- Department of Neurosurgery, University Hospitals, Leuven, Belgium
- Department of Neurosurgery, Elisabeth Ziekenhuis, Tilburg, The Netherlands
- Department of Pathology, University Hospitals, Leuven, Belgium
Frank Van Calenbergh
Department of Neurosurgery, University Hospitals, Leuven, Belgium
DOI:10.4103/2152-7806.82989Copyright: © 2011 Ardon H. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Ardon H, Plets C, Sciot R, Calenbergh FV. Paraganglioma of the cauda equina region: A report of three cases. Surg Neurol Int 18-Jul-2011;2:96
How to cite this URL: Ardon H, Plets C, Sciot R, Calenbergh FV. Paraganglioma of the cauda equina region: A report of three cases. Surg Neurol Int 18-Jul-2011;2:96. Available from: http://sni.wpengine.com/surgicalint_articles/paraganglioma-of-the-cauda-equina-region-a-report-of-three-cases/
Background:Cauda equina paragangliomas (CEP) are rare tumors. Low back pain and sciatica are the main presenting symptoms. Magnetic resonance imaging (MRI) is the study of choice and treatment consists of total excision when feasible. Definitive diagnosis can only be made after immunohistochemical investigation. CEP is classified as grade I WHO and after total removal the prognosis is excellent. Nonetheless, after subtotal removal, tumor recurrence can occur.
Case Description:We present 3 cases of CEP, preoperatively diagnosed as an intradural mass on MRI and suspected to be ependymoma. All 3 patients presented with low back pain and variable sciatic pain. Total resection of the tumor was performed after which all patients fully recovered. There is no recurrence after 13, 11, and 5 years, respectively.
Conclusion:CEP is a rare tumor. We diagnosed 3 paragangliomas out of a series of 105 intradural extramedullary tumors in adults (1994–2005). No recurrence was seen after total resection. In retrospect, both the intraoperative appearance and the MR image were not completely typical for schwannoma or ependymoma, but final diagnosis can only be made histologically.
Keywords: Cauda equina, paraganglioma, spine
Extra-adrenal paragangliomas are rare neuroendocrine tumors, which can occur throughout the body. Paragangliomas in the central nervous system occur in the carotid body, glomus jugulare, and the region of the cauda equina. Cauda equina paragangliomas (CEP) are rare tumors. The first case was, in retrospect, described in 1970.[
A 37-year-old man presented with low back pain and bilateral sciatica without neurologic deficits. MRI showed a contract enhancing intradural mass at L3–L4 with a diameter of 1.5 cm. Total resection of this vascular tumor was performed after which the patient made a full recovery. Histologic diagnosis confirmed the tumor to be a paraganglioma and there is no recurrence after 11 years.
A 41-year-old man presented with low back and right-sided leg pain. Neurologic examination only revealed sensory loss in the right leg without motor deficits. MRI showed an intradural mass at L2–L3, roughly circular, with a diameter of 2 cm and with strong but inhomogeneous gadolinium enhancement [
A 51-year-old man presented with low back pain with bilateral irradiation to the gluteal region. No neurologic deficits were present. MRI showed an intradural mass at L4, with a diameter of 1.5 cm, circular with somewhat irregular margins, moderate enhancement, and clearly located centrally in the dural sac [
CEP is a rare tumor. The first case was described in 1970 as a “secretory ependymoma of the filum terminale.” In retrospect, this tumor proved to be a CEP.[
As was also the case in our small series, there is a male predominance. Low back pain is the main symptom in 50%–87% of patients, with sciatica in 20%–74%.[
Cauda equina paragangliomas present during operation as a soft, dark red, and well-circumscribed mass, attached to the filum terminale. Endocrine and vascular symptoms due to sudden catecholamine release during removal are rare compared to surgery for cranial paragangliomas.[
We diagnosed 3 CEP on a total of 105 intradural extramedullary tumors between 1994 and 2005. All the 3 cases presented with low back pain, as most often reported in the literature. No recurrence was seen after total resection in these 3 patients. In retrospect, MRI was not completely typical for schwannoma or ependymoma, but final diagnosis can only be made histologically.
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