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NEUROIMAGES
Year : 2017  |  Volume : 65  |  Issue : 5  |  Page : 1196-1198

A rare cause of neck pain: Holocord intramedullary lipoma


Department of Radiology, Military Hospital of Tunis, Tunisia

Date of Web Publication6-Sep-2017

Correspondence Address:
Sami Kouki
Department of Radiology, Military Hospital of Tunis, 1008 Montfleury
Tunisia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/neuroindia.NI_749_16

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How to cite this article:
Kouki S, Labben E, Abdallah NB. A rare cause of neck pain: Holocord intramedullary lipoma. Neurol India 2017;65:1196-8

How to cite this URL:
Kouki S, Labben E, Abdallah NB. A rare cause of neck pain: Holocord intramedullary lipoma. Neurol India [serial online] 2017 [cited 2019 Aug 25];65:1196-8. Available from: http://www.neurologyindia.com/text.asp?2017/65/5/1196/214086


Intramedullary lipomas are uncommon benign tumors accounting for less than 1% of all spinal cord tumors.[1] Their clinical presentation is not specific and may mimic any other intramedullary tumors.[1],[2],[3] Intramedullary lipomas are often focal, and only a few cases of extensive cervico-thoraco-lumbar intramedullary lipomas have been reported.[1],[3] We report the case of a holocord intramedullary lipoma with no dysraphism.

A 40-year old male patient presented with a history of progressive neck pain, which radiated to the inner aspect of both arms and forearms. Magnetic resonance imaging (MRI) of the whole neuroaxis revealed multiple intramedullary masses with a hyperintense signal on both T1-weighted and T2-weighted fast spin-echo images. The fat saturated T1-weighted images showed a total signal suppression of these lesions confirming their lipomatous nature. There was no enhancement with gadolinium.

These masses extended along the entire spinal cord and were predominant in the cervical [Figure 1] and the cervicothoracic regions [Figure 2]. The transverse slices showed the intramedullary location of these tumors [Figure 3].
Figure 1: Sagittal magnetic resonance images of the cervical spine showing the presence of intramedullary lipomas; (a) T1-weighted unenhanced image; (b) fast-spin echo T2-weighted image; (c) fat saturated T1-weighted unenhanced image; (d) fat-saturated T1-weighted contrast-enhanced image

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Figure 2: Sagittal magnetic resonance images of the thoracic spine showing the extension of the intramedullary lipomas; (a) T1-weighted unenhanced image; (b) fast-spin echo T2-weighted image; (c) fat saturated T1-weighted unenhanced image; (d) fat saturated T1-weighted contrast enhanced image

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Figure 3: MRI: Fast spin echo T2-weighted axial images of the cervical (a) and thoracic (b) spinal cord showing the intramedullary location of lipoma (arrow)

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In addition to these lipomas, there was another intramedullary lesion located in the conus of the spinal cord. It was oval shaped and had a heterogeneous signal on both T1-weighted images and T2-weighted fast spin-echo images. It had a lipomatous component, which was confirmed by fat saturation T1-weighted images. It showed no enhancement on T1-weighted contrast enhanced images [Figure 4]. There was no evidence of spinal dysraphism. Cerebral MRI showed neither the presence of an intracranial lipoma nor of cerebral malformation. The patient did not undergo surgery.
Figure 4: Sagittal MRI of the lumbar spine showing the extension of the intramedullary lipoma that including a heterogeneous intensity mass at the conus containing lipomatous and cystic components; (a) T1-weighted unenhanced image; (b) fast spin echo T2-weighted image; (c) fat saturated T1-weighted unenhanced image; (d and e) short TI inversion recovery (STIR) image; (f) fat-saturated T1-weighted contrast-enhanced image

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Intradural lipomas are uncommon lesions accounting for 1% of all spinal cord tumors and are often associated with spinal dysraphism.[1],[2] Non-dysraphic intradural lipomas, also known as “true intradural lipomas,” are even less frequent, especially those with an intramedullary location.[2] They can affect any segment of the spinal cord; however, the thoracic and cercico-thoracic regions are the most involved segments.[1],[2],[3] Only a few cases of holocord intramedullary lipomas have been reported in the literature.[1],[2]

Symptoms often appear in the second and third decades with no significant gender predominance.[1],[4] Typically, the onset is slow and insidious, and at their first presentation, these patients have a long history of progressive neurological disability.[1],[5] Periods of worsening and remission occur during this evolution. Symptoms are non-specific and can be seen in any intramedullary lesion. Clinical presentation depends on the size and location of the lipoma.[4] The clinical manifestations include paraparesis, spasticity, gait difficulties, urinary incontinence, sensory disturbances, and localized pain.

Radicular pain is rare and there is a discordance regarding its relationship to the intramedullary location of the lipoma – in some studies, it was reported to be a characteristic symptom indicating the presence of an intramedullary lipoma, while in other studies, no such relationship was found.[1],[5] In our case, the patient presented with bilateral radicular pain.

MRI is the modality of choice in diagnosing these lesions. It shows the lipomatous nature of the tumor associated with a homogenously hyperintense signal on T1-weighted images, a hyperintense signal in T2-weighted images, and signal suppression on fat suppression sequences.[3] Its treatment is still controversial. Surgery is indicated in symptomatic patients. The main purpose of surgery is to decompress the tumor and reduce its mass effect on the neuraxis. Total removal of the tumor is never the goal as it may lead to unacceptable postoperative neurological deficits.[3],[4] These lesions also have an intimate relationship with the emerging nerve roots, and due to there being no distinct plane of cleavage, total removal is not possible.[3],[4],[5]

Microscopic examination of lipoma shows mature fat cells mixed with collagen fibers. Sometimes, admixed nerve bundles are also found in the tumor, predominantly at the periphery.[1],[5] Its pathogenesis is still unclear and several hypotheses have been proposed but none of them have been confirmed. Some authors postulate that intradural lipomas arise from the mesenchymal cells that migrate into the neural tube during its closure and differentiate into fat cells instead of the dura mater. Ammerman et al., suggested that lipomas have a hamartomatous origin.[5]

Acknowledgement

The patient has been treated by Pr Mrissa at Military hospital of Tunis, Tunisia.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Giuffre R. Intradural spinal lipomas: Review of the literature (99 cases) and report of an additional case. Acta Neurochir 1966;14:69-95.  Back to cited text no. 1
    
2.
Caram PC, Carton CA, Scarcella G. Intradural lipomas of the spinal cord; with particular emphasis on the intramedullary lipomas. J Neurosurg 1957;14:28-42.  Back to cited text no. 2
[PUBMED]    
3.
Bhatoe HS, Singh P, Chaturvedi A, Sahai K, Dutta V, Sahoo PK. Nondysraphic intramedullary spinal cord lipomas: A review. Neurosurg Focus 2005;18:ECP 1.  Back to cited text no. 3
[PUBMED]    
4.
Lee M, Rezai AR, Abbot R, Coelho DH, Epstein FJ. Intramedullary spinal cord lipomas. J Neurosurg 1995;82:394-400.  Back to cited text no. 4
    
5.
Ammerman BJ, Henry JM, De Girolami U, Earle KM. Intradural lipomas of the spinal cord: A clinicopathological correlation. J Neurosurg 1976;44:331-6.  Back to cited text no. 5
[PUBMED]    


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