Gangliocytic paraganglioma of dorsolumbar spine: A rare tumor at rare site

Abstract

Spinal paragangliomas are rare benign tumors. The gangliocytic paragangliomas (GP) of spine are even rarer. The GPs are almost exclusively seen in duodenum. In spine, the usual site of affection is cauda equina region. The involvement of other spinal levels is far less often and includes thoracic and cervical region. We report this case of GP involving the conus medullaris region and reaching up to the upper part of cauda equina. The prognosis of spinal GP is excellent after total excision, as it is classified under WHO Grade I tumor. The patient made a full recovery after tumor removal. Clinical, radiological, and pathological characteristics of this rare tumor are discussed here along with.

Gangliocytic paraganglioma of the spine

“…The majority (75%) of paragangliomas are encapsulated, usually attached to the filum terminale or, less commonly, a nerve root. 3 These tumors are intradural extramedullary in location. On MRI, the spinal paragangliomas are typically hypo-to isointense on T1-weighted images, hyperintense on T2-weighted images, and vividly enhancing on contrast studies.…”

“…2 The duodenum is the most common site of gangliocytic paraganglioma. 3 Nearly 25%of cauda equina paragangliomas are gangliocytic paragangliomas. 4 Gangliocytic paraganglioma (GP) was first reported in 1957 as ganglioneuroma by Dahl et al 2 It was named gangliocytic paraganglioma by Kepes and Zacharias in 1971.…”

“…To date, only nine cases of gangliocytic paraganglioma have been previously reported, and to the best of our knowledge, this is the largest gangliocytic paraganglioma that has been reported. [1][2][3][4][5][6]…”

Paraganglioma of the filum terminal: Case report and review of the literature

“…We updated the review from 2009 until October 2020 and could find 104 new cases including a case of our own [ [10] , [11] , [12] , [13] , [14] , [15] , [16] , [17] , [18] , [19] , [20] , [21] , [22] , [23] , [24] , [25] , [26] , [27] , [28] , [29] , [30] , [31] , [32] , [33] , [34] , [35] , [36] , [37] , [38] , [39] , [40] , [41] , [42] , [43] , [44] , [45] , [46] ]. Adding these cases to 215 previously reported cases reported in Gutenberg’s survey, the total number of the cases reached to 319 ( Table 1 ).…”

“…A small number of paragangliomas may contain mature ganglion cells in addition to the sustentacular cells, this type is designated as cauda equina gangliocytic paraganglioma [ 5 , 8 , 18 , 24 , 26 , 40 , 78 ]. Histologically, in gangliocytic paragangliomas, neurofilament staining is present in spindle cells along with ganglion cells confirming a gangliocytic variation of a paraganglioma The origin of this variation remains unclear, although it is generally believed that gangliocytic paragangliomas originate from neuroectodermal ganglion cells [ 5 , 8 , 18 , 24 , 26 , 40 , 78 ].…”