828 Neurology India | Nov-Dec 2009 | Vol 57 | Issue 6

Letters to Editor

1999;99:204-7.2. Cullen JF, Haining WM, Crombie AL. Cerebral aneurysms presenting

with visual field defects. Br J Ophthalmol 1966;50:251-6.3. Peiris JB, Ross Russell RW. Giant aneurysms of the carotid system

presenting as visual field defect. J Neurol Neurosurg Psychiatry 1980;43:1053-64.

4. Roux FX, Panthier JN, Tanghe YM, Gallina P, Oswald AM, Mérienne L, et al. Terson’s syndrome and intraocular complications in meningeal hemorrhages (26 cases). Neurochirurgie 1991;37:106-10.

Accepted on 02-07-2009

Intramedullary Ewing’s sarcoma of the spinal cord associated with hydrocephalusSir,Ewing’s sarcoma is an uncommon malignant tumor and accounts for approximately 10% of the primary malignant bone tumors.[1] Spinal cord metastases is an extremely rare and only two cases have been reported. [2,3]

We report a third patient.

A 28-year-old man presented with progressive weakness of both lower extremities of fifteen days duration and recurrent headaches, vomiting, fever and bladder of four days duration and bowel incontinence of one day duration.Neurological examination showed bilateral papilledema and abducens nerve paralysis. Flaccid areflexic weakness (motor power 0/5) in both the lower with sensory level at T11 for all the modalities of sensations. Cranial magnetic resonance imaging (MRI) showed communicating type of hydrocephalus. Spintal MRI showed abnormal signal focus (14 3 2 3 2 cm3)

at the level of T12 to L3 vertebrae [Figure 1a-c]. External ventricular drainage and exploration and decompression of spinal canal from T11 to L3 were performed. During the operation, spinal exploration found sclerotin of vertebra bodies from T11 to L3 as normal. The tumor was flesh and soft, with no clear boundary and peplos. The gross tumor was resected under micromanipulation. Both hematoxylin and eosin (H and E) and immunohistochemistry staining [Figure 2] made the diagnosis of Ewing’s sarcoma. The patient recovered well. But the sarcoma recurred and metastasis nine months after the operation, and there was no more opportunity to treat the disease.

Ewing’s sarcoma is a kind of osseous undifferentiated reticulocytic sarcoma, which was firstly reported in 1921 by James Ewing. It represents the second most common primary bone malignancy in childhood and adolescence, with an estimated annual incidence of 0.6 per million population.[4] It was depicted that the Ewing’s sarcoma was originated from immature marrow reticulum tissue, while more and more immunohistochemistry, electron microscope and cytogene studies on sarcoma indicate the tumor originated from neural ectoderm.[2,5] In this report, the tumor was originated from nerve tissue in the spinal cord. Neither clinical and MRI examination nor the operation indicated any relationship between tumor and sclerotin, and the spondylous was intact.

Initial symptoms of intramedullary Ewing’s sarcoma were completely similar to common tumor in the spinal cord, which included decreasing sensation or absence first and followed by motor dysfunction and difficulty of urination and defecation. However, intramedullary Ewing’s sarcoma had an acute onset and a short course of disease, and a progressive aggravation of nerve damage, which caused the complete flaccid paralysis.

Figure 1: (a) T1-weighted sagittal magnetic resonance image (MRI) of the thoracolumbar spine (T12 to L3) reveals a well-defined lesion, hypointense to the spinal cord, located in the substance of the spinal cord; (b) T2-weighted sagittal MRI shows that the lesion is hyperintense compared with the spinal cord;

(c) GD-DTPA enhancement scanning showed moderate heterogeneous enhancement and no abnormal signal in canalis spinalis from T12 to L3

829Neurology India | Nov-Dec 2009 | Vol 57 | Issue 6

Letters to Editor

Figure 2: Photomicrographs of the tumor specimen showing: (a) Densely packed, small round cells with a high nuclear-cyroplasmic ratio. (H and E, 3400); (b) Diffuse positive for CD99; (c,d) Negative staining results for NSE, PCK (3400)

Being short of vigilance and perceptual ability were the main reasons for the early misdiagnosis. The patient was wrongly diagnosed as cerebral meningitis because of the ardent fever and headache after the disease appeared. Three times failure of lumbar puncture as well as the cerebral meningitis irritation symptoms made therapy of infectious disease to be conducted. Only when both lower extremities paralysis and urinary and fecal incontinence happened after the fourth lumbar puncture, intramedullary Ewing’s sarcoma was detected by the spinal cord MRI inspection. Therefore, whenever came to diseases in the spinal cord that were acute and together with fever or dropsy of brain with acute intracranial hypertension, intramedullary Ewing’s sarcoma should be considered first. Overall, low T1WI signal and high T2WI signal spinal cord MRI together with moderate enhancement effect in a young adult with fever was considered as intramedullary Ewing’s sarcoma.

Lu Jia, Guoping Li, Chao You, Min He, Feng YeDepartment of Neurosurgery, West China Hospital of Sichuan

University, 37 Guo Xue Xiang, Wu Hou District, Chengdu - 610 041, China. E-mail: hxbslgp@163.com

PMID: *** DOI: 10.4103/0028-3886.59499

References

1. Mehta Y, Hendrickson FR. CNS involvement in Ewing’s sarcoma. Cancer 1974;33:859-62.

2. Kara G. Spinal cord Ewing’s sarcoma metastasis: Presentation of one case. Ann Nucl Med 2004;18:623-6.

3. Weil RJ, Zhuang Z, Pack S, Kumar S, Helman L, Fuller BG, et al. Intramedullary Ewing sarcoma of the spinal cord: Consequences of molecular diagnostics. J Neurosurg (spine 2) 2001;95:270-5.

4. Güzel A, Tatli M, Er U, Yilmaz F, Bavbek M. Multifocal Ewing’s sarcoma of the brain, calvarium, leptomeninges, spine and other bones in a child. J Clin Neurosci 2008;15:813-7.

5. Song X, Choi J, Rao C, Nallu S, Nicastri AD. Primary Ewing sarcoma of lumbar spine with massive intraspinal extension. Pediatr Neurol 2008;38:58-60.

Accepted on 02-07-2009