Dorsal spine intradural extramedullary hemangiopericytoma: a case report

Abstract

Introduction: Hemangiopericytomas are rare CNS Neoplasms constituting <1% of CNS tumours. They are more commonly seen in cranium than in spine where they present as Intradural extramedullary in location. They rarely metastasize to Lung and Bone.World Health Organization Classification of Tumors of the Central Nervous System (CNS WHO) assigned the combined term solitary fibrous tumor/ hemangiopericytoma (SFT/HPC) to such lesions in 2016 due to their same genetic makeup. Case report: Our case is about a 61-year-old diabetic male who presented with insidious onset, gradually progressive thoracic backache and asymmetric weakness with tightness and numbness of both lower limbs for last 6 months with no sphincter incontinence and bed bound for last 15 days. Clinically he had asymmetric spastic paraparesis of lower limb. His MRI Dorsal spine showed a 1.5 ґ 2 cm heterogeneously contrast enhancing Intradural lesion at D6-D7 compressing the cord from dorsolaterally with extensive cord edema from C5 to L2. Preoperative diagnosis of Meningioma/ Metastasis was made and patient underwentD5-D7 laminectomy and tumour excision. Intraoperative findings, there was no dural attachment, there were multiple tortuous blood vessels overlying greyish pink, fleshy, suckable, highly vascular, capsulated tumor posterolateral to cord. The superior aspect of tumor was adherent to cord with no clear interface. Tumor was seen separate from Left D7 nerve root. Post operatively patient improved symptomatically and neurologically. His HPE was suggestive of Hemangiopericytoma WHO Grade II (IHC markers CD34 and CD 31 was positive and KI 67% index of 10-15%). Patient has been on follow up and planned for metastatic workup. Conclusion: Spinal Hemangiopericytoma are rare disease with nonspecific radiological features making preoperative diagnosis difficult. Differential diagnosis of hemangiopericytoma should be kept in case of atypical and high - grade radiological features. Gross total resection without compromising the neurological status should be the goal to prevent local recurrence. Proper consensus regarding the follow up and adjuvant therapies of spinal Hemangiopericytoma are still lacking.