Journal of Neuro-Ophthalmology, March 1991, Volume 11, Issue 1

Unusual location of histiocytosis X. Presenting as a petrous apex syndrome.

The authors describe a case of histiocytosis X of the petrous bone presenting as petrous apex syndrome. The clinical, radiological, and histopathological features are discussed, and the pertinent literature is reviewed. To our knowledge, histiocytosis X of the petrous apex has previously been reported to cause this syndrome in only two cases.

/ ournal of Clinical Neuro- ophthalmology 11( 1): 31- 34, 1991. Unusual Location of Histiocytosis X Presenting as a Petrous Apex Syndrome Oguz <:= ataltepe, M, D., Ahmet <:= olak, M. D., Siileyman Saglam, M. D., and Behsan Onol, M. D. © 1991 Raven Press, Ltd., New York The authors describe a case of histiocytosis X of the pe­trous bone presenting as petrous apex syndrome. The clinical, radiological, and histopathological features are discussed, and the pertinent literature is reviewed. To our knowledge, histiocytosis X of the petrous apex has previously been reported to cause this syndrome in only two cases. Key Words: Histiocytosis X- Petrous apex syndrome- ­Petrous bone. From the Departments of Neurosurgery, ( 0.<;:., A.<;:., 5.5.) and Pathology ( B. O.), Hacettepe University Medical School, Ankara, Turkey. Address correspondence and reprint requests to Dr. O. <;: at­altepe at Kuzgun 50kak 30/ 1, 06540 A. Ayrana, Ankara, Tur­key. /-----------...... 31 Histiocytosis X is an uncommon multisystem disorder of unknown etiology, which is character­ized by accumulation of histiocytes in various tis­sues and which has a variable clinical course. His­tiocytosis X is a term that was first coined by Lich­tenstein in 1953 ( 1). It is a collective name for three uncommon conditions: Eosinophilic granuloma, Hand- Schuller- Christian disease, and Letterer­Siwe disease ( 1,2). Involvement of the central nervous system ( CNS) is rare. Histiocytosis X involves the hypo­thalamic- pituitary axis and causes diabetes insipi­dus, visual loss, and other neurological distur­bances. It has also been reported to involve the skull ( 1- 4). It may infrequently originate in the temporal bone, but it is extremely rare in the pe­trous apex. On reviewing the medical literature, we were able to find only two cases of Histiocyto­sis X in petrous bone ( 5,6). In this report, we present an unusual case of histiocytosis X which was located in the petrous apex of the temporal bone, presenting as a petrous apex syndrome ( PAS). CASE REPORT A 14- year- old girl was admitted to our clinic with a history of progressive horizontal diplopia begin­ning 15 days prior to her admission. She had also been suffering from headache and right facial pain for 20 days. On general physical examination, findings were within normal limits, whereas neu­rological examination revealed a complete right lat­eral rectus palsy and paresthesia in all three divi­sions of the fifth cranial nerve on the same side. Right masseter and pterygoid muscle weakness were noted. A mild right peripheral facial paresis was seen. The rest of the neurological examina­tion, including the eighth cranial nerve and oto­logical examination, were within normal limits. o. CATAU'EPE E'l' AL. PlaIn Jl- ray mm. of.. 1Iadl.... ld alytk: Ie­lion m'hf. a rtpt petrou .,. •. 1 the temporal ~ ft!'. Ormpub! d tonqpaphy (' l' ... demon­Itrmd a . l" p1y and mtaDIbIJ In. uRlnated and expandlnl hypodenle liliian ".. In the right pelIouI apex. Carana1 cr _ dIIIaonstratI! d Ibd the IIIIaa.. 0IaId III ,........ 2an In .........., the coed, 11Ft petmua ......................,." 1IIIanbuJpd In'" dw eedddIe r.- CJIIB. 1). A8Ir I IKtmof the inIelI\ mol _ n I IIhowecI _ _ •__ . mte8dlun ............., ". vuc: u- ....,. ". IeveJ8 of ,..,.., Iwa_-. ....,. ad bone mar-lOW upleallcn. ~ IIGI'iiId. At opemtian" a riFt teenporal c: raniolDall wu cmIied GIlt in the left IaIeml pmltIan. When the A C 1EeiipGdl1abe....... tIIIaaP tile b ol••• app;~ an IIJCImdmwJ __ wIIk: h baJr' IedD the IfIiddJe&. a.... In tile pebUii8., ex. ' 1' IIe tumor wu 2 an III diameter ad well c: In: um­ICdbed by dum. Needle upJraIIcn of the lumcnl ...... JeVBIed a bIacd upDate. w. tIMJaBbt tbIt the tumanl ...... hIId Ill the .... ... IInUI. The bleP! dIna CXJIdr01Ied and .... wu bIdI8I. 1' umaI' iemDted III pBe-ii8l fuhIcn. ~ examiMtIan of the .,... ' E ..... hIlIIiDcylDilll X. wIIh iiid& i hi8tiocJ1B and flOIInapIdII with _ DIIIIII . eelpmcella ( Pia. 2). The.. tlen. r. paltopemtlve aJIIiIIewuurcoa'" ful, PalIDpeIatIvwly ... wu bated wJIb. ... thInpy ( l, a rad). Her fIJIIow.. up egmhMdlaa, after tine manthI, I'ft8Jed Nlrfill! 