Paraganglioma of the filum terminale mimicking neurinoma: Case report

Abstract

Background:Paraganglioma of the filum terminale is an uncommon tumor of cauda equina region. Lumbar radiculopathies are revelations that can get complicated from cauda equina syndrome. Magnetic resonance imaging (MRI) allows the diagnosis without distinguishing it from a neurofibroma. Only histopathological study can bring certainty in the diagnosis. The treatment of choice is a complete removal.Case Description:We report a case of paraganglioma of the filum terminale in a 74-year-old female patient, admitted for cauda equina syndrome, which has been progressing for 2 years. MRI of medulla objectified an extramedullary lesion at L3–L4, diagnosed as neurinoma. A complete resection of the tumor was performed with a section of the filum terminale, which she was taking since birth. Histology confirmed the diagnosis of paraganglioma. The clinical course was characterized by a complete recovery of the deficit.Conclusion:Through this case report, the authors discuss clinical and radiological aspects of this tumor, as well as the management in the light of published data.

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 ).…”

“…Glial fibrillary acidic protein (GFAP) staining is one of these methods that can be used to differentiate these two pathologies. Accordingly, ependymal cells are GFAP positive whereas GFAP staining is negative in neoplastic cells of paragangliomas [ 3 , 4 , [10] , [11] , [12] , [13] , [14] , [15] , [16] , 19 , 20 , 22 , 26 , 29 , 30 , 32 , 33 , [37] , [38] , [39] , [40] , [41] , 45 ].…”

Microsurgical Management of a Primary Neuroendocrine Tumor of the Filum Terminale: A Surgical Technique

“…There are, however, a number of useful MRI findings attributed to the hypervascular nature of these tumors, venous congestion, and small foci of hemorrhage that aid in the differential diagnosis. Important T2 MRI findings include the classic "salt and pepper" appearance, serpiginous flow voids between the conus medullaris and the lesion, and a peritumoral hypointense hemosiderin rim suggestive of prior hemorrhage [1,2,9,13]. Additionally, NETs have avid contrast enhancement and large vascular pedicles on contrasted MRI sequences.…”

Acute onset of paraganglioma of filum terminale: A case report and surgical treatment

“…MRI is the gold standard radiological exam for the diagnosis and follow-up of paragangliomas of cauda equina or filum terminale, that may appear isotense on T1-weighted images and hyperintense on post-contrast T2-weighted sequences with homogenenous or heterogeneous enhancement [8] . However, these spinal tumors have no pathognomonic features and are frequently misdiagnosed as schwannomas, ependymomas, meningioma, teratoma, and hemangioma [9] . A serpiginous flow void from vessels associated with the upper pole of the tumor and a T2-weighted images revealing a hypointense tumor rim (cap sign) due to presence of hemosiderin, caused by a prior hemorrhage, can be present.…”