ACTA NEUROCHIRURGICA 9 by Sptinger-Verlag 1978

Acta Neurochirurgica 40, 315--321 (1978)

Neuropathologische Abteilung*, Institut fiir Pathologic der Universit/it Basel und Neurochirurgische Klinik**, Department ffir Chirurgie der Universitgt, Kantonsspital, Basel, Switzerland

Schwannoma of the Olfactory Groove Case R e p o r t a n d R e v i e w of P r e v i o u s C a s e s By J. Ulrich*, A. L~vy**, and Chr. Pfister* With 4 Figures

Summary A personal case, the third in the literature, of an olfactory groove schwannoma in a young man aged 19 is reported, and the two cases previously described are reviewed. Occurrence in young males and long histories are characteristic.

Key words: Brain turnout, malformation. According to Russell and Rubinstein (1971), schwannomas account for about 8~o of all primary intracranial turnouts. The overwhelming majority of these tumours originate from the eighth, and in a few cases from the fifth cranial nerve. They can occur in relation to yon Recklinghausen's disease, in which the 10th and 1lth, and occasionally other motor nerves also can be involved. The origin of the tumour is usually after the passage of the cranial nerve through the leptomeninges, before it penetrates the dura mater. To our knowledge only two solitary schwannomas of the olfactory groove have been described (Sturm et al. 1968, Sehrbundt et al. 1973). Three further examples in the same place were observed in yon Recklinghausen's disease (Spiller et al. 1903, Christin et al. 1920, Molter 1920). These cases were all described before neurosurgery became generally established. The reports are difficult to obtain, but they were reviewed 1973 by Sehrbundt et al. We here describe one further example of a solitary schwannoma of the olfactory groove.

0001-6268/78/0040/0315/$01.40

316

J. Ulrich et al. :

Case Report A male adolescent, aged 19, had suffered from epileptic seizures since the age of 14. A b o u t eight weeks before admission he had suddenly realized t h a t his right eye was practically blind. H e saw an ophthalmologist who referred him for neurosurgical investigations. Neul'ologieal e x a m i n a t i o n revealed a total right-sided and a partial left-sided anosmia, diminished corneal reflexes, and hypaesthesia of all areas supplied b y the right trigeminal nerve. Ophthalmological e x a m i n a t i o n showed a delayed pupillary reaction on the right side, and pallor of the optic discs, especially on the right. The right eye was blind except for a narrow region in the u p p e r right q u a d r a n t . I n the left eye there was a defect in the upper t e m p o r a l q u a d r a n t . No p i g m e n t e d spots on the skin, nor o t h e r signs of yon Reeklinghausen's neurofibromatosis were observed. Cerebrospinal fluid e x a m i n a t i o n revealed a n o r m a l cell count, an increased protein e o n t e n t (152 mg~ and a pathological Mastix-eurve. E E G was pathological, showing frontal r h y t h m s , b u t no epileptic aetivity, generalized or focal. Scintigraphy with Tc 99-Pertechnate: a hugc frontal turnout was demonstrated. X - r a y e x a m i n a t i o n , including angiograms of the right carotid a r t e r y eenfirmed the presence of a very large t u m o u r s i t u a t e d m a i n l y at the base of the :right anterior fossa, pushing t h e anterior cerebral a r t e r y backwards and over the midline, and stretching the middle cerebral a r t e r y (Fig. 1). A m e n i n g i o m a of the right olfactory groove was therefore suspected before operation. Operation: The t u m o u r t u r n e d out to be v e r y large, a b o u t the size of an orange. I t was well delimited, and was situated m a i n l y in t h e anterior cranial fossa. H e r e it was a t t a c h e d to the base of t h e skull. I t p r o t r u d e d into the right and, to a lesser extent, also into the left frontal lobe. I t inv o l v e d the anterior p a r t of the falx which had to be sacrificed during operation. I n its posterior p a r t the turnout reached t h e trigeminal n e r v e to which, however, it was n o t attached. The right olfactory bulb was n o t observed (luring operation and therefore it m i g h t h a v e been destroyed by the t u m o u r . The p o s t o p e r a t i v e eourse was complicated by a subgaleal infection which oeurred four weeks after the operation. Otherwise the eourse was uneventful. Ophthalmologieal control showed a slight i m p r o v e m e n t of the visual field. The hypaesthesia of the right half of the face disappeared. The patient, three years after the operation, still has occasional epileptie fits, but feels otherwise well. Hi.s.tology (13069/73): A collection of t u m o u r f r a g m e n t s weighing 200 g was received for exmnination. T h e y were all of a firm consistency. Some of the pieces showed a surface along a natural plane of cleavage, b u t no capsule which could be distinguished by the naked eye. The cut surfaces showed m a n y visible blood vessels. Fig. 1. R i g h t carotid angiography, a) Lateral view : E l e v a t i o n of the perieallosal and frontopolar arteries by a huge frontobasM t u m o u r . The origin of the t o r t u o u s vessels c a n n o t be identified, b) F r o n t a l v i e w : R o u n d e d shift of t h e anterior cerebral a r t e r y to the left. B e t w e e n t h e middle and the anterior cerebral artery complexes demarcation of the t u m o m ' b y his own vaseularisation

