Fallbericht - Case reports 178

Intraosseous neurilemmoma ofskull- Single case report 1. Sch iff er. R. Reif. E. l.ahat, A . Bar-Iojai, R. S tarinski Departments ofNe uros urgery , Pathology, Pediatri cs and Ha diology. Assaf'Harofeh Medical Center , Zer lfln. Isr ael

Summary Intraosseous neu rilemm oma is a rare benign tumo ur of the bone with characteristic ra diological and histological features . The most common places of this tumor are ma ndible. sac rum and vertebral bodies . A ease of a not previo usly reporte d loeation ofthis tumor in the vault ofthe skull is presented.

Das intraosseäre Neurilemmom ist ein seltener guta rtiger Tumor des Knochens mit charakteristische n röntgenologischen und histologischen Eigenscha ften. Die häufigsten Lokalisationen dieses Tumo rs sind Mandibel, Sakru m und Wirbe lkörper. Ein Patient wird vorgestellt mit eine r nicht beschriebenen Lokalisa tion dieses Tumors im Knochen des Hirnschädels.

Fig.l Skulf x-rays showingalytic lesion with scleroticbordersin thelett Ironto-orbital regten

Key-Words Neurilemmoma - Bone Tumour - Skull Tumou r

Intraosseous neurilemmoma (Schwannomal is a rare ben ign tumor ofthe bone . It may be associated with neurofibromatosis (t l. but most oflen it is not (2). The most common place is the mandible (l , 2. 3. 4). Othe r repo rted sites are sacrum , verte bral bodies, ulna , humerus, fernur , tibia, patella , sca pula, rib, maxilla, and sma ll bon es of the hands (2). To the best of our knowledge no case 01 intraosseous neur ilemmoma originating in the vault of the sku ll has been previously reported. Case Hislory A 3 yea r old boy was adm itted because of a swelling on the left fronto-orbi tal region which was noticed a day before, after a minor head injury to this area . On physical exa mination, a hard non-t ender mass of 3c m diam eter adhere nt to the skull. but not to the skin . was found in the left fronto -orbital region. Otherwi se the gener al and neurolog ical examinations wer e normal. Skull Xrays showed a lytic Iesion with selerotic bord ers in the left fronto orbit al region (Fig. 1). Cl' Scan ofthe skull show ed a soft tissue mass with hone ero sion in the same region (Fig. 2). An open biopsy show ed tlssue composed of fusiform cells in par allel a rra ngement with intertwini ng bun dles. The nuclei were slender. regular and Neurochirurgia 34 (199 1) 178-179 © Georg Thieme Verlag Stuttgart · New York

Fig.2 Cl Scan of the skull showing a sott tissue mass with bone erosion in the sameregten

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Intraosseäres Neurilemmom des Airuschädels - Einzelfallbe ric ht

lntraosseoue neurilemmoma ofs kult -: Single case report

Neurochirurgia 34 (199 1)

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orthochr omatic witho ut atypia or mitosis and the cytopla sm was also fusiform. The d iagnosis was made of a typica l neu rilemmoma com posed almost entirely of ..Antoni A" tlssue. The stroma of the tumor showed slight to mod erate collagen izatio n a nd the more perip heral are as revealed a myxomatou s tumor matrix. The tumo r wa s very homogenous. without intlammatory Infiltration. without mitotic flgures. at ypical nuclei or multi nuclea r cells. Some degenerated bone part icles were found within the tumor tissue (Fig. 3. 4). The child wa s aga in operated upon and the tum or was completely excise d. The postoperative course wa s uneventful. After one yea r of follow up 00 evidence ofrecur rence was noted .

Fig.3 Tlssue material showing coherentbenign fusitorm celfs, HE x 250

Intraosseous neuril emm oma (Schw a nn orna) is a very unu su al tumo ur. Aeeording to Faweett (1 ), wh o made a review of the liter ature and re porte d a survey of 31 additiona l eases, they aeeounted for less th an 0 .2 % of primary bone tumo rs. Fa weett (1 ) also remarks that the mand ible is the most common place of the intr aoss eou s neurilemmom a app ar ently beca use it contai ns a long segment of the ma ndibula r nerve. Gordon (3) remarks tha t the nerves in the long bones are lan ger than in the ma ndible, but the frequency of intraosseou s neurilem moma in these bones is much lower than in the mandible. These tumors a re cha racterized by loeal swelling a nd there may be of tenderness at the site (4); in our eas e the swelling was not tender . The radi ologie fea tures in our case were as in the other reported eases (1, 2, 3), eha rae terized by a Iytie defeet in th e bon e wit h sclerotic margins , cort ical erosio n without bon e forrnation, and absence of calcification or oss ification. The histologic features were also simila r to the report ed ea ses (1, 2, 3, 4) an d revealed a typieal neuril emmoma of . Antoni A" type. Complete loeal rese etion is the treatme nt of ehoiee in these tumors (1, 3, 4), without reeurren ee (3). No oth er treatment is advise d. References I

Fig.4 Low powerview of the tumor tissue showing thecharacteristtc pietute of a neurilemmoma, HEx 100

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Fiuoceu. K. 1.. D. C. Dahlin: Neurilernmoma of bone. Am. J. Clin. Pathol. 47 119671759- 766 De la Monte, S. M.. K. H. Dorfman. R. Chandra. M. Malawer: Intraosseous Schwannoma. Histologie featur es, ultrastructure and review of the literature. Human Parhol. 15 (1984) 551-558 Gordon. E. J.: Solitary intraosseous neurilemmoma of the tibia . Review of intra osseous neurilemmoma and neurofibroma. Clin. Orthop. 117 (197 6) 271- 282 Sa mter. T. G.. F. Vellios. W. G. Shaf er: Neurilemmoma of bone. Report of 3 cases and review ofth e litera ture. Radiology 75 (t 960) 215-221

Juana Schiffer MD Department ofNeurosurgery Assaf lla rofeh Medical Center Zerifin 70300/lsrael

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Discussion

Intraosseous neurilemmoma of skull--single case report.

Intraosseous neurilemmoma is a rare benign tumour of the bone with characteristic radiological and histological features. The most common places of th...
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