1 eye IPIGft- 8 FIG. 1. Preoperative axial ( A) an. d coronal ( B) CT scan cl. emonstrates. a smoothly marginated, hypodense le­510n located In the right petrous apex Th )• uggest'Ive 11UI'd Ieve!. Bone windows ( C.) deemroenwtasta ~ xpanded bone. Th~ lesion bulged into the ': i~~ I: fossa, and ~ he overlying bone in the right petro s paper- thin. us apex /-------, HISTIOCYTOSIS X AND PETROUS APEX SYNDROME 33 FIG. 2. Histological appearance of the lesion. Numerous multinucleated giant cells ( arrow) are noted, in addi­tion to eosinophils and histiocytes ( HE x 460). ment; the other neurological examination findings were within normal limits, except for minimal masseter weakness and hypoesthesia in the der­matomes of the fifth cranial nerve. Postoperative CT scan taken 3 months after the operation showed that the lesion had been sclerosed ( Fig. 3). DISCUSSION Histiocytosis X is a common disorder of the re­ticuloendothelial system and has been described as a " non- neoplastic disorder of unknown etiology, pathogenically akin to an inflammatory reaction with multiple clinical manifestations mirroring the widespread distribution of the histiocytic system in the body" ( 7). It is most commonly seen in chil­dren and young adults ( 4,7). The younger patients typically present with multiple and recurrent areas of involvement ( Hand- Schuller- Christian and Let­terer- Siwe disease), while solitary nonrecurring le- FIG. 3. Postoperative CT scan taken 3 months after the operation shows the lesion. sions ( eosinophilic granuloma) are more character­istic of the disease seen in older children and young adults. Eosinophilic granuloma is the most benign of the three clinical forms, with lesions lo­calized to bone ( 2,4,7). Histiocytosis X may affect reticuloendothelial cells anywhere in the body. Skull bones, hypothal­amus, optic chiasm, orbits, and cerebral paren­chyma are the best- known involved sites in the central nervous system ( 1,2,4). In histiocytosis X any bone in the body may be affected, but most lesions are found in the skull, ribs, and long bones. In a review of several large series, 20- 40 percent of the cases show involvement of the skull, and 5- 10 percent have temporal bone lesions. Unifocal le­sions are twice as common as multifocal lesions ( 1,4,7). When eosinophilic granuloma affects the temporal bone, the disease may erode mastoid cor­tex, semicircular canals, and cochlea, destroy the tegment, and extend into the cranial vault ( 2,4). Histiocytosis X in the temporal bone may be clin­ically silent at first. As the lesion becomes expan­sile, erosion of any portion of the temporal bone may occur ( 4,8). In histiocytosis X, cranial nerve palsies are very rare, despite extensive bone destruction. When the petrous apex is involved, as in our case, it may produce fifth and sixth cranial nerve palsies. In the patients with severe mastoiditis, the inflammatory process may extend to the petrous apex of tempo­ral bone, producing localized inflammation of the meninges and a classical Gradenigo's syndrome ( 9- 12). Other lesions may produce PAS, including tumors such as meningioma, plasmocytoma, tri­geminal neuroma, as well as aneurysm of the in- J elin Neuro- ophthalmol, Vol. 11, No. L 1991 34 o ( ATALTEPE ET AL. trapetrosal segment of the internal carotid artery ( 11- 13). In our case, the histiocytosis X which had been localized in the petrous apex had an unusual presentation. Tumors of the petrous apex occur infrequently and are usually not diagnosed until they reach a considerable size, causing multiple cranial nerve deficits or intracranial complications ( 3). Headache, one of the most prominent symp­toms in tumors of the petrous apex, is probably an indication of