Neuronal Tumors of the Central Nervous System: Radiologic Findings and Pathologic Correlation

Abstract

Pure neuronal and mixed neuronal-glial tumors of the central nervous system are uncommon but fascinating because they are less aggressive than the more common glial tumors and their prognosis is excellent. Neurologic manifestations are varied and include seizures, symptoms of increased intracranial pressure, and neurologic deficits according to tumor location. Many neuronal tumors of the central nervous system demonstrate characteristic radiologic findings. At magnetic resonance (MR) imaging, gangliocytomas demonstrate low signal intensity on T1-weighted images, high signal intensity on T2-weighted images, and frequent enhancement on gadolinium-enhanced T1-weighted images. Characteristic MR imaging findings of Lhermitte-Duclos disease are a nonenhancing mass in a cerebellar hemisphere with a striated pattern. Central neurocytomas are typically located in the lateral ventricles near the foramen of Monro with a characteristic attachment to the septum pellucidum. Ganglioneurocytoma is a rare variant of central neurocytoma that is characterized by differentiation toward ganglion cells. In ganglioglioma, a well-defined cystic mass with a solid mural nodule is typically seen. Extension of enhancement to the leptomeninges is characteristic of desmoplastic infantile ganglioglioma and correlates with the firm dural attachment of the solid component. Dysembryoplastic neuroepithelial tumor has a well-demarcated, multilobulated or gyriform appearance.

Pleomorphic Xanthoastrocytoma of Childhood: MR Imaging and Diffusion MR Imaging Features

“…Inner table scalloping is a feature of slow-growing superficial brain tumors of childhood, such as ganglioglioma and dysembryoplastic neuroepithelial tumor. 17 Whereas Crespo-Rodríguez et al 10 reported inner table remodeling in 3 of 6 patients with PXA younger than 12 years, in the study by Yu et al, 16 only 1 of the 19 patients (both children and adults with an age range of 8 -70 years) demonstrated calvarial remodeling.…”

Hamartomas from head to toe: an imaging overview

“…Most cases occur in adults, usually in the third and fourth decades, with no gender predilection. 13 Pathogenesis remains uncertain: there may be a hamartomatous, neoplastic or congenital origin. However, association with Cowden syndrome suggests that it could fit the hamartoma-neoplasia spectrum of disease.…”

“…However, association with Cowden syndrome suggests that it could fit the hamartoma-neoplasia spectrum of disease. 13,14 Patients may be asymptomatic or present with symptoms secondary to mass affect, such as headache, ataxia, cranial nerve palsy, vertigo or cognitive impairment. 15 CT demonstrates a non-calcified hypoattenuating cerebellar mass, with no significant enhancement.…”

Ganglioglioma and Migraine Headache

“…On CT, these lesions are most commonly hypoattenuating (38%), followed by mixed density (32%) and hyperattenuating (15%) (4). Calcifi c deposits most commonly occur in association with mixed solid/cystic masses and are seen in approximately 30% of gangliogliomas (2,5). Based on CT appearance alone, calcifi ed gangliogliomas may be indistinguishable from oligodendrogliomas.…”