Neurology India Medknow Publications on behalf of the Neurological Society of India ISSN: 0028-3886 EISSN: 1998-4022 Vol. 58, Num. 6, 2010, pp. 971-972

Neurology India, Vol. 58, No. 6, November-December, 2010, pp. 971-972

Letter to Editor

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

Ravi Dadlani¹, Nandita Ghosal², AS Hegde¹

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
Correspondence Address: Ravi Dadlani, Department of Neurosurgery, Sri Satya Sai Institute of Higher Medical Sciences, Bangalore, India, ravi.dadlani@gmail.com

Date of Acceptance: 01-Sep-2010

Code Number: ni10278

PMID: 21150080
DOI: 10.4103/0028-3886.73776

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.

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.

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