Functional paraganglioma: a rare conus-cauda lesion

Abstract

Conus-cauda syndrome is caused due to involvement of the lower end of the spinal cord and arising bunch of nerve roots. It is caused commonly due to traumatic injury, spinal stenosis, spinal tumors, inflammatory, and infectious conditions, but paraganglioma is a rare cause. These tumors are rarely functional and secrete catecholamine. Till now only five case reports of functional spinal paragangliomas are available to the best of our knowledge. We report a 50-year-old hypertensive male patient with a lobulated lesion extending from lower border of D12 to L2, which was reported as ependymoma on imaging studies done preoperatively. This lesion was confirmed to be a functional paraganglioma postoperatively after the patient died because of its furious complication, thus highlighting the importance of its preoperative diagnosis and management. In conclusion conus-cauda functional paragangliomas are very rare entity. Diagnosing them in preoperative condition is critical from the therapeutic point of view, both medical and surgical. During surgery these tumors should be handled very gently to avoid spillage of catecholamines into blood. These tumors require assistance of expert anesthetist and endocrinologist in the perioperative period.

Paraganglioma of the cauda equina: a tertiary centre experience and scoping review of the current literature

“…This was not encountered in our case series. Searching the literature, we found a handful of cases (including in other spinal locations) with secretory functions and mainly diagnosed intraoperatively [5,21,29,65,79].…”

“…Two patients had subtotal resection [53,67], one patient had multiple other LBP resolved sacral lesions that had grown requiring surgical intervention [75] and the two other patients had no initial postsurgery MRI scan to confirm the total resection of the tumour [51,61]. Data about secretory features was available for 96 cases (79%), of which only two cases (2%) were reported to be secretory [5,21].…”

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 ).…”

“…Despite the neuroendocrine origin of these tumors, the majority of all cauda equina paragangliomas are functionally non-secretory. This means that systemic symptoms of catecholamine activity such as paroxysmal hypertension are quite rare in this particular tumor [ 3 , 4 , 9 , 36 , [51] , [52] , [53] ].…”

“…Rarely, during tumor manipulation and excision by the surgeon; an unexplained paroxysmal hypertension resistant to any hypotensive agents might be experienced. Management of this complication without properly diagnosing the underlying functional paraganglioma is difficult issue and might be fatal [ 36 ]. Hopefully, hypertension is relieved after the complete removal of the tumor [ 36 ].…”