Tiirkisli Neiirosiirgery 11: 138 - 141, 2001 Demirci: EosillOpliilic Grniiiiloinn of ilie Oplir Cliinsiii

Primary EosinophilicChiasm:

Granuloma of theCase Report

Optic

Optik Kiazmanin Primer Eozinofilik Granülomu:Olgu Sunumu

IRSADI DEMIRCI, SÜKRÜ AYKOL

Gazi University School of Medicine, Department of Neurosurgery, Ankara

Received: 26.10.1999 ~ Accepted : 22.12.1999

Abstract: it is extremely rare to see eosinophilic granulomaof the central nervous system without osseous ormultisystemic involvemenL Only two adult cases withisolated cranial nerve involvement have been reported toda te. This report describes an adult patient with isolatedeosinophilic granuloma of the optic chiasm. We suggestthat eosinophiIic granuloma should be included in thedifferential diagnosis of tumors involving the cranialnerves, and particularIy the optic chiasm.

Key words: Eosinophilic granuloma, histiocytosis X,cranial

INTRODUCTION

Eosinophilic granuloma (EG) is adisordercharacterized by chronic non-neoplasticgranulomas that contain proliferating histiocytes,plasma cel1s, and eosinophilic inflammatory cells.it is considered to be a unifocal or multifocal form

of the diseases associated with Langerhans' cel1histiocytosis. The etiology of EG remainsuncertain, but some believe that it involves anundefined immunologic disturbance caused by anunknown antigen (6). The lesions may be foundin the bone narrow, skin, oral mucosa, retro-orbitaltissue, lymph nodes, spleen, liver, lung,

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Özet: Kemik ve muItisistem tutulumu olmaksizin santral

sinir sisteminde eozinofilik granülomanin görülmesioldukca nadirdir. Simdiye kadar izole kranial sinirtutulumu sadece iki adult olguda bildirilmistir. Optikkiazmanin izole eozinofiIik granulomu olan bir adult olgutanimlandi ve cranial sinirleri, özellikle optik kiazmayitutan tümörIerin ayirici tanisinda düsünülmesi gerektigibelirtildi.

Anahtar kelimeler: EozinofiIik granuloma, histiositozisX, kranial

gastrointestinal tract, and central nervous system(CNS) (13).

In EG involving the CNS, the three mostcommon sites of occurrence are the cranial vault, thesuprasellar region, and the spinal column,respectively (1). it is extremely ra re to find EG in theCNS without any osseous or multisystemicinvolvement, and only two cases of isolated cranialnerve involvement have been published to date(13,24). In this report, we deseribe an unusual caseof primary EG of the optic chiasm, and stress the needto incIude this condition in the differential diagnosisfor lesions affecting the cranial nerves.

TiirkisJi Neiirosiirgery 11: 138 - 141, 2001

CASE REPORT

A 25-year-old woman presented with a historyof bilateral blurred vision of 4 months' duration. She

also reported having exaggerated thirst in theprevious 2 months, with the signs of polydipsia andpolyuria. There was no history of galactorrhea,amenorrhea, weight gain, sleep pattern disturbance,or changes in affective behavior or memory. Herphysical examination on admission was normal, buta neurological examination demonstrated markedbilateral optic atrophy, severely reduced visual acuityin both eyes CO.1),and bitemporal hemianopsia. Theresults of routine laboratory studies were normal,apart from an elevated erythrocyte sedimentationrate (43 mm/h) and eosinophilia (7%). She was alsodiagnosed with diabetes insipidus of central origin.

Radiological ExaminationCranial computerized tomography (CT)

revealed a 2x1.5 cm mass lesion in the suprasellarcistern. The lesi on was isodense on non-contrast

images, and showed marked contrast enhancemenLMagnetic resonance imaging (MRI) revealed that thelesion involved the optic chiasm. it was isointenseon Tl-weighted images and hyperintense on T2­weighted images. We also noted markedhomogeneous enhancement af ter the administrationof gadolinium diethylenetriamine penta-acetic acid(Gd-OTPA). MRI also show ed that the lesion

extended to the pre-chiasmatic and post-chiasmaticportions of the optic tract. The cranial portion of theinfundibulum could not be distinguished from theoptic chiasm or from the mass lesion itself, whereasthe caudal portion was normal in size and shape, andshowed a normal contrast enhancement pattern(Figure 1). Aneurysm was ruled out by magneticresonance angiography.

