Journal ofNeurology, Neurosurgery, and Psychiatry, 1977, 40, 790-794

Giant cell tumour of the sphenoid bone withdural extensionSAMUEL C. OHAEGBULAM AND INDU M. GUPTA

From the Neurosurgical Unit, Department of Surgery and Department of Morbid Anatomy,University of Nigeria Teaching Hospital, Enugu, Nigeria

S U M M A R Y A case of giant cell tumour of the sphenoid bone is reported. Besides the familiarpresentation with headaches, ocular manifestations, and upper cranial nerve lesions, this caseshowed previously undescribed features such as grand mal seizures, hemiparesis with facial palsy,and dural extension. The reported high incidence in females is questioned. The tumour histo-logically showed some cartilage.

The rarity of giant cell tumours of the sphenoidbone has been emphasised by previous authors,(Echols, 1945; McNerney, 1949; Handousa, 1951;Ramamurthi et al., 1955; Kanaka et al., 1966).Giant cell tumours of bone (osteoclastomas) arebelieved to arise from non-osteogenic stromal cellsof the bone marrow near the epiphysis. Most ofthem are found in the epiphyseal region of longbones. Smaller bones like those of the hand andfoot, ribs, mandible, scapula, and the vertebrae,may also be the seat of this tumour. Giant celltumours are very uncommon in the head and,when they occur, they tend to show predilectionfor the mandible and the maxilla (Geshickter andCopeland, 1949). The case we are reporting hereis the sixteenth so far published in Englishliterature.

Case report

HISTORYA 25 year old male patient was referred to theUniversity of Nigeria Teaching Hospital com-plaining of headaches, blurring of vision, pain inthe left eye, and heaviness of his right limbs. Thepatient first developed left hemicranial headachesabout eight months before his presentation to thehospital. These were later associated with nauseaand vomiting. Several weeks after the onset of theheadaches, he complained of blurring of visionand pain in the left eye. Then he noted heavinessand clumsiness of his right limbs. Two monthsbefore admission he experienced definite weakness

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of the right half of his body. For several months,he had exhibited episodes of confusion, and hehad had several grand mal epileptic attacks. Onadmission to hospital, he was so drowsy and con-fused that he could not 'tell his own story.

EXAMINATIONThere was a conspicuous prominence of the lefttemporal region. He was drowsy, confused, anddysphasic. His vision was about 6/36 on the rightside, and he could only count fingers with the lefteye. He had bilateral papilloedema which wasmore severe on the left side. The left pupil wasslightly dilated and sluggish in reaction. There wassupranuclear 7th cranial nerve palsy on the rightside and slight impairment of hearing on the leftside. He had a right spastic hemiparesis with ex-aggerated reflexes and an equivocal plantar re-sponse. Romberg's test was positive, and thepatient tended to fall to the right side.

INVESTIGATIONRoutine urine, stool, and blood analysis were nor-mal. Serum calcium was 2.45 mmol/l (9.8 mg/dl).Radiology of the skull showed destruction of thesella turcica and rarefaction of the left temporalbone (Fig. 1). Left carotid angiography showeddisplacement and stretching of the middle cerebralartery consistent with a temporal space occupyinglesion. A tumour blush was also visualised (Fig. 2).

TREATMENTThe left external carotid artery was ligated in theneck before a left fronto-temporal craniotomywas performed to expose an extradural fleshy,

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Giant cell tumour of the sphenoid bone with dural extension

Fig. 1 Lateral radiograph of the skull showingdestruction of the sella turcica.

vascular tumour in the region of the sphenoidridge. The intracranial part of the tumour wasexcised but it became obvious that complete re-moval would be impossible as the tumour extendedmedially to the body of the sphenoid bone. It wasalso invading the dura mater adjacent to thetumour. The dura mater which was infiltrated bythe tumour was excised. In the postoperativeperiod, there was complete neurological recoveryand the patient was ambulatory and asympto-matic The patient was advised to undergo radio-therapy in view of the pathological diagnosis ofgiant cell tumour of the sphenoid, and was dis-charged home two weeks after surgery on anti-convulsant therapy. However, the patient onlyresorted to radiotherapy eight months later. Atthe completion of the course of radiotherapy inanother hospital, he had an attack of grand malepilepsy. Nine months later, he was readmittedto our hospital and was still found to have noneurological deficit and to be asymptomatic. Hehas been followed up for one year and has re-mained the same.

PATHOLOGICAL EXAMINATION OF THE TUMOUR

The excised tumour consisted of several irregulargreyish pieces of tissue with tiny gritty areas. Intwo bits, dura mater could be identified on grossinspection. Microscopically, the tumour showed auniformly similar picture of tumour giant cellswith abundant stromal cells, many of them inmitosis. Spicules of destroyed bone were evidentin between. The tumour was richly vascularised

(a)

Fig. (a) AP view of left carotid angiogram showingstretching and medial displacement of the middlecerebral artery. The anterior cerebral artery did notfill. (b) Lateral view of the same angiogram showingstretching of terminal portion of the internal carotidand upward displacement of the middle cerebralartery. A tumour blush is visible in the temporalregion.