Sehwannoma of the Olfae6ory Groove

Fig. i a

Fig. 1 b

317

318

3. Ulrich et al. : Schwannoma of t,ho Olfactory Groovo

Microscopic examination demonstrated the tumour to consist of colnparatively few spindle-shaped cells, whose nuclei in some regions were arranged in rows with many thin parallel reticulum fibers running from one row to the other (Fig. 2). Other regions eonsisted of less clearly orientated reticulin and eollagen fibres, but ~lso eontained very long spindle-shaped cells which were distributed haphazardly between the fibres (Fig. 3). There were very large venous channels (Fig. 4), delimited from the rest of the tumour by their endothelium only. No structures reminiscent of the olfaetory bulb or other central nervous structures were seen.

Fig, "2. One of the regions exhibiting very pronounced palissading of the nuclei (Van Gieson stain, × 70)

Discussion As far as we know, this is the sixth ease of a s c h w a n n o m a of the olfactory groove to be described. Three of the previous cases suffered from y o n Recklinghausen.'s neurofibromatosis. Although the clinical features of the previous two eases of solitary olfactory n e u r i n o m a were n o t specific, t h e y were still quite characteristic in some respects. Like our case t h e y were y o u n g males, as opposed to p a t i e n t s with m e n i n giomas, who are u s u a l l y older. P a t i e n t s with s e h w a n n o m a s are more often female t h a n male. The h i s t o r y can be of variable length. I t m a y he a few weeks ( S e h r b u n d t et al.) or it m a y be years (Sturm et al.). I n the case presented here, the epileptic seizures were p r o b a b l y the first s y m p t o m . Therefore, the history was of five years duration.

Fig. 3. Higher magnification of the same region as pictured in Fig. 2 (Van Gieson stain, X 175)

Fig. 4. Large venous vessels in the tumour, often delimited by endothelium only. No palissading of nuclei in this region (Van Gieson stain, × 70) 21 Acta Neurochirurgica, Vol. 40, Fase. 3--4