dural stretching by an expanding tu­mor at the petrous apex. The major complaints of our patient were headache and diplopia. Other common symptoms of histiocytosis X are involve­ment of the fifth, sixth and seventh nerves. In­volvement of the third division of the fifth cranial nerve causes paresthesia over the distribution of this nerve ( 3). These cranial nerve involvements were present in our case. Histiocytosis Xof the temporal bone is radiolog­ically similar to other skull lesions produced by histiocytosis X; it consists of " punched out" le­sions rather than expansile lesions and shows a well- circumscribed osteolytic defect without any periosteal or osseous reaction. The margins may be irregular rather than smooth ( 10,14- 17). However, the bone of the petrous apex contains marrow spaces and would be more likely to allow for ex­pansion of a tumor than the other solid bony com­ponents of the skull ( 3). In our case, CT scan re­vealed a sharply and smoothly marginated expan­sile lesion in the petrous apex. Therefore, in our case CT appearance may be confused with a number of pathological conditions which may present as a smooth- edged expansile lesion in the petrous bone, bone mucocele, aneurysm of the in­trapetrous internal carotid artery, giant apical air cells, a cholesterol or epidermoid cyst of the pe­trous apex, etc. In our case, our preoperative di­agnosis was epidermoid cyst of the petrous apex, which can be mostly encountered in this region. The nonenhancement and hypodensity of the le­sion on CT scan supported this preoperative diag­nosis. TClin Neurcrophthalmol, Vol. 11, No. 1, 1991 Finally, we wish to point out that this case report should alert the clinician to histiocytosis X as a possible cause of a petrous apex syndrome. REFERENCES 1. Lichtenstein L Histiocytosis X: integration of eosinophilic granuloma of bone. " Lellerer- Siwe disease" and " SchiiUer­Christian disease" as related manifestations of a single no­sologic entity. Arch Pathol 1953; 56: 84- 102. 2. Chu T. D'Angio GL. Favara B, et al. Histiocytosis syndrome in children. LAncet 1987; 24: 208- 9. 3. Gacek RR Diagnosis and management of primary tumors of the pelrous apex. Ann Otol RhinoILAryngoI1975; 84( suppl 18): 1- 20. 4. Sweet RM. Komblut AH. Hyams WS. Eosinophilic granu­loma in the temporal bone. LAryngoscope 1979; 89: 1545-- 52. 5. Boniver R. Lepage G. Thibaut A. Le granulome eosinophile du rocher: a propos d'un cas particulier. Acta Otorhinolaryn­gol Belg 1972; 26: 369- 74. 6. Neutsch WD. Usbeck W. Uber die ungewohnliche LokaJi­sation eines eosinophilen Granuloms in der Felsen­beinpyramide. leutralbl Chir 1965; 90: 980- 3. 7. Enriquez P. Dahlin DL. Hayles AB, et al. Histiocytosis X: a clinical study. Proc Staff Meet Mayo Clin 1967; 42: 88- 99. 8. Cunningham MJ, Curtin H. Butkiewicz L Histiocytosis X of the temporal bone: CT findings. J Comp Assist Tomog 1988; 12: 70- 4. 9. Gradenigo G. A special syndrome of endocranial otitic com­plications ( paralysis of the motor oculi extemus of otitic origin). Ann Otolaryngol 1904; 13: 637- 9. 10. Livingstone PA: Differential diagnosis of radiolucent le­sions of the temporal bone. Radiol Clin N Am 1974; 12: 571­583. 11. Neil MR Gradenigo's syndrome. In Walsh FB. Hoyt WF, eds. Clin neuro- ophthalmology, vol 2. 4th ed. Baltimore! London: Williams & Wilkins, 1985: 702- 3. 12. Yamashito J, Asato R. Handa H. et aI. Abducens palsy as an initial symptom of trigeminal schwannoma. J Neurol Neu­rosurg Psychiatry 1977; 40: 1190-- 7. 13. Sarwar M. Abducence nerve paralysis due to giant aneu­rysm in the medial carotid canal. JNeurosurg 1977; 46: 121- 3. 14. Baghdassarian SA, Shammas HF. Eosinophilic granuloma of orbit. Ann OphthalmoI1977; 9: 1247- S1. 15. Castel Jc. Diard F. Chateil JF, et aI. Interet de la tomoden­sitometrie dans les attentes histiocytaires de la base du crane chez l'enfanl. Ann Radiol 1988; 31: 151~. 16. Lo WWM. Bohman LGS. Brackman DE. et aI. Cholesterol granuloma of the pelrous apex: CT diagnosis. RJuliology 1984; 153: 705-- 711. 17. King IT, Benjamin Jc. Marrison AW. Epidermoid and cho­lesterol cysts in the apex of the petTous bone. Br JNeurosurg 1989; 3; 451--- 62. /--------"""""\.