Journal of Postgraduate Medicine
 Open access journal indexed with Index Medicus & ISI's SCI  
Users online: 2392  
Home | Subscribe | Feedback | Login 
About Latest Articles Back-Issues Article Submission Resources Sections Etcetera Contact
 
  NAVIGATE Here 
  Search
 
 :: Next article
 :: Previous article 
 :: Table of Contents
  
 RESOURCE Links
 ::  Similar in PUBMED
 ::  Search Pubmed for
 ::  Search in Google Scholar for
 ::Related articles
 ::  Article in PDF (190 KB)
 ::  Citation Manager
 ::  Access Statistics
 ::  Reader Comments
 ::  Email Alert *
 ::  Add to My List *
* Registration required (free) 

  IN THIS Article
 ::  Abstract
 ::  Case History
 ::  Discussion
 ::  References
 ::  Article Figures

 Article Access Statistics
    Viewed7774    
    Printed164    
    Emailed2    
    PDF Downloaded212    
    Comments [Add]    
    Cited by others 2    

Recommend this journal


 


 
CASE REPORTS
Year : 2005  |  Volume : 51  |  Issue : 3  |  Page : 220-222

Craniospinal dissemination of clival chondroid chordoma


Department of Pathology, B. Y. L Nair Hospital, Mumbai, India

Correspondence Address:
S V Shinde
Department of Pathology, B. Y. L Nair Hospital, Mumbai
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


PMID: 16333198

Rights and PermissionsRights and Permissions


 :: Abstract 

Chondroid chordoma commonly presents as clival osseous and extradural mass. A 15-year-old boy presented with progressive visual deficit, headaches and diplopia since three years. Computed tomography (CT scan) showed a skull base tumour, but was wrongly reported at the time as chronic sphenoidal sinusitis and nasal polyps. In the past three months, he developed dysphagia, urinary retention and constipation. Terminally, he had weakness of all limbs. Fundoscopy showed optic atrophy. Temporal and spatial variation in symptoms led to a clinical diagnosis of multiple sclerosis with optic neuritis. Partial brain autopsy revealed small gelatinous tumour nodules in the subarachnoid space of middle cranial fossa encasing base of brain like arachnoiditis. Tumour deposits extended down into the spinal cord along the subarachnoid space as far as vision allowed. Histopathology and immunohistochemistry confirmed a diagnosis of chondroid chordoma. Awareness of this rare mode of dissemination will avoid misdiagnosis and delay in treatment


Keywords: Craniospinal-chordoma-subarachnoid


How to cite this article:
Shinde S V, Monipanda K. Craniospinal dissemination of clival chondroid chordoma. J Postgrad Med 2005;51:220-2

How to cite this URL:
Shinde S V, Monipanda K. Craniospinal dissemination of clival chondroid chordoma. J Postgrad Med [serial online] 2005 [cited 2020 Apr 5];51:220-2. Available from: http://www.jpgmonline.com/text.asp?2005/51/3/220/19026


Chondroid chordoma commonly presents as clival tumour in young individuals and has a favorable prognosis.[1] Drop metastases and dural dissemination usually follow multiple surgical interventions due to implantation or hematogenous spread.[2],[3],[4] The present case in a young boy showed a craniospinal dissemination of clival chondroid chordoma along the subarachnoid space in the absence of any surgical intervention.


