Walsh & Hoyt: Ganglion Cell Tumors

Tumors primarily composed of mature ganglion cells may occur in both the peripheral and the central nervous system. The peripheral tumors do not usually produce neuro-ophthalmologic symptoms or signs and are not discussed in this chapter. Ganglion cell tumors of the CNS are less common than their peripheral counterparts and are of two main types. Gangliocytomas are composed predominantly of mature ganglion cells supported by a stroma of spindle cells and containing calcospherites. Gangliogliomas are composed of a mixture of mature ganglion cells and mature glial cells and thus are true mixed neurogliogenic tumors. The two entities of gangliocytoma and ganglioglioma are not always distinct, and the umbrella term, ganglion cell tumor, may be used. Ganglion cell tumors occur most frequently in children and young adults. Nevertheless, the age range is broad: from several months to 80 years of age. Ganglion cell tumors regardless of their location occur with equal frequency in males and females.