Turkish Neurosurgery 4: 119 - 131. 1994 Gök: Spinal Haemangioblastoma and Imaging

Spinal Cord Haemangioblastoma and MR Imaging

ABDÜLVAHAP GÖK. SUZAN ZORLUDEMIR. MUSTAFA YILMAZ

Gaziantep University. Medical Faculty. Department of Neurosurgery. (AG) and Department of Neurology (MY).Çukurova University. Medical Faculty. Department of Pathology (SZ)

Abstract: Haemangioblastomas are true vascular neoplasms whichprogress inexorably to a catastrophic clinical situation if left un­treated. Theyare mostly intramedullary tumours rarely seen inthe extramedullary space.An unusual ease with a solitary spinaleord haemangioblastoma loeated purely in the intradural

INTRODUCTION

Haemangioblastoma consists of a proliferatian ofvascular spaces and endothelial cells separated by fat­laden stromal cells(4). It occurs as a solitary vascularlesian or assodated with von Hippel-Lindau's Diseasewhich is a heredofamilial disease transmitted by anautosomal dominant gene and characterized byvascular tumours of the retina and central nervous

system or cysts or tumours of the kidney. pancreasor lung(2.10.16.20).

It comprises approximately 2 to 3 % of primaryspinal cord neoplasms(20).Most of these tumours areloeated in the intramedullary space (14.15.18.20)andrarely in the extramedullary space(14. 19).Myelography. selective angiography and MRI aid inestablishing thE' diagnosis (5.6.11.14.20).

Cas e Report

This 45-year-old female presented with a historyof pain in the thorade region and difficulty in walk­ing for 6 months.On neurological examination therewas significant weakness in both upper ex­tremities.more on the right.and in both legs. Posteriorcolumn dysfunction including absent proprioceptionand a positive Romberg sign was observed. Sensorylevel to pinprick was at C5-6on the right. EvaIuatian

extramedullary spaee is presented. MR imaging accurately localizedthe mass and showed the prease delineation of the tumour afterGd-DTPA.

Key Words: Haemangioblastoma.Magnetie Resonanee

of the spinal area with MR demonstrated a massbrilliantly enhanced with the contrast Gd-DTPAdispladng the cord to the left side and localizing inthe intradural extramedullary space between C5 andT2 levels (Fig 1.Fig.2).

Fig. 1 : Pre-and post-eontrast magnetie resonanee images (sagittalview) showing the turnoral mass loealized betweenC6 andTl.

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Turkish Neurosurgery 4: 129 - 131. 1994

Fig. 2 : Post-contrast magnetic resonance image (corona] view).

showing the enhanced mass in the right side of theccrd and compressing it to the Jeft side.

Operation and Postoperative Course

The operation was performed in the prone posi­tion and total laminectomy was performed to theC6.C7.TI and T2 vertebrae.Under the operatingmicroscope the dura was opened longtudinaUy. Amass compressing the cord and displacing it to theleft side was observed in the subarachnoid space.Onthe pial surface there was a vessel coil composed offeeding arteries and a large draining vein at theaanial end of the tumour. The distalone third of the

tumour was captured by the posterior nerve fibersof thp CS root. The tumour was dark red and veryfragile. The dissection of the nerve fibers from thetumor surface was impossible due to massivehaemorrhage. so the nerve fibers were saaificed. thefeeding vessels coagulated and the draining vein wasinterrupted. The tumour was removed in one piece.The postoperative course was uneventful. After amonth there was only anaesthesia in the CS der­matome.

Pathological Hxamination

MaaoscopicaUy the tumor was 4xlx1.5 cm. The

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Gök: Spina] HaemangiobJastoma and Imaging

sectioned surface was spongy and red colour. Thesurgical specimen was med in LO percent formalinembedded in paraffin and stained with Hematoxylin­eosin. reticulin factor S.

Microscopically the tumour was composed oflarge numbers of dosely-packed blood vessels.linedby plump endothelial ceUs.There were a few hyper­co/0matic stromal ceUs between the capillary chan­nels (Fig 3). Histopathological diagnosis washaemangioblastoma.

Fig. 3 : The vascu1ar and stroma] ceJls are seen.H-E X 375

Discussion

Haemangioblastomas are Jare.benign vasculartumors of the central nervous system. They occur assolitary lesions (12,17,19) or assodated with vonHippel-Lindau's Disease(2.io.16). Theyare frequent­ly in assodation with cysts(9.13.IS) and more thanone lesion may be seen in the spinal cord at one time(2.9.IS). Syri,nx is present in approximately 70 % ofcases. pial varices in roughly 40 %. and root involve­ment in about 15% (20).Symptoms and signs are dueto cord compression and progress to paraparesis.posterior column dysfunction and sensory loss(11.1S.17). Haemangioblastomas may be foundanywhere in the spinal cord but occur mostly in thecervical and thoradc region s (5.S.9). it is noteworthythat subarachnoid haemorrhage in the unoperatedcases is extremely rare(7.12).

Plain X-Rays have no diagnostic value.but onmyelographic examination complete or partial blockmay be observed(i.S) and Myelo-CT maydemonstrate the extramedul1ary isodense mass at thesame level.

Turkish Neurosurgery 4: 129 - LJ1. 1994

The method of choice in diagnosis was selectiveangiography with catheterization of the arteries of thespina1 cord with the use of the subtraction techni­que (12.17.20).At present Magnetic Resonance Im­aging with Gd- DTPA enhancement is the most usefu1tool for predse localization and definition of thetumor margin and differentiation of any accompany­ing cyst(3.5.iU4). Once a spinallesion has beendemonstrated the assodation ofhaemangioblastomawith von Hippel- Lindau's disease should be madeto exdude cerebellar. brainstem and viscerallesions

(8.20).At the same time the entire spinal cord shouldbe examined to rule out multiplehaemangioblastomas.

The treatment of choice is total extirpation utiliz­ing microtechnique. These are highly vasculartumours and if acddent1y cut into or decompressed.bleeding not only obscures the anatomieal planes.butmay result in catastrophic problems(13). Remova1 canbe achieved with reasonable morbidity.

Correspondence: Abdülvahap GökGaziantep Tip Fakültesi.Nörosirürji Klinigi.27070 Kolejtepe. Gaziantep

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