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].