A case of mobile intradural paraganglioma in the lumbar spine

Shanmugam Muthiah; Vijaykumar Sivaramamoorthy; Rath Kumar Pradipta; Ramakrishna Easwaran

doi:10.4103/joss.joss_20_21

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CASE REPORT

Year : 2022 | Volume : 9 | Issue : 1 | Page : 55-58

A case of mobile intradural paraganglioma in the lumbar spine

Shanmugam Muthiah, Vijaykumar Sivaramamoorthy, Rath Kumar Pradipta, Ramakrishna Easwaran
Department of Neurosurgery, Neuro One Hospital, Trichy, Tamil Nadu, India

Date of Submission	21-Oct-2021
Date of Acceptance	21-Jan-2022
Date of Web Publication	9-Mar-2022

Correspondence Address:
Shanmugam Muthiah
Department of Neurosurgery, Neuro One Hospital, 55/1 Karur Bye Pass Road, Trichy - 620 002, Tamil Nadu
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/joss.joss_20_21

Abstract

Mobile intradural extramedullary tumor is a rare entity with only 26 cases reported in the English literature. There is no case report of spinal paraganglioma migrating spontaneously and causing the acute neurological deficit, to the best of our knowledge. We present the first case report of a mobile spinal paraganglioma and review the literature.

Keywords: Acute deficits, filum terminale, mobile paraganglioma, neuroendocrine tumor

How to cite this article:
Muthiah S, Sivaramamoorthy V, Pradipta RK, Easwaran R. A case of mobile intradural paraganglioma in the lumbar spine. J Spinal Surg 2022;9:55-8

How to cite this URL:
Muthiah S, Sivaramamoorthy V, Pradipta RK, Easwaran R. A case of mobile intradural paraganglioma in the lumbar spine. J Spinal Surg [serial online] 2022 [cited 2022 May 27];9:55-8. Available from: http://www.jossworld.org/text.asp?2022/9/1/55/339267

Background

Spinal paragangliomas (SPs) are rare, slow-growing neuroendocrine tumors.^[1] Since 1970, less than 260 cases have been reported in the literature.^[2],[3] Mobile intradural extramedullary tumor (IDEM) is a rare disease entity.^[2],[3] So far, only 26 cases have been reported, with schwannoma being the most common along with one case each of neurenteric cyst^[4] and ependymoma.^[3],[5] There is no case report of a mobile spinal paraganglioma causing the spontaneous acute neurological deficit. Intraoperatively, the mobility of intraspinal tumor might result in failure to locate the tumor.

Case Report

A 52-year-old man presented with pain in the right leg for 6 months. About 20 days back, the pain had become more and disabling with occasional back pain. The pain was worsened by being jolted during rides while getting out of bed or chair, and on bending. He felt slightly numb in the right leg at times. He had not noticed weakness. Examination had shown restriction of straight leg raising to 45° bilaterally. There were no motor sensory or reflex deficits. There was no back tenderness, and lumbar flexion was full.

Magnetic resonance imaging (MRI) of the lumbar spine [Figure 1]a and [Figure 1]b had shown an oval intradural extramedullary mass lesion at L2 level with good enhancement and with a broad base of attachment to the dura. The tumor was isointense to cerebrospinal fluid (CSF) in T2, slightly hyperintense to CSF in T1, and showed good enhancement, especially in the peripheral portion. He was advised to undergo L2 laminectomy and excision of the tumor at the earliest. The patient did not come for surgery. He returned a month later with sudden onset of progressive weakness in the legs, worse on the left. He felt numb in the left leg. He had asymmetric paraparesis with power-Grade 4/5 on the right and 2/5 on the left. The bowel and bladder functions were intact.

Figure 1: (a and b) Magnetic resonance imaging of the lumbar spine plain and contrast showing oval intradural extramedullary contrast enhancing mass lesion at L2 level

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He was taken up for emergency surgery at night. At surgery, the L2 level was localized radiographically with lateral C-arm image intensifier screening before marking the incision and also after the laminar exposure [Figure 2]. L2 laminectomy was done, and the dura was opened in the midline. The arachnoid was opened to expose the rootlets of the cauda equina. There was no visible tumor in the intradural compartment at this level (L2). Angling the microscope and looking or palpating with an angled probe above and below the ends of the exposure also did not reveal the intradural tumor. A second check with the image intensifier screening confirmed that the L2 lamina had indeed been removed [Figure 2]. The exposure was immediately closed and the patient still under endotracheal anesthesia, was shifted to the MRI suite. The “intraoperative” MRI in the supine position showed that the tumor had shifted by one and quarter level upward and it was lying under the upper part of the L1 lamina. It had also become altered in its T2 signal character turning from T2 hyperintense to T2 hypointense suggesting intratumoral bleed [Figure 3].