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Samuel C. Ohaegbulam and Indu M. Gupta

and haemorrhagic. In areas adjacent to thethickened collagenous dura mater the tumourgiant cells were closely opposed to it and variously

infiltrating it. Considerable hyalinising areas to-gether with areas simulating cartilage with spottycalcification, not unlike those seen in a chondro-blastoma, alternated with large areas made up ofcharacteristic giant cell tumour with 'osteoclastic'giant cells throughout the tumour tissue (Fig. 3a,b). In none of the sections were there features ofstromal sarcomatous reaction to suggest osteo-sarcoma.

Discussion

Previous authors have reviewed the literature(Geissinger et al., 1970; Emley, 1971; Gupta et al.,1975) and have made some observations about theclinical presentation, pathology, and treatment ofthis tumour. Their conclusions are re-examinedhere in the light of our present knowledge of thistumour.

AGE AND SEX INCIDENCEAbout 10 of the 16 cases so far reported occurredin the second and third decades of life, in agree-ment with the conclusions of Hutter et al. (1962)who found in their series of 76 cases of benigngiant cell tumours of other sites, that the meanage was 23 years. The patient reported here is25 years old. In 1970, female patients constituted72.72% of the 11 cases reported at that time(Geissinger et al., 1970). In 1975 (Gupta et al.),that figure dropped to 66.6%. If the case we re-

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Fig. 3 (a) Photomicrograph illustrating characteristic giant cell tumour.Note stromal celts with interspersed numerous giant cells. Haematoxylinand eosin, X150. (b) Photomicrograph showing large hyalinising areas withspotty calcification and giant tumour cells. Haematoxylin and eosin, X150.

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Giant cell tumour of the sphenoid bone with dural extension

ported here is included, the female percentagedrops to 62.5. It appears, therefore, that the sexincidence is still unknown, and any comparisonwith the sex incidence of giant cell tumours ingeneral is premature from the limited number ofcases so far on record.

CLINICAL PRESENTATIONHeadache and cranial nerve lesions have beencommon modes of presentation of giant cell tu-mours of the sphenoid. The headache has tendedto antedate other symptoms by several months(Geissinger et al., 1970). Blurring of vision, oculo-motor, trochlear, and abducent nerve lesions areother common signs. Trigeminal nerve paresis,proptosis, endocrine disturbances, nasopharyngealmass, nasal obstruction, nausea and vomiting, re-troorbital pain, and olfactory hallucinations havealso been reported. Supranuclear facial palsy,hemiparesis, and grand mal epilepsy are additionalsigns which, to our knowledge, have never beenencountered in cases of giant cell tumour of thesphenoid bone. The case reported here is probablythe first to show these signs.

RADIOLOGICAL STUDIESPlain radiographs of the skull revealed destructionof the sella turcica in almost all cases in whichthis investigation was carried out including ourcase. Carotid and vertebral angiography as wellas pneumoencephalography were performed inonly a few cases, and were reported to have con-tributed little more than what was already knownfrom the plain skull films. However, in ourpatient, carotid angiography was very helpful indefining the intracranial part of the tumour, andthe information gained was vital in planningtherapy.

TREATMENTThe treatment of this tumour has varied fromexcision to mere biopsy followed by radiotherapy.Most authors, probably influenced by the goodresponse of the giant cell tumours of long bonesand jaw to irradiation (Friedman and Pearlman,1968), recommend biopsy and radiotherapy in thetreatment of giant cell tumour of the sphenoidbone. Although, in most cases, the tumour or partof it is inaccessible for total surgical removal,data so far available permit only cautious opti-mism about the response of this variety of giantcell tumour to radiotherapy. In the two casesreported by Pitkethly and Kempe (1969), no neuro-logical progression was observed two years and sixmonths respectively after biopsy or partial excision

and radiotherapy. Geissinger et al. (1970) reportedthat their patient, after biopsy and radiotherapy,remained asymptomatic for seven months.Michael (1959) and Eller et al. (1968) reportedthat after partial excision of tumour and radio-therapy their patients showed recalcification ofthe sella turcica after four years and three years re-spectively though cranial nerve palsies persisted.However, the patient reported by Peimer (1954)who underwent radiotherapy after biopsy, hadprogressive tumour 20 months later. Gupta et al.(1975) reported that their patient at first respondedto radiotherapy but three weeks later deterioratedto the extent that surgical excision became neces-sary. These conflicting results suggest that thenatural history of the sphenoid variety of giantcell tumour is yet unknown and is probablydifferent from the giant cell tumour of the longbones and the mandible.Our patient remained asymptomatic and neuro-

logically normal for eight months after subtotalexcision of his tumour before radiotherapy wasgiven. In the absence of neurological deficit, theonly index for monitoring his response to thistreatment is the state of recalcification of thesella turcica.

