Management of delayed intracranial and intraspinal metastases of intradural spinal paragangliomas

The preferential site of extra-adrenal paragangliomas is the head and neck region. However intradural spinal paragangliomas are commonly described and are considered as benign entities. We report the case of a paraganglioma of the cauda equina followed after complete surgical removal by intracranial and intraspinal cerebrospinal fluid metastases. Seven years after the first operation, a cystic cerebellar lesion was successfully treated by surgery. During a long follow-up, four locations in the posterior fossa, a lumbar recurrence and metastatic nodules at T6 and S1-S2 level were also discovered. Radiotherapy stopped the lesions' growth and allowed improvement of the neurological status. Through a review and analysis of the literature, we discuss the management of this unusual evolution.