- Department of Neurosurgery, National Institute of Mental Health and Neuroscience, Bengaluru, Karnataka, India
- Department of Neuropathology, National Institute of Mental Health and Neuroscience, Bengaluru, Karnataka, India
Amey R. Savardekar
Department of Neurosurgery, National Institute of Mental Health and Neuroscience, Bengaluru, Karnataka, India
DOI:10.4103/2152-7806.180093Copyright: © Surgical Neurology International This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
How to cite this article: Nagarjun MN, Savardekar AR, Kishore K, Rao S, Pruthi N, Rao MB. Apoplectic presentation of a cauda equina paraganglioma. Surg Neurol Int 11-Apr-2016;7:37
How to cite this URL: Nagarjun MN, Savardekar AR, Kishore K, Rao S, Pruthi N, Rao MB. Apoplectic presentation of a cauda equina paraganglioma. Surg Neurol Int 11-Apr-2016;7:37. Available from: http://surgicalneurologyint.com/surgicalint_articles/apoplectic-presentation-of-a-cauda-equina-paraganglioma/
Background:Cauda equina paragangliomas (CEPs) are rare spinal tumors that are mostly misdiagnosed preoperatively as ependymomas or schwannomas on magnetic resonance imaging (MRI). Clinically, they usually present with the gradual onset of back pain and radiculopathy rather than an acute cauda equina syndrome.
Case Description:A 36-year-old female presented with an acute flaccid paraparesis/cauda equina syndrome. Based upon MRI studies, the predominant differential diagnoses included ependymoma or schwannoma. The intraoperative findings revealed an acute intralesional hemorrhage or apoplexy, responsible for the acute clinical deterioration. Histopathology and immunohistochemistry (IHC) revealed that the tumor was a paraganglioma.
Conclusion:CEPs commonly present with mild symptoms and signs rather than the acute-onset of a flaccid paraparesis/cauda equina syndrome as seen in this case. Here, the authors review the radiological and histopathological characteristics of CEP and emphasize the role of IHC in differentiating “CEP” from the more common ependymomas.
Keywords: Apoplexy in spinal tumors, cauda equina paraganglioma, intradural extramedullary spinal tumor, intralesional hemorrhage in spinal tumors, tumors of the filum terminale
Paragangliomas are rare, representing approximately 3% of cauda equina tumors.[
Clinical and radiographic presentation
A 36-year-old female, presented with a 4-day history of the acute-onset of a flaccid paraplegia with urinary retention, accompanied by partial sensory loss below L1, and complete sensory loss below L3. On magnetic resonance imaging (MRI), the lesion was well-demarcated, extending from T12 to L2 [Figure
Magnetic resonance imaging showing (a) T1-weighted sagittal, (b) T2-weighted sagittal, (c) contrast-enhanced T1-weighted sagittal, (d) STIR sequence coronal images (hyperintense signal, suggestive of intralesional bleed), (e) contrast-enhanced T1-weighted coronal image (small rim of enhancement at the superior pole), (f) T2-weighted axial image, and (g) contrast-enhanced T1-weighted image at the superior pole (D12-L1 level showing rim-enhancement)
Surgical intervention and follow-up
She emergently (within 24 h) underwent a T12-L2 laminectomy for tumor excision. Intraoperatively, the lesion was dark red in color, originated from the filum terminale, was ventral to the cauda equina, and had a well-defined, tense capsule due to the intralesional hemorrhage. The histopathology was consistent with a paraganglioma containing areas of fresh hemorrhage [
Photomicrographs of paraganglioma showing (a) well-circumscribed lesion (starred) with predominant hemorrhage. (b) The cellular component is arranged in sinusoidal pattern separated by thin-walled blood vessels. (c) Cells have a plasmacytoid appearance, with abundant cytoplasm. (c - inset) The intracytoplasmic granules express chromogranin (a: H and E, ×50; b: H and E, ×100; c: H and E, ×400; c-inset = immunoperoxidase, chromogranin)
Origin and magnetic resonance findings of cauda equina paragangliomas
Paragangliomas are neuroendocrine tumors arising from specialized neural crest cells (chief cells) and are commonly encountered in the fifth and sixth decades of life.[
Histopathology of cauda equina paragangliomas
On histopathological examination, CEPs are well-encapsulated, benign tumors (WHO Grade I), characterized by the presence of “zellballen,” or a nesting of cell groups, and trabecular cords of cells within thin fibrovascular stroma.[
Immunohistochemistry of cauda equina paragangliomas
On IHC, the “chief cell” within the tumor is glial fibrillary acidic protein stain (GFAP) negative, while being neuron-specific enolase, synaptophysin, and chromogranin positive.[
CEPs are solid, well-encapsulated, highly vascular benign lesions, which commonly present in an insidious fashion. Although CEPs have been reported to present with spinal subarachnoid hemorrhage, presentation with an acute flaccid paraparesis/cauda equina syndrome attributed to an intratumoral hemorrhage has not been previously documented.[
Financial Support and Sponsorship
Conflicts of Interest
There are no conflicts of interest.
1. Ardon H, Plets C, Sciot R, Calenbergh FV. Paraganglioma of the cauda equina region: A report of three cases. Surg Neurol Int. 2011. 2: 96-
2. Demirçivi Ozer F, Aydin M, Bezircioglu H, Oran I. Paraganglioma of the cauda equina: A highly vascular tumour. J Clin Neurosci. 2010. 17: 1445-7
3. Levy RA. Paraganglioma of the filum terminale: MR findings. AJR Am J Roentgenol. 1993. 160: 851-2
4. Li P, James SL, Evans N, Davies AM, Herron B, Sumathi VP. Paraganglioma of the cauda equina with subarachnoid haemorrhage. Clin Radiol. 2007. 62: 277-80
5. Sousa J, O’Brien D, Crooks D. Paraganglioma of the filum terminale. J Clin Neurosci. 2005. 12: 584-5
6. Wippold FJ, Smirniotopoulos JG, Pilgram TK. Lesions of the cauda equina: A clinical and pathology review from the Armed Forces Institute of Pathology. Clin Neurol Neurosurg. 1997. 99: 229-34
7. Yang SY, Jin YJ, Park SH, Jahng TA, Kim HJ, Chung CK. Paragangliomas in the cauda equina region: Clinicopathoradiologic findings in four cases. J Neurooncol. 2005. 72: 49-55