Paragangliomas of the spine: a retrospective case series in a national reference French center

Abstract

Primary paragangliomas (PG) of the spine are extremely rare entities. The present study reviews our experience over a period of 30 years. Methods: This is a retrospective, single center, observational study. Patients treated for a spinal PG with confirmed anatomopathological diagnosis were included. The McCormick classification was used as a reference for clinical evaluation. Follow-up MRI and clinical assessment took place at 6 weeks, 3 months, 6 months and one year after surgery and on yearly basis after. Results: Six cases have been operated in our institution. The mean age was 37.8 (median 35.5, 30-53). The mean follow-up period was 9.6 years (median 9.5, 1-23). Preoperative duration of symptoms varied between a few hours to 4 years. Low back pain was most common sign. One presented with hemorrhage and acute onset of paraplegia. All patients underwent single surgery, with the exception of one case, which had two surgeries on the same anatomical site and a third surgery on another location of the same tumor type. Preoperatively, McCormick scale was I in four cases, and II and IV in one case, respectively. Postoperatively, all patients in McCormick I retained the same class; one patient in McCormick II passed to McCormick III; the case in McCormick IV recovered to McCormick II. Five of eight surgeries achieved total resection, while two surgeries accomplished a partial microsurgical excision and one a gross total resection. Three patients had spinal leptomeningeal dissemination. Two of them benefited from extended spine radiotherapy, while the other of a "wait-and-scan" policy. Spinal leptomeningeal dissemination was stable in all patients at last follow-up. Conclusion: We consider surgery as primary treatment in all PG. In our experience, preoperative diagnosis is difficult and caution must be taken to perioperative course in these cases. We do not routinely perform postoperative radiation if there is a residual tumor. We regularly perform clinical and radiological follow-up, so as to be able to document recurrent cases, which have been reported even up to 30 years after primary surgical excision.

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

“…Primary paragangliomas of the spine are rare, slowly growing, benign intradural extra medullary tumours [20,67].They are most commonly located in the cauda equina and filum terminale [4,20,67] representing approximately up to 3.5% of the cauda equina lesions [42]. They are classified as World Health Organization (WHO) grade I tumours, due to their indolent behaviour and histologically benign appearance.…”

“…4). We have included 51 [4,15,20,21,30,49,51,58,60,61,67,68,78] (Tables 2 and 3). The mean patients' age was 48.8 ± 1.2 (range 17-75 years).…”

“…Following surgery, 93% had no tumour recurrence, whilst recurrence was reported in 5 cases (7%) [51,53,61,67,75] (Table 4). 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].…”

“…In the central nervous system, the vast majority are located in the jugular glomus, as well as the carotid bodies which are the most common extra-adrenal site [64]. Paragangliomas of the spine as primary tumours are extremely rare with just over 200 cases reported in the English literature so far [43,67]. The peak incidence is in the 5th decade, with male predominance [20,26,67].…”

“…Paragangliomas of the spine as primary tumours are extremely rare with just over 200 cases reported in the English literature so far [43,67]. The peak incidence is in the 5th decade, with male predominance [20,26,67]. The main anatomical spinal site is cauda equina and filum terminale [3,20,26,32,58,67].…”