A 42-year-old man with a densely vascular spinal mass -: Diagnosis -: De novo malignant ganglioglioma (GG) of the spinal cord with unusually prominent dysplastic vessels ("malignant angioganglioglioma")

September 2006. A 42-year-old man was admitted to the neurosurgery department because of paraparesis and sensory deficits of both feet. A solitary exophytic lesion of the thoracic spine was seen on MRI, and angiography further revealed the presence of feeding and draining vessels and intra-lesional shunting. The preoperative diagnosis was spinal hemangioblastoma. A gross total resection was performed. By histological examination, the lesion was a tumor composed of neoplastic astrocytes and cells immunopositive for neuronal markers and CD34. The neuronal subpopulation was quite polymorphous and consisted of large anaplastic neurons including binucleate forms and smaller immature looking cells. Vessels were abundant and showed dysplastic changes such as sclerosis, calcium incrustations and extreme dilatation. Because of necrosis and marked proliferative activity, the tumor was considered a de novo malignant ganglioglioma (GG). In conformity with the diagnosis of malignancy, the tumor gave rise to extensive cerebrospinal deposits in the intracranial and spinal compartments 12 months post-diagnosis. De novo malignant GG of the spine are very rare tumors of which few cases are on record. Interestingly, in our case the rich malformative vasculature and the corresponding angiographic image were most compatible with descriptions of "mixed" angiogliomas. The diagnosis of spinal GGs should rely on both histologic hallmarks and unequivocal immunopositivity for several neuronal markers because of reported aberrant expression of synaptophysin by non-neoplastic spinal neurons.