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

Abstract

Cauda equina paragangliomas are rare benign extra-adrenal neuroendocrine tumours arising from the neural crest cells associated with autonomic ganglia. These tumours are often mistaken preoperatively for ependymomas or schwannomas. Patients present with axial or radicular pain with or without neurological deficits. Recurrence, secretory features and length of follow-up are controversial. We conducted a retrospective cohort study of paraganglioma through searching a prospectively maintained histopathology database. Patient demographics, presentation, surgery, complications, recurrence, follow-up and outcome between 2004 and 2016 were studied. The primary aim was to collate and describe the current evidence base for recurrence and secretory features of the tumour. The secondary objective was to report outcome and follow-up strategy. A scoping review was performed in accordance with the PRISMA-ScR Checklist. Ten patients were diagnosed (M:F 7:3) with a mean age of 53.6 ± 5.1 (range 34–71 years). MRI scans revealed intradural lumbar enhancing lesions. All patients had complete microsurgical excisions without adjuvant therapy with no recurrence with a mean follow-up of 5.1 ± 1.4 years. Tumours were attached to the filum terminale. Electron microscopic images demonstrated abundant neurosecretory granules with no evidence of catecholamine production. A total of 620 articles were screened and 65 papers (including ours) combining 121 patients (mean age 48.8 and M:F 71:50) were included. The mean follow-up was 3.48 ± 0.46 (range 0.15–23 years). Back pain was the most common symptom (94%). Cure following surgery was achieved in 93% of the patients whilst 7% had recurrence. Total resection likely results in cure without the need for adjuvant therapy or prolonged follow-up. However, in certain situations, the length of follow-up should be determined by the treating surgeon.

Cauda equina neuroendocrine tumors: A single institutional imaging review of cases over two decades

“…Our findings are similar to the previously reported literature on CE-NETs in terms of age at presentation and clinical symptomatology. 8,17 None of the tumors in our series showed any hemorrhage, which has been variably reported in 10% (2/19) of cases by Yang et al and 15% (2/13) of cases by Fiorini et al in their series. 10,17 This may at least be partly due to the larger tumors encountered in those series, with a mean craniocaudal dimension of 4.9 and 3.4 cm, respectively, in contrast to 2 cm in our series.…”

“…1,4,5 CE-NETs are rare, accounting for <3% of spinal intradural tumors, with an estimated annual incidence rate of 3 cases per million. [6][7][8] Since their initial description in 1970, nearly 300 cases have been reported, mostly as sporadic case reports and small series. 9 CE-NETs generally occur in adults, peaking in incidence between the fourth and sixth decades (age range: 9-75 years; mean 46-47 years) and have a slight male predominance (M:F::1.5:1).…”

How to avoid intraoperative complications of active paragangliomas?

“…Shtaya et al . [ 11 ] reported in their recent and excellent review on PGs that circa 200 cases have been reported so far in the English literature. The mean age of the patients was 48.8 ± 1.2 years.…”

Diagnosis and Treatment of Peripheral and Cranial Nerve Tumors with Expert Recommendations: An EUropean Network for RAre CANcers (EURACAN) Initiative

“…Cauda equina neuroendocrine tumors usually affect adults, with a peak of incidence in the fourth–sixth decades of life, with a median age of 46 years (range 6–85) [ 58 ]. Notably, when neuroendocrine tumors affect the spinal cord, the cauda equina represents the most frequent location, but uncommon locations in the cervical and thoracic regions have been reported [ 59 , 60 , 61 ]. Most of cauda equina neuroendocrine tumors are considered sporadic, with slow-growing behavior, and neurological symptoms correlated to the site of presentation, including low back and/or radicular pain, numbness, paraparesis, sphincter symptoms, or complete cauda equina syndrome [ 62 , 63 ].…”