511rgical ProcedureThe lesion was approached via a right pterional

craniotomy. We opened the sylvian fissure and gentlyretracted the frontal lobe. On inspection, the opticchiasm was enlarged and appeared grayish brownin color. We dissected the optic chiasm free from theadjacent tissues, and made a midline incision in theoptic chiasm parallel to the nerve fascides. The lesi onwas firm, 2x1.5 cm, moderately vascularized massthat was invading the nerve tissue. There was nogrossly apparent hypothalamic involvemenLOperative di ss ec tion show ed that the tumor wasinterfering with the continuity of almost all the nervefascic1es in the chiasm. As a consequence, onlysubtotal resection was possible.

Demirci: EosiiiopJii/ic Gmiiii/oma of tJie üptic Ciiiasiii

Figure 1: Axia! and corona! post-contrast Tl-weightedimages and nuclear grooves in Langerhans' cells(H&E x400).

Patliological ExaminationThe resected specimen was so small that we

were unable to divide it for light and electronmicroscopic studies. Histological examinationrevealed a mixed inflammatory infiltrate indudinglymphocytes, plasma cells, eosinophils, andhistiocytes. The infiltrating cells tended to be denserne ar blood vessels. High-power magnificationshowed that some of the histiocytes had groove dnudei. Immunohistochemical studies revealed that

the histiocytes were positive for S-100, but negativefor glial fibrillary acetic protein (GFAP). On this basis,we labeled the m Langerhans' cells (Figure 1)

Treatment and Folloiu-upPostoperative neurological examination

revealed deficits identical to those found

preoperatively. Af ter the histopathological diagnosiswas established, the patient was assessed for signsof skeletal and systemic involvemenL She underwenthematological analysis, abdominal ultrasonograpy,CT, a skeletal radiographic survey, and radionudide

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TiirkisJi Neiiyosurgery 11: 138 - 141, 2001

bone scanning, but no abnormalities were found.Low-dose radiation therapy (20 Gy in 10 fractionsover 2 weeks) was administered, after which thepatient was discharged. Follow-up MRI at 2 monthspost-surgery showed no obvious decrease in the sizeof the mass. The patient died at 31 months aftersurgery due to brain invasion by the tumor.

DISCUSSION

Eosinophilic granuloma is part of the spectrumof diseases associated with Langerhans' cellhistiocytosis. Histiocytosis X is the term that was firstcoined by Lichtenstein in 1953 (8) to describe aheterogeneous group of disorders. Today, this groupis considered to include Hand-Schüller-Christiandisease, Letterer-Siwe disease, and EG of the bone (7).

Gagel first described involvement of the CNSin histiocytosis Xin 1941 (9).At that time, only a smaIlnumber of cases of primary EG in the CNS had beenreported. More recently, investigators havepublished larger series of patients with EG in theCNS, but these cases have also featured osseous ormultisystemic involvement (11,12,17). Thehypotharamus is of ten affected in cases ofdisseminated histiocytosis X, but isolatedhistiocytosis X of the hypothalamus is very rare(2,21,27).Infiltration of the hypothalamus or pituitarygland or both has been reported in 50% ofhistiocytosis X patients at autopsy (5,11,14,28).Oiabetes insipidus is an important initial symptomwhen this structure is involved (4). Similar to thecase reported by Smolik et al (24), in our patient thehypothalamus did not appear to be involved onradiological examination or at surgery.

Our patient' s preoperative CT scanning show edan isodense lesion with marked contrast

enhancement. There was no surrounding edema.These findings were similar to those noted in the caseof EG of the occulomotor nerve reported byHardenack et aL.(13). In previously described casesof solitary cerebral eosinophilic granuloma, thelesions have appeared either hypodense or isodenseon CT (4,11,20,23). In our case, MRI revealedisointensity on T1-weighted images andhyperintensity on T2-weighted images, and this wasin line with other reported findings. However, weobserved marked homogeneous enhancement of thelesion after Gd-OTPA administration. This contrastswith other authors' results (10,22),but confirms othermore recent observations (6,13,15). Initial1y, wesuspected that the mass was a glioma based on its

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Deiiiirci: EosiilOpJiilic Gml/iiloiiia of tJie Optic CJiias/ll

location, signal, and contrast enhancement pattern.Our findings indicate that the radiological featuresof intracranial EG are highly variable, and werecommend that diagnosis of this type of lesionshould not be based on radiological studies.

Since all the extirpated tissue was fixed informalin, it was not possible to performultrastructural studies and identify Langerhans' cells.Had it been possible to do electron microscopy, wemight have been abI e to demonstrate Birbeck'sgranules. However, the characteristic histological andimmunohistochemical findings were sufficient toestablish the diagnosis. These findings included thepresence of many eosinophils, grooves in thehistiocyte nuclei, cytoplasmic S-100 positivity, andGFAP negativity that excluded the possibility of aglial origin.

