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|LETTER TO EDITOR
|Year : 2014 | Volume
| Issue : 5 | Page : 543-545
Gangliocytic paraganglioma of filum terminale: Report of a rare case
Mukund Namdev Sable1, Aasma Nalwa1, Vaishali Suri1, Pankaj Kumar Singh2, Ajay Garg3, Mehar Chand Sharma1, Chitra Sarkar1
1 Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
2 Department of Neurosurgey, All India Institute of Medical Sciences, New Delhi, India
3 Department of Neuroradiology, All India Institute of Medical Sciences, New Delhi, India
|Date of Submission||10-Jul-2014|
|Date of Decision||07-Aug-2014|
|Date of Acceptance||03-Oct-2014|
|Date of Web Publication||12-Nov-2014|
Department of Pathology, All India Institute of Medical Sciences, New Delhi
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Sable MN, Nalwa A, Suri V, Singh PK, Garg A, Sharma MC, Sarkar C. Gangliocytic paraganglioma of filum terminale: Report of a rare case. Neurol India 2014;62:543-5
|How to cite this URL:|
Sable MN, Nalwa A, Suri V, Singh PK, Garg A, Sharma MC, Sarkar C. Gangliocytic paraganglioma of filum terminale: Report of a rare case. Neurol India [serial online] 2014 [cited 2020 Feb 26];62:543-5. Available from: http://www.neurologyindia.com/text.asp?2014/62/5/543/144456
Gangliocytic paraganglioma (GP) is an extremely rare benign composite tumor, characteristically seen in the second portion of duodenum. , The lesion was first described by Dahl et al. in 1957. Kerpes and Zacharias termed this entity as "GP" in 1971. , Histologically GPs are of three cell types: Neuroendocrine, ganglion and spindle cells. The histomorphological features are common with both paraganglioma and ganglioneuroma.  The GPs of the filum terminale are extremely rare tumors with only few isolated cases described in the English literature. We herein report a case of GP as an incidentally detected lesion in the filum terminale.
A 58-year-old man presented with complaint of backache radiating to the right lower limb for the last six months. There was no history of previous hospitalization for any medical or surgical illness. Neurological evaluation showed mild weakness in right external hallucis longus muscle (4/5) with no sensory deficits. Magnetic resonance imaging (MRI) of lumbo-sacral region showed an intradural lesion (2.5 × 2.0 × 1.5 cm) at L2 spinal level. The lesion was hyperintense on T 1 weighted image (WI), hypointense on T 2 WI and showed homogenous enhancement following gadolinium administration. In addition, linear enhancing vascular structures were seen adjacent to the lesion [Figure 1]a-c. Patient was taken up for surgery. Intraoperatively, a mulberry like, soft to firm, moderately vascular tumor was seen arising from the filum and was adherent to the nerve roots of conus. Post-operatively the patient was asymptomatic and is doing well after follow up of 16 months. Routine histological study showed a well circumscribed tumor comprising of three distinct types of cell populations, that is small neuroendocrine cells, ganglion cells and spindle cells lying separately as well as admixed with each other at places. Paraganglioma like neuroendocrine area showed uniform population of epithelial cells having round nuclei with speckled chromatin and anastomozing vascular channels in the background. These cells were diffusely immunopositive for cytokeratin, chromogranin and synaptophysin. Collections of ganglion cells (immunopositive for chromogranin and neurofilament protein) and schwannian cell population (immunopositive for S-100 and neurofilament protein) formed the ganglioneuromatous component [Figure 2]a-c. There was no evidence of pleomorphism, hyperchromasia, mitotic activity or necrosis. There was no evidence of hemorrhage, necrosis or mitotic activity. A final diagnosis of GP was rendered.
|Figure 1: MRI showing an (a) intradural lesion at L2 which is hyperintense on T1-WI (white arrow) and (b) Hypointense on T2-WI (white arrow) with (c) Linear enhancing vascular flow voids (arrow head)|
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|Figure 2: (a) Photomicrograph showing a tumor composed of small nests of neuroendocrine cells (*) admixed with ganglion cells (thin arrow) and schwanniann cells (thick arrow), H and E, ×100. (b) Immunohistochemical staining for chromogranin highlights neuroendocrine (*) and ganglion cell (thin arrow) components, IHC ×100. (c) The schwannian component, diffusely immunopositive for S-100, IHC ×100|
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The spectrum of intradural extramedullary tumors of the peripheral nervous system detected within the spinal canal in adults, predominantly includes schwannomas, neurofibromas and to a lesser extent paragangliomas while, retroperitoneal neuroblastomas are more common in children.  Paragangliomas are neuroendocrine tumors, 85-90% of which arise from the adrenal gland. Extra-adrenal paragangliomas are most commonly seen in the carotid body, they have also been reported in the central nervous system (CNS) at the pineal region, petrous ridge, sella turcica and the spinal canal.  Paragangliomas of cauda equina have been well documented though, paraganglioma of the filum terminale is extremely rare and gangliocytic differentiation in a paraganglioma at this region is even rarer.  To the best of our knowledge the index case is the sixth reported case of a composite GP in the cauda equina/filum terminale region [Table 1]. ,,,, Although histogenesis of GP is controversial they usually originate from neural crest cells which give rise to sympathoadrenal lineage which further undergo divergent differentiation to form neuroendocrine and ganglion cell components. 
|Table 1: Literature review of gangliocytic paraganglioma involving exclusively filum terminale|
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The GPs are benign, encapsulated and slow growing tumors. The most common histological differential is an ependymoma which has a poorer prognosis and higher frequency of occurrence in this region.  There are a few case reports of GPs with malignant features described in duodenum.  However, till date histological features predicting malignant transformation have not been defined.
The patients usually present with lumbar pain, motor or sensory loss in lower extremities or bowel and bladder dysfunction.  The MRI of intradural extramedullary lesions is non-diagnostic and differential diagnosis includes schwannoma, ependymoma, meningioma or solitary metastasis. The presence of vascular flow-voids in the vicinity of a well-defined intradural extramedullary mass narrows the differential diagnosis to hemangioblastoma or paraganglioma. 
Few cases of spinal GP reported in the literature have been treated by surgical resection. Adjuvant radiotherapy does not guarantee prevention of tumor recurrence and should be reserved for unencapsulated or incompletely excised lesions.  To conclude, GPs of the filum terminale are extremely rare lesions with their origin in CNS being still unclear. In view of rarity, a long term follow up along with assessment of molecular pathogenesis is essential to identify their biological behavior and therapeutic interventions.
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[Figure 1], [Figure 2]
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