Cauda Equina Paraganglioma: A Review

Abstract

Although rare, cauda equina paraganglioma (CEP) is definitely established in the table of cauda equina tumours. There are no particular investigations to document its preoperative diagnosis, unless a vasomotor amine syndrome is present to guide the differential diagnostic spectrum. Magnetic resonance imaging is the examination of choice for the presurgical assessment, but there are still overlapping findings, mainly among CEP, ependymoma, schwannoma, and hemangioblastoma. It is also the only means for postoperative follow-up. Computed tomography may fail to reveal all intrathecal lesions. Total excision is the gold standard for lesions with such a benign or at least protracted clinical course, as observed with the CEP. In contrast to well-known oncologic principles in micro, ultrastructural, and immunohistochemical prediction analysis, these tumours behave in an independent fashion. Promising efforts are being conducted in immunohistochemistry and DNA search, but the mainstay of our knowledge regarding its natural history still stems from our sporadic observation of its clinical behaviour.

Comment on “Paraganglioma of the cauda equina: A highly vascular tumour”

“…[3][4][5][6][7] Surprisingly, overproduction of catecholamines occurred in only one of these five patients. 7 Despite the neuroendocrine origin of these tumours, only four patients were found to have cauda equina paraganglioma with functional hormonal activity, 2,8-10 one of which refers to my publication.…”

Imaging diagnosis and the role of endovascular embolization treatment for vascular intraspinal tumors

“…In 1972, Lerman et al reported a ganglioneuroma-paraganglioma, using present terminology, of the filum terminale. 54 According to the literature, 33,50,62,88,93 spinal paragangliomas are found more frequently in the intradural extramedullary compartment and have a high affinity for the cauda equina or filum terminale. Less common sites include cervical and thoracic regions.…”

“…As for tumors of the cauda equina or filum terminale, the most common symptoms on presentation are lower back pain and sciatica 4,62,88,93 with a mean duration of 4 years. Sensory or motor deficits have been noticed in 35% of cases.…”

“…Findings on plain radiographs and CT scans include bone erosion (scalloping), widened interpedicular distance, flattening of the pedicles, and concave deformity of the dorsal margin of lumbar vertebral bodies, which are signs of a slowly growing lesion. 62 Scoliosis and spondylolisthesis have been reported sporadically. On CT images, paragangliomas appear as isodense masses with homogeneous enhancement due to their rich vascularization.…”

“…After gadolinium administration, there is an intense homogeneous enhancement. 50,62,88,93 A hypointense rim (cap sign) is usually seen on T2-weighted images, 51 which is suggestive of an intradural well-encapsulated tumor and is attributed to the paramagnetic effect of hemosiderin or ferritin from previous hemorrhages (Fig. 9).…”