320

J. Ulrich et at. :

The i n v o l v e d structures are u s u a l l y t h e olfactory lobes, t h e optic nerves, a n d t h e f r o n t a l lobes. F u r t h e r m o r e , osseous s t r u c t u r e s such as t h e n a s a l sinuses can be i n v a d e d . I n t h e ease of S t u r m t h e t u m o u r p r o d u c e d bulging of t h e glabella to t h e outside. This was responsible for a severe a l t e r a t i o n of t h e p a t i e n t ' s facial expression. Histologically, t h e v e r y strict r e g i m e n t a t i o n of t h e cell nuclei can be v e r y striking, as in air ease a n d in t h a t of S t u r m et al. I n spite of t h e size of t h e t u m o u r s , prognosis seems to be c o m p a r a t i v e l y good. F r o m t h e reports published, at least years of s u r v i v a l in good clinical s t a t e are possible. This, of course, is what. one would e x p e c t from tlhe essentially benign h i s t o l o g y of the t u m o u r . The m o s t puzzling question is t h a t of origin.Very rarely, s e h w a n n o m a s of t h e central nervous s y s t e m h a v e been r e p o r t e d (New 1972, Gibson et al. 1966). Therefore, an origin in t h e o l f a c t o r y bulb itself, which is a p a r t of t h e central n e r v o u s system, is conceivable. Such an origin is also suggested b y t h e d e s c r i p t i o n of Christin et aI. (1920), who described b i l a t e r a l olfactory s e h w a n n o m a s which seemed to h a v e arisen in t h e o l f a c t o r y bulbs themselves. The fact t h a t alI p a t i e n t s were y o u n g males m a k e s one consider an origin from e m b r y o n i c structures. Of these t h e e m b r y o n i c t e r m i n a l nerve is one of t h e m o s t obvious c a n d i d a t e s (Sturm et al. 1968). F u r t h e r more, an origin in the fila olfactoria, t h e anterior e t h m o i d a l nerves, or t h e m e n i n g e a l branches of t h e t r i g e m i n a l nerve is possible. A p a r t from t h e y o u t h of t h e p a t i e n t s , t h e p u b l i s h e d eases do n o t p r o v i d e a n y evidence for these theories. Acknowledgements We are grateful to Professor L. J. Rubinstein for reviewing the histological preparations of this ease, to Miss M. Nebiker and Mr. H. R. Zysset for photographicM assistance, and to Mrs. T. Piccand for typing the manuscript.

References Christin, E., Naville, F., A p r o p o s de neurofibromatoses centrales. Leurs formes familiales et hdr~ditaires. Les neurofibromes des nerfs optiques. C a s h 6volution atypique. Diversitds des structures histologiques (6rude elinique et anatomique). Ann. Med. 8 (1920), 30--50. Gibson, A. A.M., Hendriek, E. B., Conen, P . E . , Intracerebral Sehwannoma. Report of a case. J. Neurosurg. 24 (1966), 552--557. Molter, K., (~ber gleichzeitige cerebrMe, medullgre und periphere Neurofibromatosis (Inauguraldissertation, Universitgt zu Jena). J e n a : W e n d t und Klauwell. 1920. New, P. F. J., Intracerebral Sehwannoma. Case report. J. Neurosurg. 36 (1972), 795--797. Russell, D . S . , Rubinstein, L . J . , Pathology of" tumors of the ~erw)us system, 3rd ed., p. 286. London: E. Arnold. 1971. Sehrbundt Viale, E., Pau, A., Turtas, S., Olfactory groove neurinomas. J. Neurosurg. Sci. 17 (1973), 193--196.

S c h w a n n o m a of the Olfactory Groove

321

Spiller, W. G., t t e n d r i c k s o n , V~T. F., A report of two cases of m u l t i p l e sarcomatosis of the central n e r v o u s s y s t e m a n d of one case of i n t r a m e d u l lary p r i m a r y s a r c o m a of t h e spinal cord. A m e r i c a n J. Med. Sci. 126 (1903), 10--33. Sturm, K. W., Bohnis, G., K o s m a o g l u , V., ~ b e r ein N e u r i n o m der L a m i n a cribrosa. Zbl. Neurochir. 29 (1968), 217--222.

Author's address: Dr. J. Ulrich, lqeuropathologische ffir Pathologic, Universit/~t Basel, Basel, Switzerland.

21"

Abteilung, Institut

Schwannoma of the olfactory groove. Case report and review of previous cases.

ACTA NEUROCHIRURGICA 9 by Sptinger-Verlag 1978 Acta Neurochirurgica 40, 315--321 (1978) Neuropathologische Abteilung*, Institut fiir Pathologic der...
1MB Sizes 0 Downloads 0 Views