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LETTER TO EDITOR
Year : 2012  |  Volume : 60  |  Issue : 2  |  Page : 252-254

Disseminated cranio-spinal intradural mesenchymal chondrosarcoma


1 Department of Neurosurgery, Apollo Health City, Jubilee Hills, Hyderabad, Andhra Pradesh, India
2 Department of Pathology, Apollo Health City, Jubilee Hills, Hyderabad, Andhra Pradesh, India

Date of Submission13-Jan-2012
Date of Decision26-Jan-2012
Date of Acceptance11-Mar-2012
Date of Web Publication19-May-2012

Correspondence Address:
Rahul Lath
Department of Neurosurgery, Apollo Health City, Jubilee Hills, Hyderabad, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.96432

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How to cite this article:
Sharma P, Ranjan A, Gowrishankar S, Lath R. Disseminated cranio-spinal intradural mesenchymal chondrosarcoma. Neurol India 2012;60:252-4

How to cite this URL:
Sharma P, Ranjan A, Gowrishankar S, Lath R. Disseminated cranio-spinal intradural mesenchymal chondrosarcoma. Neurol India [serial online] 2012 [cited 2022 Jan 18];60:252-4. Available from: https://www.neurologyindia.com/text.asp?2012/60/2/252/96432


Sir,

A 46-year-old male presented with rapidly progressing asymmetrical paraparesis, bladder and bowel dysfunction, and episodes of irrelevant talk of 15 days' duration. On examination he was in altered mental state with right 7 th , 9 th , 10 th , 11 th and 12 th cranial nerve paresis and poor cough reflex. Motor power in the lower limbs was Grade 1/5 with hypotonia and absent sensations below L1 level. Perianal sensations were intact with decreased anal tone. Deep tendon reflexes were absent in the lower limbs and plantars were equivocal. Magnetic resonance imaging (MRI) of the spine showed multiple panspinal intradural lesions involving predominantly the dorso-lumbar region. The lesions were isointense on T1-weighted and T2-weighted images and enhanced with contrast [Figure 1]a-c. The largest lesion was at the L1/L2 level. Computed tomography (CT) scan and MRI of brain showed ventriculomegaly with no obvious mass lesion. There was mild meningeal enhancement. Positron emission tomogram (PET) CT scan showed no evidence of disease outside the central nervous system [Figure 2]. The possibility of disseminated malignancy or granulomatous inflammation was considered and L1/L2 laminectomy and excision biopsy of the intradural lesion was done. Intraoperatively dura was normal in appearance and on opening the dura, arachnoid was intact but opaque. After opening the arachnoid there was a vascular, soft, suckable tumor which was infiltrating the surrounding cauda equina. Tumor was attached to the pia mater. Post surgery there was no worsening in the deficits. A day later he became drowsy and his sensorium decreased rapidly and he underwent emergency ventriculo-peritoneal shunt as CT scan of brain showed increase in ventricle size when compared to previous scan. Cerebrospinal fluid (CSF) was under pressure and following the shunt his sensorium improved. Histopathology showed fragments of a cellular tumor composed of sheets of round cells with vesicular nuclei and a rim of cytoplasm which appeared clear in areas. Mild anisonucleosis and scattered mitoses were identified. In these areas thin-walled dilated vessels were seen between the proliferating cells imparting a stag-horn appearance [Figure 3]a. In other areas, the cells were more loosely packed in a myxoid and chondroid background. On immunohistochemistry, pancytokeratin, Glial Fibrillary Acidic Protein (GFAP) and Leukocyte Common Antigen (LCA) were negative excluding the diagnosis of carcinoma, glioma, and lymphoma respectively. The MIB-1 index in the cellular areas averaged 15-20%. The cellular areas showed diffuse strong cytoplasmic membrane positivity with CD99, and in the less cellular chondroid areas the cells showed nuclear and cytoplasmic positivity for S100 protein and there was also diffuse strong positivity for vimentin [Figure 3]b-d. The features were diagnostic of a mesenchymal chondrosarcoma.
Figure 1: Sagittal Magnetic Resonance Image (MRI) of the dorso-lumbar spine showing an ill-defined isointense lesion in T1 (a) and T2 (b)- weighted images at the L1-L2 level. There was dural enhancement and inhomogenous lesional enhancement with Gadolinium (c)

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Figure 2: Whole body positron emission tomogram (PET) scan showing mild tracer uptake at the upper lumbar spine with normal tracer distribution in the rest of the body

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Figure 3: Hematoxylin and Eosin (× 200) (a) Left half of the image shows the cellular round cell areas with thin-walled dilated stag horn vessels and the right half of the image is sparsely cellular with cells in a myxoid and chondroid background. Immunohistochemistry panel showing diffuse strong cytoplasmic membrane positivity with CD99 (× 200) (b), cells in the chondroid area showing nuclear and cytoplasmic positivity for S100 protein (× 200) (c) and diffuse positivity for vimentin (× 100) (d)

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As his general condition was deteriorating rapidly he was started on spinal radiation therapy (RT). Option of chemotherapy in view of disseminated disease was also considered. In spite of starting RT his general condition deteriorated rapidly and he died five days after starting RT.

Extra-osseous mesenchymal chondrosarcoma of the nervous system is a rare entity and meninges are the most common site. [1] These tumors have malignant cartilaginous tissue in the background of round undifferentiated mesenchymal cells. [2],[3] It is difficult to make a diagnosis of intraspinal mesenchymal chondrosarcoma on preoperative imaging. [4] To our knowledge 13 cases of primary intraspinal mesenchymal chondrosarcoma with dural attachment have been reported and only three of these were intradural. [1],[2],[3],[4] Two were attached to the pia mater and one to the dura mater. All three cases were localized and remained symptom-free during the follow-up duration. No case of disseminated disease has been reported. Prognosis with a mesenchymal chondrosarcoma is poor because of hematogenous and lymphatic metastases. [3],[4] Radiation and chemotherapy have been used in the postoperative period by some authors. [1],[2] Our patient had craniocervical disseminated disease and focal RT to the dorso-lumbar spine and chemotherapy for the disseminated disease were considered. However, the disease progressed very rapidly with a fatal outcome.

 
  References Top

1.Ranjan A, Chacko G, Joseph T, Chandi SM. Intraspinal mesenchymal chondrosarcoma. Case Report. J Neurosurg 1994;80:928-30.  Back to cited text no. 1
[PUBMED]  [FULLTEXT]  
2.Huang KF, Tzaan WC, Lin CY. Primary intraspinal mesenchymal chondrosarcoma: a case report and literature review. Chang Gung Med J 2003;26:370-6.  Back to cited text no. 2
[PUBMED]  [FULLTEXT]  
3.Belhachmi A, Akhaddar A, Gazzaz M, Elasri C, Elmostarchid B, Boucetta M. Primary spinal intradural mesenchymal chondrosarcoma. A pediatric case report. J Neuroradiol 2008;35:189-91.  Back to cited text no. 3
    
4.Li YH, Yao XH. Primary intradural mesenchymal chondrosarcoma of the spine in a child. Pediatr Radiol 2007;37:1155-8.  Back to cited text no. 4
[PUBMED]  [FULLTEXT]  


    Figures

  [Figure 1], [Figure 2], [Figure 3]

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