Introduction
Paragangliomas (PG) are catecholamine-secreting neuroendocrine tumours that arise from neuroendocrine cells of the extra-adrenal autonomic paraganglia. Most PG are benign, however up to 35% have malignant potential.[1]
Although some PG, particularly those arising in the skull base and neck, do not present with symptoms of catecholamine excess, intratumoral metabolism of catecholamines to metanephrines occurs independently of catecholamine release. As a result, biochemical testing is indicated in every patient with a paraganglioma even if the patient does not present with a clinical picture of catecholamine hypersecretion. [2]
Primary PG of the spine are extremely rare neoplasms. Classified asWorld Health Organization (WHO) Grade I tumors [3], due to their slow growth and histologically benign appearance. Data on prevalence and epidemiology is incomplete due to its rarity. The classical anatomical site is the cauda equina and filum terminale . Although the source of spinal PG as primary site remains somewhat unclear, some have suggested an origin in the sympathetic neurons in the thoracic and lumbar lateral horns of the spinal cord or heterotopic neurons, which lie along these branches proximal to the sympathetic trunk [3,4].
Primary treatment remains complete surgical resection, with preservation of the surrounding nerve roots[5]. However, in the current literature, there is a gap of knowledge with regards to several aspects, namely the role of radiotherapy and preoperative embolization, as well as therapeutic strategies at recurrence and in the case of leptomeningeal dissemination.
Leptomeningeal dissemination of a PG is an extraordinarily rare phenomenon described in a small number of cases worldwide [6,7,8].