Leptomeningeal dissemination of a low-grade lumbar paraganglioma: case report

Abstract

<jats:p>Leptomeningeal dissemination of paraganglioma is rare, with only 2 prior cases in the literature. The authors present the case of a metastatic low-grade lumbar paraganglioma via leptomeningeal dissemination. This report emphasizes the utility of 3,4-dihydroxy-6-<jats:sup>18</jats:sup>F-fluoro-<jats:sc>l</jats:sc>-phenylalanine (<jats:sup>18</jats:sup>F-FDOPA) PET scanning for diagnosis, as well as the combination of radiation therapy and alkylating chemotherapeutic agents for the treatment of this rare phenomenon. The patient was a 61-year-old woman who presented with low-back pain and was found to have an isolated L-3 intrathecal tumor on MRI. Sixteen months after gross-total en bloc resection of the paraganglioma, the patient again became symptomatic with new neurological symptoms. MRI findings revealed enhancing leptomeningeal nodules throughout the spine. <jats:sup>18</jats:sup>F-FDOPA PET/CT scanning was used to confirm the diagnosis of disseminated paraganglioma. Intrathecal thiotepa, radiation therapy, and systemic therapy with capecitabine and temozolomide have been used sequentially over a 2-year period, with each able to stabilize tumor growth for several months. The authors also summarize the 2 other reports of leptomeningeal dissemination of paragangliomas in the literature and compare the course and management of the 3 cases.</jats:p>

Leptomeningeal disease: current diagnostic and therapeutic strategies

“…showed no impact on outcome [ 82 – 86 ]. Similarly, case reports have suggested efficacy against LMD secondary to paragangliomas [ 87 ], adenocarcinomas [ 88 – 90 ], SCLC [ 91 ], breast cancer [ 62 ], and melanoma [ 92 – 94 ]. However, a phase II randomized controlled trial of 19 patients with LMD from solid tumors was prematurely stopped due to poor accrual, with median time to progression of 28 days (95% CI: 14–42 days), median survival of 43 days (95% CI: 28.7–57.3 days), and only two patients finishing the trial [ 95 ].…”

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 ].…”

“…Leptomeningeal dissemination of a paraganglioma of the filum terminale has been reported in one occasion [ 33 ].…”

Intramedullary metastases detected on 18F FDG-PET/CT imaging

“…al. in a recent article [7]. Spinal cord metastasis from prostate cancer detected with 68Ga-PSMA PET/ CT with MRI fusion was reported by Langsteger W et al [8].…”