more cases and further molecular analyses are required to confirm our findings.

Angiocentric glioma: a report of nine new cases, including four with atypical histological features.

Angiocentric glioma in a 4-year-old boy: imaging characteristics and review of the literature

We report one case of brain glioma that developed in the scar of an old brain trauma. A 45-year-old man who presented with seizures; MRI showed a large mass in the right temporal region. Surgical biopsy showed a glioblastoma multiforme. The patient had suffered a cranial trauma in a road accident 10 years previously with an intracerebral hematoma in the right temporal region. This case fulfills the established criteria for a traumatic origin of brain tumors and adds further support to the relationship between cranial trauma and the onset of glioma. As stated by other authors, an association between head trauma and brain tumor risk cannot be ruled out and should be studied further.

/pdf/post-traumatic-glioma-report-of-one-case-and-review-of-the-3i495gt2tk.pdf

Post-traumatic glioma: report of one case and review of the literature.

Angiocentric gliomas have recently been reclassified as a separate central nervous system tumor. Few cases have been reported, and most of those correspond to slow-growing, low-grade neoplasms in very young pediatric patients. Here we describe magnetic resonance imaging findings (including diffusion imaging, spectroscopy and tractography) in an unusual higher-grade neoplasm with pathologic features suggestive of an angiocentric glioma in a 15-year-old male. The tumor had mild heterogeneous enhancement on magnetic resonance imaging, and a low apparent diffusion coefficient (9.9 × 10(-4) mm(2)s(-1)), consistent with an intermediate-to-high cellularity tumor. Spectroscopic imaging showed elevated choline/phosphocreatine and choline/N-acetyl aspartate ratios, suggesting an unusually aggressive tumor. We conclude that angiocentric glioma should not be excluded from consideration at primary diagnosis, particularly in teenaged patients nearing adulthood.

http://www.radiologycases.com/index.php/radiologycases/article/download/1134/pdf

Imaging characteristics of an unusual, high-grade angiocentric glioma: a case report and review of the literature.

Angiocentric glioma with rich blood supply.

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Angiocentric glioma with rich blood supply.