Combined paraganglioma and glioma of conus medullaris and cauda equina

Abstract

A mixed paraganglioma and glioma occurred in the conus medullaris and cauda equina of a woman. The tumor was subdural, encapsulated, and vascular. It was first manifested with symptoms and signs of compression of the cauda equina when she was 19 years of age and was excised when she was 24. The tumor was attached to the conus medullaris, the dura mater, and a nerve root of the cauda equina. Striking light and dark cells with an organoid arrangement mingled with a few glial fibrillary acidic protein (GFAP)-positive neuroglia forming a combined paraganglioma and glioma. Twenty-three paragangliomas were reported in the literature including the present case. The sex and age of recorded 19 patients demonstrated ten males and nine females with an average age of 46.3 years. Twenty tumors were subdurally situated at the conus medullaris and cauda equina. Three neoplasms were epidural and thoracic in location.

Paragangliomas of the lumbar region

“…Size. Although data were provided for 122 cases regarding tumor size, in only 60 cases were the three dimensions detailed; 5,7,8,[11][12][13]16,20,23,24,27,32,35,[40][41][42]48,51,53,55,67,73,74,80,82,86,91,98,104 in 17 cases two dimensions were reported, 29,31,52,71,81,83,86,88,91,95 and in 45 only one dimension was detailed. 4,21,25,37,46,50,54,60,62,63,69,86,…”

“…Myelography. Myelography was performed in 69 cases 2,[4][5][6]11,12,[15][16][17]20,21,[27][28][29]35,37,40,41,53,55,62,63,69,[71][72][73][74][81][82][83]86,89,[93][94][95]104 and revealed evidence of some degree of obstruction.…”

“…3,5,75,82,84,87 In the CNS they have been described in the pineal and pituitary glands, cerebellopontine angle, spinal cord, and cauda equina region. 3,22,36,70,72,81 Spinal paragangliomas may originate from sympathetic neurons situated in the thoracic and lumbar horns of the spinal cord, which send their axons to the sympathetic trunk via the communicating branches. It is also possible that heterotopic neurons lie along these branches proximal to the sympathetic trunk, and this site may be the original locus of tumor formation.…”

“…52 The diagnostic procedure of choice is MR imaging, which should be undertaken prior to the exploration of intraspinal masses. The lesion is usually hypo-or isointense to the conus medullaris on T 1 -weighted sequences, whereas it is hyperintense on T 2 -weighted sequences, sometimes inhomogeneous, 6,25,48,81,92,103 and in some cases cystic areas have been reported. 6,10,14,101 After Gd injection, there was marked enhancement; 6 in other cases a serpiginous area of flow voids was observed, which suggested vessels capping the tumor.…”

Acute Paraplegia From Hemorrhagic Paraganglioma Of Filum Terminale: Case Report And Review Of Literature

“…2,5,27,43 In the CNS, PGLs may occur in the filum terminale or cauda equina, sella turcica, cavernous sinus, pineal gland, pituitary gland, cerebellopontine angle, and petrous ridge. 2,5,13,18,28,34,39 Paraganglionic cells and the neural crest have a common origin, and during embryogenesis, they migrate along the neural tube. 22,37 Since paraganglionic cells do not belong within the CNS, it is thought that PGLs result from dysfunction of embryonic paraganglia cell migration or non-regression.…”