PATHOLOGYHistologically, giant cell tumours of the sphenoiddo not differ in any material way from those en-countered elsewhere in the body. Profuse bleedingduring biopsy was a feature in an earlier case(Gupta et al., 1975), and also characterised thepresent one during surgery. A significant point ofinterest in the present case lay in excluding thetheoretical possibility of giant cell tumour arisingfrom dura mater (meningeal), in view of theextensive dural involvement clinically mimickingmeningioma. Osteoblastic meningiomas are wellknown (Russell and Rubinstein, 1971), and whenit is considered that dura mater is capable ofosteoblastic differentiation in meningiomas, it isconceivable that a tumour of dural origin coulddifferentiate along the line of a giant cell tumour.From the biopsy material alone primary duralgiant cell tumour was suspected but was ruled outlater from the radiological and operative findings.Russell and Rubinstein (1971) have described amalignant giant cell tumour 'osteoclastoma' ofthe left parietal region which penetrated the duramater and adjacent cortex. In none of the giantcell tumours of sphenoid bone previously reportedin the literature was there any mention of duralinvolvement.

Cartilaginous differentiation in the tumour

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Samuel C. Ohaegbulam and Indu M. Guptatissue is certainly rare and makes one wonderwhether the tumour could be categorised as theso-called 'chondromatous' variant of giant celltumour (Codman, 1931).

The authors gratefully acknowledge the assistanceof Dr J. T. K. Duncan, Lagos University Teach-ing Hospital, Nigeria in carrying out the radio-therapy and Mr A. Brooks of the University ofNigeria in preparing the illustrations.

References

Codman, E. A. (1931). Epiphyseal chondromatousgiant cell tumours of the upper end of thehumerus. Surgery., Gynaecology and Obstetrics, 52,543-548.

Echols, D. H. (1945). Giant cell tumor of the sphenoidbone-report of a case. Journal of Neurosurgery,2, 16-20.

Eller, J. L., Deckler, J. T., and Brittis, A. L. (1968).Roentgen therapy for a giant cell tumor of thesphenoid bone. A case report. Radiology Clinics ofNorth America, 37, 36-44.

Emley, W. E. (1971). Giant cell tumor of thesphenoid bone. A case report and review of theliterature. Archives of Otolaryngology, 94, 369-374.

Friedman, M., and Pearlman, A. W. (1968). Benigngiant cell tumor of bone: radiation dosage for eachtype. Radiology, 91, 1151-1158.

Geissinger, J. D., Siqueira, E. B., and Ross, E. R.(1970). Giant cell tumors of the sphenoid bone.Journal of Neurosurgery, 32, 665-670.

Geschickter, C. F., and Copeland, M. M. (1949).Tumors of Bone. 3rd Edition, pp. 288-323. J. B.Lippencott Co: Philadelphia.

Gupta, I. M., Gupta, 0. P., and Samant, H. C. (1975).Giant cell tumor of the sphenoid bone. A nnals ofOtology, Rhinology and Laryngology, 84, 359-363.

Handousa, A. B. (1951). Osteoclastoma in relationto the nose. Journal of Laryngology and Otology,65, 549-559.

Hutter, R. V. P., Worcester, J. N., Jr., and Francis,K. C. (1962). Benign and malignant giant celltumors of bone: A clinico-pathological analvsis ofthe natural history of the disease. Cancer, 15, 653-690.

Kanaka, T. S., and Balasubramaniam, V. (1966). Giantcell tumour of the skull. Neurology (India), 14,57-60.

McNerney, J. C. (1949). Giant cell tumor of bonesof the skull. Journal of Neurosurgery, 6, 169-174.

Michael, L. A. (1959). Giant cell tumor of the sinuszs.Laryngoscope (St Louis), 69, 320-328.

Peimer, R. (1954). Benign giant cell tumors of theskull and nasal sinuses. Archives of Otolaryngology,60, 186-193.

Pitkethly, D. T., and Kempe, G. L. (1969). Giantcell tumors of the sphenoid. Journal of Neuro-surgery, 30, 301-304.

Ramamurthi, B., Visvanathan, G. S., and Pillai, K. M.(1955). Osteoclastoma of the skull. Journal ofNeurosurgery, 12, 287-290.

Russell, D. S., and Rubinstein, L. J. (1971). Path-ology of Tumours of the Nervous System. 3rdEdition, pp. 60 and 262. Edward Arnold (Publishers)Ltd.: London.

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