 :: Case History Top


A 15-year-old boy presented three years ago with diplopia and bilateral lateral rectus palsy following a minor nasal trauma. Computed tomography (CT) scan at the time was reported as being unremarkable. In the following six months, the patient started having intermittent throbbing frontal headaches with progressive visual deficit. The CT scan showed a skull base tumour, but was reported at that time as nasal polyps and chronic sphenoidal sinusitis. During the previous three months, he had dysphagia and suffered from an inability to initiate urination and defecate. Terminally, he came to Mumbai with urinary retention, weakness of all limbs and projectile vomiting. Neurological examination revealed hypotonia of all limbs along with areflexia and sensory loss below thoracic (T) 6 level. Cerebrospinal fluid (CSF) examination was unremarkable. Fundoscopy revealed bilateral optic atrophy and macular oedema. Complete haemogram revealed only mild anaemia (Haemoglobin 8.3 gm%). Ultrasonography (USG) of the abdomen revealed bilateral hydroureter, mild hydronephrosis and cystitis. Urine examination showed trace proteins and sugar and few pus cells. Renal and liver function tests were normal. USG abdomen and x-ray chest did not show any focal lesion. Clinical diagnosis was Devic's disease (Multiple Sclerosis with optic neuritis) with urethral stricture. The patient expired before Magnetic Resonance Imaging (MRI) could confirm the diagnosis.

Partial brain autopsy revealed diffuse gelatinous tumour nodules encasing the base of the brain in a flat sheet, like arachnoiditis [Figure - 1] and causing local destruction of the optic nerve and pituitary. Tumour deposits extended down the foramen of Magnum along the subarachnoid space as far as vision allowed.

Histopathology showed a lobular tumour [Figure - 2] with chondroid and chordoid areas. Chordoid foci showed typical physalipherous and cuboidal cells in cords and trabeculae. The cells were Periodic Acid Schiff (PAS) positive and diastase digestible. These areas were merging with chondroid matrix with atypical cells in lacunae [Figure - 3], thus satisfying Heffelfinger's critera.[1] The matrix was Alcian blue positive. Psammoma bodies and metaplastic bone formation were seen. Immunohistochemistry found the tumour to be strongly positive for EMA (epithelial membrane antigen), S100, vimentin and cytokeratin, while GFAP (glial fibrillary acid protein) positivity was focal and weak, thus confirming the diagnosis of a chondroid chordoma.


 :: Discussion Top


Chordomas are tumours arising from rests of primitive notochord along the craniospinal axis, especially at the cranial and sacral end.[1] Chondroid chordoma, first described by Heffelfinger in 1973, is a variant of chordoma that straddles the bridge between conventional chordoma and chondrosarcoma.[1] Twenty-eight to thirty-four per cent of skull base chordomas are of the chondroid variety.[1]

Common clinical presentations[1] of clival chordoma include diplopia, visual defect, headache, VI-XII cranial nerve palsies, hypopituitarism and intranasal mass.[5] Rarely, does it present as pure intradural chordoma.[6] Drop metastases and subarachnoid dissemination from clival origin to cervical and thoraco-lumbosacral regions usually occurs late in the course of the disease and after multiple surgical and radiation therapies.[2],[3],[4] This route of spread is either due to surgical implantation or hematogenous dissemination[2],[3],[4] and is more common with anaplastic chordomas.[3]

On hindsight, our case had all the typical features of a clival space-occupying lesion, though they were initiated by minor trauma. Dysphagia and nasal polyps were probably due to nasopharyngeal spread of clival tumour. Weakness, hypotonia, areflexia could be attributed to cervico-thoraco-lumbar spread, while bladder and bowel symptoms were due to the sacral spread of the tumour. The cranial symptoms preceded quadriparesis, which preceded urinary symptoms. This sequence suggests primary craniospinal subarachnoid progression of tumour, since our case had no surgical intervention. Surprisingly, the CSF examination did not detect any malignant cells. The patient had no symptoms of hypopituitarism and no metastases were detected by USG or on chest x-ray.

On CT scan, chordoma presents as a destructive bone lesion with periosteal elevation and a solid-cystic soft tissue mass with foci of calcification.[1],[6],[7] The chondroid variety calcifies more frequently than the conventional type.[1] On MRI, it is hyperintense and enhances heterogeneously with contrast material.[7] Myelography is preferred to MRI for diagnosis of subarachnoid nodules, since there may be loss of contrast between lesions and CSF on MRI.[4] In our case, the tumour did not form a tumour mass, but encased the base of brain like a flat sheet resembling arachnoiditis. Perhaps, this is why it was not picked up on CT scan.

