Brain surface spindle cell glioma in a patient with medically intractable partial epilepsy: A variant of monomorphous angiocentric glioma?

Abstract

We report a case of brain surface angiocentric glioma in a 6-year-old Japanese boy with medically intractable partial epilepsy. MRI showed somewhat ill-defined high-signal lesions on fluid-attenuated inversion recovery and T2-weighted imagings of the right occipitoparietal cortex. At surgery, a yellowish tumor was localized in the superficial cortex. Histologically, the tumor was predominantly composed of elongated astrocytic cells forming rings around blood vessels. Tumor cells circumferential to vessels predominanted in low cellurarity areas, whereas radial alignment with perivascular pseudorosettes was observed in more cellular regions. These perivascular pseudorosettes closely resembled those of ependymoma. The tumor cells showed variable cytoplasmic immunoreactivity with GFAP. These findings were more likely monomorphous angiocentric glioma, which was first described by Wang et al. in 2005. However, in our case the tumor had a small foci of polymorphous appearance and a comparatively high MIB-1 labeling index (8%). Therefore, the present case may be an atypical form of monomorphous angiocentric glioma. However, no de novo anaplastic monomorphous angiocentric glioma similar to our case has yet been reported in the literature. It remains to be determined whether the behavior of monomorphous angiocentric glioma is an example of benign biological characteristics or whether it more closely resembles a low-grade malignant tumor.

Angiocentric glioma: the infiltrative glioma with ependymal differentiation

“…. The total number of reported cases in the English literature is currently still less than 50 (2,(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21). It is most commonly encountered in early adulthood or childhood but a few late onset cases have also been reported (12,(13)(14)(15)(16)(17)(18)(19)(20)(21).…”

“…It is most commonly seen supratentorially (2,4). The tumoral cells reveal both astrocytic and ependymal differentiation shown either by immunohistochemistry or electron microscopy (Em) analysis (1,5). Since it is a rarely seen tumor, we present a case of AG (ICD-O 9431/1, WHO Grade I) with the review of the literature.…”

“…One case in the midbrain (20) and one in the thalamus (11) have also been reported. The clinical presentation is with long-standing intractable epileptic seizures (5,19,22). Seizures are completely cured by surgical excision.…”

Angiocentric Glioma and Surrounding Cortical Dysplasia Manifesting as Intractable Frontal Lobe Epilepsy-Case Report-

“…[1][2][3][4][5][6][8][9][10][11] The defining histological features of this tumor are the striking perivascular pattern of growth, schwannoma-like palisade arrangement of tumor cells, and characteristic``dot-like'' EMA staining of microlumens of the tumor, suggesting ependymal differentiation in part. The neuroimaging features include solid T 2 -weighted hyperintense lesion in the superficial cerebrum with no contrast enhancement.…”

“…Since then, a total of 36 cases have been reported. [1][2][3][4][5][6][8][9][10][11] Angiocentric glioma is a slow-growing supratentorial tumor with characteristic radiological features, frequently manifesting as recurrent seizures in children and young adults. Histologically, the tumor is characterized by a perivascular pattern of growth and dotlike staining of microlumens of tumor cells with epithelial membrane antigen (EMA).…”