Figure 2: Intraoperative fluoroscopy image confirming L2 tumor level (lamina removed)

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Figure 3: Magnetic resonance imaging lumbar spine T2W imaging showing upmigration of the tumor and it was lying under the upper part of the L1 lamina. It had also become altered in its T2 signal character (turning from T2 hyperintense to T2 hypointense)

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The patient was shifted back to the operating room and once again placed in the prone position. The laminectomy was extended one level above, and the dural incision was extended upward. The intraarachnoidal mass was found anterior to the cauda equina roots. It was purple-blackish in color and on grasping it, the wall ruptured. The altered blood content flowed out and was suctioned away. The shrunk mass could now be brought out of the arachnoid. It was found attached at the two poles to the filar vein which appeared thrombosed [Figure 4]. The vein was sectioned above and below to remove the mass in toto. The patient improved gradually in the leg weakness.

Figure 4: Intraoperative specimen removed in toto appearing purplish-black in color with attachment of pedicle on either side

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Histopathology showed a tumor composed of nests of chief cells in the zellballen pattern surrounded by capillary spaces lined by endothelial cells. There were large areas of ischemic necrosis. Immunohistochemistry showed all chief cells to be positive for synaptophysin and negative for glial fibrillary acidic protein [Figure 5]a, [Figure 5]b, [Figure 5]c, [Figure 5]d.

Figure 5: (a) blue arrow showing rounded group of cells surrounded by capillary spaces lined by endothelial cells, (b) slide showing large areas of ischemic necrosis, (c) immunohistochemical showing synaptophysin positive, (d) slide of typical zellballen pattern characteristic of spinal paragangliomas

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Discussion

Paragangliomas are tumors of neural crest cells occurring in adrenal and extraadrenal regions.^[1],[6] Extraadrenal paragangliomas are called chemodectomas if they arise in the carotid body or glomus jugulare. In the central nervous system, they are found in the suprasellar region, cerebellopontine angle, and pineal region.^[1] Rarely, they present in the spine as an intradural extramedullary mass, commonly in the lumbar region.^[1],[6] SP tends to be highly vascular, with a purplish appearance, friable in consistency, and hemorrhagic.^[6] The attachment is to the filum terminale (85%) either directly or through a vascular pedicle or to one or more nerve roots.

It is very rare for spinal paraganglioma to present with acute neurological deficit^[1] and they are often insidious in onset. So far, only three cases have been reported with acute deficits caused by paraganglioma of the cauda equina region/filum terminale.^[1] None of the three cases with acute deficits were mobile tumors. Among 26 reported cases of mobile IDEM, none presented with acute deficits due to migration. No case of migration of spinal paraganglioma causing acute neurological deficit has been reported in the literature, and ours is the first of its kind. In addition, the intratumoral bleed shown by the intraoperative MRI and confirmed at surgery is also responsible for acute neurological deterioration in our case, apart from its mobility.

Mobile intradural extramedullary tumor

The reasons for finding tumor at a different level during surgery could be

Wrong level of exposure
Nonrecognition of segmentation anomalies such as sacralized L5 or assimilated C1 arch
True tumor migration.

Migration of intradural extramedullary tumors of the spine has been reported since 1963.^[2],[3],[7] Till date, 26 cases of mobile IDEM have been reported.^[2],[8] The mechanism of migration of IDEM is still not clearly understood. The probable mechanisms by which IDEMs migrate are:^[2],[9]

Changes in intraspinal pressure due to patient's posture, straining, sneezing, coughing (Valsalva), or lumbar puncture^[10]
Dilatation of the subarachnoid space by the extramedullary tumor resulting in altered CSF dynamics tending to push the tumor upward
Elongation of the nerve root by tension resulting from the weight of the tumor. A redundant root would allow mobility of an attached tumor^[2],[7]
Migration during the surgery due to the positive pressure ventilation or the positioning of the patient.^[11],[12]

In our case, we think the mobility might have been due to change in intraspinal pressure or due to its attachment to a redundant filum terminale/filar vein as clearly shown in [Figure 3]. In addition, the T2 signal intensity change between the preoperative MRI and the one during surgery (after migration) indicated an intratumoral spontaneous hemorrhage that was confirmed by the subsequent operative finding. It is conceivable that this hemorrhage had a part to play in the mobility of the tumor by increasing its mass. In the literature, usually, the mean migration distance was about 1.28 vertebral bodies (range, 0.5–5 vertebral bodies).^[2],[7] The migration was predominantly toward the rostral direction.^[2],[7],[9] In our case, it had migrated by 1.25 vertebral body level.

Conclusion

Mobile IDEMs are rare with no previous report of mobile spinal paraganglioma. SP generally presents insidiously, but the mobility of the tumor or intratumoral spontaneous hemorrhage might cause acute neurological deterioration. Both these factors have been documented in our case by the surgical, intraoperative MRI, and histological findings.

Clinical significance

Mobile spinal paragangliomas are rare and so far, no mobile spinal tumors presented with acute neurological deficits.
Among SP, which are usually insidious in onset, only three cases presented with acute deficits, but all three were not mobile tumors
The mobility of tumor or intratumoral bleed may result in acute neurological deterioration.

Informed consent

Obtained.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published, and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial Support and Sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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