Differential diagnoses of skull base chondroid chordoma include myxoid chondrosarcoma, chordoid meningioma, metastases of signet ring adenocarcinoma and chordoid glioma. Light microscopic features and immunohistochemistry can differentiate between these entities.[1],[8],[9],[10]. Chondroid chordoma presents commonly in young females, has a lower recurrence rate and better survival as compared to conventional chordoma.[1] This prognosis is attributed to chondroid differentiation in notochord.[1] Our case however showed a relatively rapid decline.

The present case highlights a rare presentation of chondroid chordoma as flat sheet-like encasing of base of brain resembling arachnoiditis. The tumour disseminated through the subarachnoid space along the craniospinal axis in the absence of any interventional procedures that could have initiated metastases. Compression and infiltration of the spinal cord with multiplicity of symptoms in time and space made it mimic a demyelinating disorder. Clinical awareness of this mode of presentation will prevent delay in diagnosis and treatment.

 
 :: References Top

1.Heffelfinger MJ, Dahlin DC, MacCarty CS, Beabout JW. Chordomas and cartilaginous tumors at skull base. Cancer 1973;32:410-20.   Back to cited text no. 1    
2.Uggowitzer MM, Kugler C, Groell R, Lindbichler F, Redner H, Sutter B, et al . Drop metastases in a patient with a chondroid chordoma of the clivus. Neuroradiology 1999;41:504-7.   Back to cited text no. 2    
3.Hall WA, Clark HB. Sacrococcygeal chordoma metastatic to the brain with review of the literature. J Neuro Oncol 1995;25:155-9.   Back to cited text no. 3    
4.Krol G, Sze G, Arbit E, Marcove R, Sundaresan N. Intradural metastases of chordoma. AJNR 1989;10:193-5.   Back to cited text no. 4    
5.Scartozzi R, Couch M, Sciubba J. Chondroid chordoma of the nasal septum. Arch Otolarygol Head Neck Surg 2003;129:244-6.   Back to cited text no. 5    
6.Steenberghs J, Kiekens C, Menten J, Monstrey J. Intradural chordoma without bone involvement. J Neurosurg Spine 2002;97:94-7.   Back to cited text no. 6    
7.Meyers SP, Hirsch WL, Curtin HD, Barnes L, Sekhar LN, Sen C. Chordomas of the skull base: MR features. Am J Neuroradiol 1992;13:1627-36.   Back to cited text no. 7    
8.Rosenberg AE, Brown GA, Bhan AK, Lee JM. Chondroid Chordoma-A Variant of Chordoma-Morphologic and Immunohistochemical study. Am J Clin Pathol 1994;101:36-41.   Back to cited text no. 8    
9.Couce ME, Aker FV, Scheithauer BW. Chordoid Meningioma A clinicopathologic study of 42 cases. Am J Surg Pathol 2000;24:899-905.   Back to cited text no. 9    
10.Pasquier B, Peoc'h M, Morrison AL, Gay E, Pasquier D, Grand S, et al . Chordoid Glioma of the third ventricle. Am J Surg Pathol 2002;26:1330-42.  Back to cited text no. 10    


Figures

[Figure - 1], [Figure - 2], [Figure - 3]

This article has been cited by
1 Intradural drop metastasis of a clival chordoma
Martin, M.P., Olson, S.
Journal of Clinical Neuroscience. 2009; 16(8): 1105-1107
[Pubmed]
2 Prostate cancer metastasis to clivus causing cranial nerve VI palsy
Malloy, K.A.
Optometry. 2007; 78(2): 55-62
[Pubmed]



 

Top
Print this article  Email this article
Previous article Next article
Online since 12th February '04
2004 - Journal of Postgraduate Medicine
Official Publication of the Staff Society of the Seth GS Medical College and KEM Hospital, Mumbai, India
Published by Wolters Kluwer - Medknow