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 »  Abstract
 »  Introduction
 »  Case report
 »  Discussion
 »  References

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Year : 2001  |  Volume : 49  |  Issue : 2  |  Page : 208-10

Giant orbital and intracranial xanthogranuloma--a short report.

26, II Main Road, C.I.T. Colony, Mylapore, Chennai, 600004, India.

Correspondence Address:
26, II Main Road, C.I.T. Colony, Mylapore, Chennai, 600004, India.

  »  Abstract

Xanthogranuloma are known to arise in the paranasal sinus or orbit. They may also arise primarily in the brain. Those arising from the sinuses or orbit might involve the intracranial cavity to some extent. But an extensive involvement of the cranial compartment is very rare. This report describes one such case.

How to cite this article:
Chidambaram B, Santosh V. Giant orbital and intracranial xanthogranuloma--a short report. Neurol India 2001;49:208

How to cite this URL:
Chidambaram B, Santosh V. Giant orbital and intracranial xanthogranuloma--a short report. Neurol India [serial online] 2001 [cited 2021 Jun 17];49:208. Available from:

   »   Introduction Top

Xanthogranuloma has been recognised as a primary skin lesion for quite sometime. It was only much later that involvement of eye by xanthogranuloma was recognised. Lesions of the orbit, without involvement of the globe, are very uncommon. They may arise in the sinuses too. Occasionally the nervous system may be affected. We are reporting a giant xanthogranuloma with massive intracranial extension.

   »   Case report Top

A 15 years male was referred by an ophthalmologist for increasing proptosis of right eye. There was no other complaint. Examination of the affected eye revealed moderate proptosis. The eyeball had been pushed laterally and downwards. Only elevation and adduction were restricted. Visual acuity was 6/18. There was bilateral papilloedema. The left eye was otherwise normal. CT and MRI revealed a large lesion involving the orbit, the ethmoidal sinuses and the anterior cranial fossa. There were areas of varying densities [Figure - 1]. Through a bicoronal scalp incision frontal craniotomy was done. The roof of orbit had been eroded in many places, by the tumour. On bone reflection, the tumour was found to have infiltrated the bone. The outer table was also involved. It was rough and showed evidence of tumour infiltration. The tumour was vascular, reddish grey and suckable only in certain portions. It had eroded the roof of orbit and had extended extensively inside the orbit. The tumour capsule was opened and debulking was done. The
capsule was mobilised away from the dura. The dura was intact and had not been breached in any place. The tumour capsule was mobilised away from the dura all around, and was removed. Thus the entire intracranial portion of the tumour could be removed. The frozen section of tissue was interpreted as malignant tumour of undetermined origin. Hence, it was decided to limit the exploration to within the intracranial compartment. Only a small portion of the intraorbital extension was removed. The histology, however later showed 'a xanthomatous lesion, divided into lobules by fibrous septae and vascular channels.
Intermingled with these were many plasma cells, lymphocytes and giant cells. In an occasional giant cell, phagocytosis of lymphocyte was evident'. The histological features were characteristic of xanthogranuloma [Figure - 2].

   »   Discussion Top

Xanthogranuloma is primarily a skin condition. The skin lesions are usually multiple and pigmented yellow and they have a tendency to resolve spontaneously. Because of the comparatively greater incidence in children, they have also been called 'juvenile xanthogranuloma' by Helwig and Hackney.[1] The lesions (in the skin or elsewhere) can occur in association with various systemic granulomatosis like Endheim Chester disease, Weber Christian disease, Hand Schuller Christian syndrome etc. They may be associated with hypercholesterolaemia. Some distinguish a normocholesterolaemic xanthogranuloma and a hypercholesterolaemic variety.[2] Sometimes the lesions involve structures other than skin, like lung etc. Eye is the commonest region involved among the non dermatological structures. Xanthogranuloma of the eye may be in the globe or the orbit. Lesions arising in the globe are usually from iris or ciliary body.
Xanthogranulomata of the orbit are rare, and bony erosion is still rarer. Zimmerman reported on five cases of orbital lesions, and in one of them the bone was eroded.[3] Saunders and Miller detailed three cases of orbital involvement. One of them had destruction of the posterior lateral wall of the orbit.[4] Shields et al[5] reported one case with orbital involvement without any bone change. Involvement of the brain and its coverings are also recorded, though not common. The lesions have been reported in connection with dura[6],[7] and brain parenchyma. More often they arise in the choroid plexus of lateral and 3rd ventricle.[8],[9],[10],[11] Lesions in the brain can be multiple.[12] They have been described in association with colloid cysts.[13] Rarer lesions include the Meckel's cave[14] and sacral roots.[15] In orbital tumours, destruction of bone may be associated with minimal infiltration into the ethmoid sinuses and anterior cranial fossa. However, massive extension into the anterior cranial fossa has not been reported. In the present case, the lesion had involved the orbit, and the ethmoidal sinuses. It had extended into the anterior cranial fossa, wherein it had attained a massive size. It is very unlikely to have arisen in the anterior cranial fossa connection with the dura and spread into the orbit. The management of these lesions is essentially expectant. But where the diagnosis is in doubt biopsy is mandatory. Though some claim good results with corticosteroids and/or radiation, the reports are mainly anecdotal. Spontaneous regression is slow and unpredictable. When there is a big mass, debulking or total removal is necessary.