Treatment for EG remains controversial. Some

authors suggest observation only (19).In the literature,radio- or chemotherapy is generaIly not recommended,provided that the lesions are localized, and are totallyremoved surgicaIly (6).However, some researchers doadvocate radiotherapy after total excision (12).Although EG is moderately radiosensitive (21), theoptimal dose of radiation is still under debate. Formultifocal EG, one report has stated that neitherradiation nor steroid therapy is effectiye (8).Still, mostcases involving subtotal resection and biopsy, ormultifocal disease have been treated with radiotherapy0,21,27), radiotherapy plus chemotherapy (7) orchemotherapy alone (25,16). The effects ofchemotherapy and the optimal combination ofchemotherapeutic agents are also unclear (7). Smoliket aL.(24) administered a midline dose of radiationtherapy (2700 r over 3 weeks) to their patient's opticchiasm. FoIlow-up examination at approximately 8months post-surgery showed that this was effective.Low-dose radiation has also been reported to causeregression of hypothalamic EG (26). However, weobserved no positive effect of low-dose radiationtherapy at the early postoperative stages in ourpatient. She died at 31 months after surgery due tobrain invasion by the tumor.

The case we have presented contributes littlenew information to what is known about radiologicaldiagnosis, histological diagnosis, and therapeuticapproaches for EG involving the CNS or specificallythe optic chiasm. However, it does underline the needto include EG in the differential diagnosis for cranialnerve lesions, particularly those involving the opticchiasm.

1. Balen D, Çolak A, Özcan OE: CNS involvement of

Langerhans cell histiocytosis. Report of 23 surgicallytreated cases. Neurosurg Rev 19: 247-252, 1996

2. Bernard JD, Aguilar MJ: Localized hypothalamichistiocytosis X: report of a case. Arch Neurol 20: 368­372, 1969

3. Brisman JL, Feldstein NA, Tarbell NJ, Cohen D, CarganAL, Haddad J, Bruce JN: Eosinophilic granuloma of theeliyus: case report, follow up of two previously reportedcases and review of the literature on cranial base

eosinophilic granuloma. Neurosurg 41(1): 273-279, 19974. Cerda NM, Broseta i,Peydro OA, Barbera J, Barcia SJL,

Lombart BA: Primary eosinophilic granuloma of thefrontallobe. Virchows Arch A Pathol Anat Histol 388:

221-228, 1980

5. Czarnecki EI, Spickler EM: MR demonstration ofWegener granulomatosis of the infi.indibulum, a causeof diabetes insipidus. AJNR 16: 968-969, 1995

6. D' Avella D, Giusa M, Blandino A, Angileri FF, Rosa GL,Tomasello F: Microsurgical excision of a primary isolatedhypothalamic eosinophilic granuloma. J Neurosurg 87:768-772, 1997

7. Forrest E, Gallacher SI, Hadley D, Soukop M, Boyle IT:Central nervous system histiocytosis X-imaging andresponses to chemotherapy. Scott Med J 38: 148-9, 1993

8. Fukazava T, Yanagihara T, Hamada K, Hamada T:Multifocal eosinophilic granuloma presenting aspragressive brainstem and cerebellar dysfunction. JNeurol Neurosurg Psychiatry 57:980-982, 1994

9. Gagel O: Eine granulationsgeschwulst in gebeit deshypothalamus. Z Ges Neurol Psychia 172: 710-722, 1941

10. Graif M, Pennock JM: MR imaging of Histiocytosis X inthe Cental nervous system. AJNR 7: 21-23, 1986

11. Greenwood SM, Martin JS, Towfighi J: Unifocal

eosinophilic granuloma of the temporal lobe. SurgNeuroI17(6): 441-444,1982

12. Grois NG, Favara BE, Mostbeck GH, Prayer D: Centralnervous system disease in Langerhans cell histiocytosis.Hematology 10ncology Clinics North America 12(2):287-305, 1998

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A: Primary eosinophilic granuloma of the oculomotornerve. J Neurosurg 81: 784-787, 1994

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Acknowledgemeiit: We wish

Turgut Tali and M. Naci Edali forpreparing this paper.

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The comman solitan) lesian of the skull is known as

eosinophilic granuloma, and tlie multiple lesions of the

skull that are of ten associated diabetes insipidus are partof Hand-Schiiller-Cliristian Syndrome

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