Mixed ganglioglioma and cavernous angioma in the temporal lobe - case report

Abstract

Objective: Gangliogliomas are rare CNS tumors defined by the presence of both neoplastic ganglion and glial cells. They are recognized by the WHO classification mostly as Grade I, although some examples showing atypia have been observed, originating from the glial component. They occur mostly in the pediatric population and young adults. Association of ganglioglioma with cavernoma is extremely rare and it could be considered as an angioganglioglioma. We present a case of synchronous ganglioglioma and cavernous angioma in 57-year-old-man with epilepsy. Methods: The patient was admitted to the Department of Neurosurgery with a left side paresis, somnolent and vomiting. He acknowledges the presence of vascular mass discovered more than 30 years ago, when he got the first seizure and denied operation. CT and MRI showed an increased lesion with recent bleeding at right temporal lobe followed by edema. Craniotomy disclosed a hemorrhagic poorly demarcated tumor which was partially removed. Grossly, the tumor tissue had solid consistency and dark brown color. Standard procedure of paraffin embedded section routinely stained with H&E was performed. Results: Histological examination revealed cavernous angioma with low grade gangliglioma located at the periphery of the angioma. The vascular component was admixed and observed in some regions of the ganglioglial mass, presenting with hyalinized ectatic vascular channels, perivascular hemosiderin deposits, gliosis and vascular calcifications. Glial cells were identified by immunopositivity for GFAP, ganglion cells for Synaptophysin and Chromogranin, cavernous vessels for SMA and CD34. The proliferative index for Ki-67 was lower than 1%. Conclusion: We present an extremely rare case of mixed ganglioglioma and cavernous angioma. The term “angioganglioglioma” was proposed to define a transitional form between angioglioma and ganglioglioma. Several other cases of mixed tumor and vascular malformation have been described in the literature. Further, it may constitute a new distinct clinicopathological entity with neoplastic and hamartomatous features.