Primary supratentorial intracerebral malignant paraganglioma

Paragangliomas are extra-adrenal neuroendocrine tumors that derive from neural crest. In general, they are benign tumors but few cases had shown a tendency to metastasize. Malignant forms have been reported previously with intracranial metastasis from duodenal origin, but primary intracranial origin represents a rare and unusual location for such tumors. Here, we report a rare case of a 48-year-old lady who presented with symptomatic right-sided insular mass with negative metastatic work up. A complete surgical resection had been done with an unexpected diagnosis of primary gangliocytic paraganglioma with malignant features.