-
Neurosurgical Review Feb 2022Cauda equina paragangliomas are rare benign extra-adrenal neuroendocrine tumours arising from the neural crest cells associated with autonomic ganglia. These tumours are... (Review)
Review
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.
Topics: Adult; Aged; Cauda Equina; Ependymoma; Humans; Magnetic Resonance Imaging; Middle Aged; Paraganglioma; Peripheral Nervous System Neoplasms; Retrospective Studies
PubMed: 34021421
DOI: 10.1007/s10143-021-01565-7 -
Global Spine Journal Jun 2021Systematic review.
STUDY DESIGN
Systematic review.
OBJECTIVE
To compare outcomes of complete versus incomplete resection in primary intramedullary spinal cord ependymoma.
METHODS
A comprehensive search of the MEDLINE, CENTRAL, and Embase databases was conducted by 2 independent investigators. Random-effect meta-analysis and meta-regression with seven covariates were performed to evaluate the reason for the heterogeneity among studies. We also used individual patient data in the integrative analysis to compare complete and incomplete resection based on 4 outcomes: progression-free survival (PFS), overall survival (OS), postoperative neurological improvement (PNI), and follow-up neurological improvement (FNI).
RESULTS
A total of 23 studies were identified, including 407 cases. Significant heterogeneity among included studies was observed in risk estimates (I for PFS, FNI, and PNI were 49.5%, 78.3%, and 87.2%, respectively). The mean follow-up time across cases was 48.6 ± 2.35 months. Cox proportional multivariable analysis revealed that the complete resection can prolong PFS (model, hazard ratio = 0.18, CI 0.05-0.54, = .004,) and improve the FNI (binary logistic regression, adjusted odds ratio = 16.5, CI 1.6-171, = .019). However, PNI and OS were similar in patients with incomplete resected spinal cord ependymoma compared with complete resection (binary logistic regression respectively and Cox multivariable analysis, > .5).
CONCLUSION
The data presented in this study showed that OS was not significantly affected by the degree of surgery. However, complete resection of intramedullary ependymomas provides the optimal outcomes with longer PFS and better long-term neurological outcomes than incomplete resection.
PubMed: 32783515
DOI: 10.1177/2192568220939523 -
Neuro-oncology Practice Mar 2020Anaplastic ependymoma with extraneural metastases is associated with a poor clinical outcome. Metastatic spread to the parotid gland is a rare clinical entity that...
BACKGROUND
Anaplastic ependymoma with extraneural metastases is associated with a poor clinical outcome. Metastatic spread to the parotid gland is a rare clinical entity that requires multidisciplinary intervention. Herein, we present a systematic review of anaplastic ependymoma with extraneural metastases and report on a case with metastases to both parotid glands.
METHODS
Electronic databases were searched from their inception to February 2019. Inclusion criteria included reports of anaplastic ependymoma with extraneural metastasis. Studies were excluded if the tumor grade was not reported. A case illustration is provided.
RESULTS
The search yielded 15 cases of anaplastic ependymoma with extraneural metastases, including the present case. Mean age at diagnosis was 15 years. The initial tumor location was predominantly supratentorial (93.3%). All cases demonstrated leptomeningeal seeding before extraneural metastasis. Mean survival from initial diagnosis was 4.5 years. Metastasis to the parotid gland occurred in 2 cases, including the present case. We present a 17-year-old female patient who underwent gross total resection of a supratentorial, paraventricular anaplastic ependymoma followed by adjuvant external beam radiation therapy. The patient developed recurrent leptomeningeal seeding, treated with Gamma Knife radiosurgery over a 5-year period. She returned with a parotid mass and cervical lymphadenopathy and underwent parotidectomy and modified radical neck dissection. She continued to experience recurrences, including the left parotid gland, and was ultimately placed in hospice care.
CONCLUSIONS
Anaplastic ependymoma with extraneural metastasis is rare. A combination of repeated surgical resection, radiation therapy, and chemotherapy can be used to manage recurrent and metastatic disease, but outcomes remain poor.
PubMed: 32626590
DOI: 10.1093/nop/npz041