  »   References Top

1.Helwig EB, Hackney VC : Juvenile xanthogranulomatous disease of infants and children. Am J Path 1954; 30 : 625-626.   Back to cited text no. 1    
2.Yamada H, Kurata H, Nomura K et al : Adult xanthogranulomatous intracranial lesion involving familial hypercholesterolemia. Jpn J Med1989; 28 : 757-761.   Back to cited text no. 2    
3.Zimmerman Le : Ocular lesions of juvenile xanthogranuloma. Trans Am Acad Ophthalmol Otolaryngol 1965; 69 : 412-442.   Back to cited text no. 3    
4.Sanders TE, Miller JE : Infantile xanthogranuloma of orbit. Trans Am Acad Ophthalmol Otolaryngal 1965; 69 : 458-464.   Back to cited text no. 4    
5.Shields CL, Shields JA, Bachanon HW : Solitary orbital involvement with juvenile xanthogranuloma. Arch Opt 1990; 108 : 1587-1589.   Back to cited text no. 5    
6.Jan Joom AB, Yakub B, Sharif HS : Intracranial xanthogranuloma of the dura in Hand - Schuller Christian Disease. Neurosurgery 1993; 78 : 297-300.   Back to cited text no. 6    
7.Pick P, Jean E, Horoupian D et al : Xanthogranuloma of the dura in systemic Weber Christian disease. Neurology 1983; 33 : 1067-1070.   Back to cited text no. 7    
8.Schultz KD Jr, Petronio J, Narad C et al : Solitary intracerebral juvenile xanthogranuloma. Case report and review of the literature. Pediatr Neurosurg 1997; 26 : 315-321.   Back to cited text no. 8    
9.Hicks MJ, Albrecht S, Trask T et al : Symptomatic choroid plexus xanthogranuloma of the lateral ventricle. Case report and brief critical review of xanthogranulomatous lesions of the brain. Clin Neuropathol 1993; 12 : 92-96.   Back to cited text no. 9    
10.Kadota T. Mihara N, Tsuji N et al : MR of xanthogranuloma of the choroid plexus. Am J Neuroradiol 1996; 17 : 1595-1597.   Back to cited text no. 10    
11.Muenchau A, Lass R : Xanthogranuloma and xanthoma of the choroid plexus: evidence for different etiology and pathogenesis. Clin Neuropathol 1997; 16 : 72-76.   Back to cited text no. 11    
12.Nagatani K, Waga S, Takeuchi J et al : Multiple intracranial xanthogranulomas - case report. Neurol Med Chir (Tokyo) 1990; 30 : 960-965.   Back to cited text no. 12    
13.Kudesia S, Das S, Shankar SK et al : Colloid cyst xanthogranuloma of the third ventricle - a case report. Indian J Pathol Microbiol 1996; 39 : 221-223.   Back to cited text no. 13    
14.Kimura H, Oka K, Nakayama Y et al : Xanthoma in Meckel's cave. A case report. Surg Neurol 1991; 35 : 317-320.   Back to cited text no. 14    
15.Kitchen ND, Davies MS, Taylor W : Juvenile xanthogranuloma of nerve root origin. Br J Neurosurg 1997; 9 : 233-237.   Back to cited text no. 15    


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