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LETTER TO EDITOR
Year : 2010  |  Volume : 58  |  Issue : 6  |  Page : 971-972

Unusual primary intradural and extradural thoracic spinal hemangiopericytoma: Radiological, diagnostic and therapeutic dilemmas


1 Department of Neurosurgery, Sri Satya Sai Institute of Higher Medical Sciences, Bangalore, India
2 Department of Pathology, Sri Satya Sai Institute of Higher Medical Sciences, Bangalore, India

Date of Acceptance01-Sep-2010
Date of Web Publication10-Dec-2010

Correspondence Address:
Ravi Dadlani
Department of Neurosurgery, Sri Satya Sai Institute of Higher Medical Sciences, Bangalore
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.73776

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How to cite this article:
Dadlani R, Ghosal N, Hegde A S. Unusual primary intradural and extradural thoracic spinal hemangiopericytoma: Radiological, diagnostic and therapeutic dilemmas. Neurol India 2010;58:971-2

How to cite this URL:
Dadlani R, Ghosal N, Hegde A S. Unusual primary intradural and extradural thoracic spinal hemangiopericytoma: Radiological, diagnostic and therapeutic dilemmas. Neurol India [serial online] 2010 [cited 2021 Jun 23];58:971-2. Available from: https://www.neurologyindia.com/text.asp?2010/58/6/971/73776


Sir,

Hemangiopericytomas are vascular tumors derived from pericytes. [1] Spinal hemangiopericytomas are extremely rare. Of the 39 cases reported, only 6 have been intradural. [2],[3] We report an extremely rare case of a mid-thoracic primary spinal hemangiopericytoma with both intradural and extradural components without an obvious dural breech. This probably the first report of such a presentation of a spinal hemangiopericytoma.

A 38-year-old lady presented with a history of progressive paraparesis and hypoesthesia of 50% to all sensory modalities below the D6 level bilaterally. She had no involvement of bladder or bowel.

Preoperative magnetic resonance imaging (MRI) of the spine revealed a well-defined spinal lesion, extending from T5 to T7, hypointense on T1-weighted images and hyperintense on T2-weighted images. There was uniform contrast enhancement of the lesion [[Figure 1]a-c]. The dura is seen as a thin iso-intense line running through the lesion both on coronal and axial images [[Figure 1]b and c]. Although in retrospect this is identified as the dura, preoperatively this finding was overlooked both by the radiologist and the surgeon. Intraoperatively the lesion was very vascular and extradural, extending to the neural foramen along 3 nerve roots. The dural tube was displaced to the right. On decompression, the dural tube realigned to the center. Durotomy was deferred since there was no evidence of any dural breech and the intraoperative ultrasonographical visualization of the lesion was poor, probably because the intradural lesion was placed anterior to the spinal cord. Postoperatively the motor power improved by about 1 grade, and repeat imaging [[Figure 1]d and e] revealed residual intradural tumor. The patient was subjected to a re-exploration, and the residual intradural tumor was excised completely [[Figure 2]a and b]. Subsequent to the second surgery, her spasticity reduced and paraparesis improved to grade 4+ and she was able to walk with assistance at the time of discharge from the hospital. Histopathology examination revealed a typical appearance of a hemangiopericytoma on H and E staining and immunohistochemistry [Figure 3]. The patient has received adjuvant radiotherapy and has been on follow-up since surgery. She has not demonstrated any recurrence in the past 1 year.
Figure 1: (a), (b) and (c) are mid-sagittal, coronal and axial T1W post-contrast preoperative (magnetic resonance) MR images, respectively. In (a), a well-defined spinal lesion is seen at the mid-thoracic level with extension along the exiting nerve roots. White arrows in (b) and (c) represent the faint dural outline which was overlooked at first surgery. (d) (sagittal) and (e) (axial) are also T1W post-contrast images after the first surgery, revealing the residual intradural component of the tumor

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Figure 2: (a, b) T1W post-contrast MR images after the second surgery. They demonstrate complete excision of the lesion

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Figure 3: (a) Smear preparation of hemangiopericytoma showing tumor cells arranged around blood vessels (arrow) (hematoxylin and eosin stain, ×400). (b) Paraffin section of hemangiopericytoma (hematoxylin and eosin stain, ×100) showing (inset) prominent vascularity with a few tumor cells immunoreactive for vimentin (Avidin biotin peroxidase method, ×400). (c) High-power view of the tumor (hematoxylin and eosin stain, ×400) showing (inset) immunonegativity for epithelial membrane antigen (Avidin biotin peroxidase method, ×400). (d) Tumor cells showing rich pericellular reticulin network (reticulin stain, ×400)

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There have been reports of extradural hemangiopericytomas arising primarily from bone and of others that arise from dura and invade the extradural space [3] and others invading adjacent anatomical spaces; sub-pial to subdural [4] and from subdural to intramedullary. [5] Of these six patients only one has been reported to occur in the thoracic spine. [3] Recurrence rates in both types are similar but disease-free survival appears to be greater in the group with intradural lesions. [3] The radiology is usually described as nonspecific. Hemangiopericytomas although being malignant lesions are amenable to complete excision. Every effort must be made to differentiate this entity from other more benign conditions such as meningiomas, schwannomas and solitary fibrous tumors. [4] Radiotherapy has been used as adjuvant therapy, [3] but in view of the small number of case reports, conclusive data is lacking. The importance of identifying this entity lies in the fact that extensive scrutiny of the preoperative imaging is a must in identifying rare intradural pathologies. Hemangiopericytomas have neither any distinctive imaging nor can they be identified conclusively during surgery. Although until now these tumors were recognized either intradurally or extradurally, this case demonstrates the unusual occurrence in both compartments without an obvious dural breech. A high degree of suspicion should be maintained and a conscious effort must be made to identify subtle radiological findings.

 
  References Top

1.Harris DJ, Fornasier VL, Livingston KE. Hemangiopericytoma of the spinal canal: Report of three cases. J Neurosurg 1978;49:914-20  Back to cited text no. 1
    
2.Fitzpatrick D, Mahajan J, Lewkowitz M, Black K, Setton A, Woldenberg R. Intradural hemangiopericytoma of the lumbar spine: A rare entity. AJNR Am J Neuroradiol 2009;30:152-4.  Back to cited text no. 2
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3.Betchen S, Schwartz A, Black C, Post K. Intradural hemangiopericytoma of the lumbar spine: case report. Neurosurgery 2002;50:654-7.  Back to cited text no. 3
[PUBMED]  [FULLTEXT]  
4.Kashiwazaki D, Hida K, Yano S, Seki T, Iwasaki Y. Subpial hemangiopericytoma with marked extramedullary growth: Case report. Neurosurgery 2007;61:1336-7.  Back to cited text no. 4
    
5.Chou CW, Hsu SP, Lin SC, Chen MH, Shih YH, Lee LS, et al. Primary intradural hemangiopericytoma with intramedullary invasion. J Chin Med Assoc 2009;72:536-41.  Back to cited text no. 5
[PUBMED]  [FULLTEXT]  


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