This article includes discussion of ganglioglioma, gangliocytoma, ganglioglioneuroma, ganglion cell tumor, ganglioneuroglioma, ganglioneuroma, and neuroastrocytoma. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.
Ganglioglioma is an uncommon tumor with neuronal tissue and glial cells. In this article, the author discusses diagnosis and treatment of ganglioglioma in detail.
• Though mostly benign, several cases of anaplastic ganglioglioma have been reported.
• Gross total resection of the tumor remains the only effective treatment in most cases.
• Prognosis is excellent when the tumor is completely resected.
Historical note and terminology
Ganglioglioma is a mixed tumor that contains both ganglion and glial cells. The term "ganglioneuroma" is used for a tumor consisting primarily of neoplastic neuronal tissue with few non-neoplastic glial elements. The gangliogliomas arise from neuronal cells and primitive multipotential precursors. Virchow realized the existence of nervous system tumors containing both neuronal elements and glia and classified these in 2 groups: (1) those containing myelinated nerve fibers and (2) those comprised of unmyelinated fibers (Virchow 1815). Perkins introduced the term "ganglioglioma," but Courville is credited with establishing the term "ganglioglioma" (Perkins 1926; Courville 1930).
Malignant ganglioglioma is rare. Malignant ganglioglioma was first reported in 1925 by Bielschowsky, who provided the description of a multifocal ganglioglioma that disseminated throughout the ventricular system and subarachnoid space (Bielschowsky 1925). Sporadic cases have been reported since the initial description of malignant ganglioglioma. Malignant transformation in ganglioglioma generally occurs in the glial component that often demonstrates distinct anaplastic features, resembling anaplastic astrocytoma or even glioblastoma (Kurian et al 1998; Dash et al 1999). The ganglion cells are generally considered to lack a neoplastic nature. However, one report of the first unequivocal case of a ganglioglioma harboring aberrant TP53 product that was expressed predominantly in the neuronal component suggests that ganglion cells can participate in the neoplastic process of gangliomas (Fukushima et al 2005). Desmoplastic infantile ganglioglioma is a rare intracranial tumor of childhood that involves the cerebral cortex and the leptomeninges. Pathologically, this rare intracranial tumor shows glial and ganglionic differentiation, accompanied by an extreme desmoplastic reaction. These are low-grade neoplasms that are questionably benign (Taranath et al 2005).
Continued refinement of the diagnostic and nosological criteria for this glioma subtype continued into the 1980s, the literature consisting mainly of gross and light histopathological characterizations of the tumor as well as case descriptions; more recently, electron microscopy has gained increasing importance in differentiating these tumors from other gliomas based on cellular ultrastructural details. Articles representative of the earlier part of this era are those by Courville, Alpers and Grant, and Kuhlenbeck and Haymaker (Courville 1930; Alpers and Grant 1931; Kuhlenbeck and Haymaker 1946).
Histochemistry and immunocytoreactivity have been used to diagnose ganglioglioma. Currently accepted techniques utilize the immunohistochemical reactivity of synaptophysin, a cell surface glycoprotein found on neurones, to demonstrate ganglion cells. Russell and Rubenstein's text and Burger and Scheithauer's monograph both give an overview of these techniques and present detailed, histopathological descriptions of gangliogliomas (Russell and Rubenstein 1989; Burger and Scheithauer 1994; McLendon and Provenzale 2002).
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