A case of adult anaplastic cerebellar ganglioglioma

Abstract

Background:Anaplastic posterior fossa ganglioglioma in adults is exceedingly rare. To date, only one case of adult anaplastic posterior fossa ganglioglioma has been reported in the English literature and none has been described at the cerebellum. To our knowledge, this report is the third case of malignant posterior fossa ganglioglioma in adults and the first at the cerebellum. In general, this entity can be misdiagnosed preoperatively as a primary posterior fossa neoplasm, and by reporting our clinical and radiographic observations we want to add to the existing literature on this rare entity.Case Description:A 40-year-old man presented with a history of headaches and dizziness and progressive gait disturbance and was diagnosed with anaplastic ganglioglioma in the posterior fossa.Conclusions:Although rare, our case demonstrates that anaplastic ganglioglioma should be considered in the differential diagnosis of infratentorial tumors in adult patients.

Infratentorial Relapsing Neuroglial Tumors in Adults: Management and Unsolved Issues—A Systematic Review

“…Subtotal resection (STR) was performed in 44% of the cases, gross total resection (GTR) in 26% of the cases, simple biopsy and partial removal in 15% of the cases. Adjuvant chemotherapy was reported in 7% of the cases after STR for a cerebellar anaplastic ganglioglioma [ 11 ] and a ganglioglioma of the fourth ventricle [ 9 ]. Adjuvant radiotherapy was found in 22% of the cases and specifically after STR of anaplastic ganglioglioma of the fourth ventricle [ 9 ], anaplastic ganglioglioma of the cerebellum [ 11 ], cerebellar ganglioblastoma [ 12 ], CPA ganglioblastoma [ 13 ] and cerebellar xanthoastrocytoma–pleomorphic ganglioglioma [ 14 ].…”

“…Adjuvant chemotherapy was reported in 7% of the cases after STR for a cerebellar anaplastic ganglioglioma [ 11 ] and a ganglioglioma of the fourth ventricle [ 9 ]. Adjuvant radiotherapy was found in 22% of the cases and specifically after STR of anaplastic ganglioglioma of the fourth ventricle [ 9 ], anaplastic ganglioglioma of the cerebellum [ 11 ], cerebellar ganglioblastoma [ 12 ], CPA ganglioblastoma [ 13 ] and cerebellar xanthoastrocytoma–pleomorphic ganglioglioma [ 14 ]. Finally, in 15% of the cases, disease recurrence occurred between 1 and 12 months after surgery (with a mean period of 11 months).…”

“…Tumor resection is widely accepted as the treatment of choice for these histopathological groups of tumors [ 11 , 35 ]. The surgical approach is related to tumor location.…”