Aeta Neurochirurgiea 28, 315--322 (1973) 9 by Springer-Verlag 1973

Departments of Neurological Surgery and of Neuropathology, Royal Infirmary, Sheffield, S 6 3 DA, England

Posterior Fossa Ceruminoma By

R. (L Rossato and W. R. Timperley With 8 Figures

Summary A case is reported of a cermninoma invading tlu'ough the pe~rous temporal bone and meninges and into the eerebellmn and brain stem. The tumour presented initially as a progressive facial palsy and later involved other cranial nerves. The poor prognosis and the importance of early recognition and radical treatment of this tumour is emphasized. Key words: Ceruminoma--Petrous temporal bone--Cerebello Pontine angle--Facial Nerve. Turnouts arising from the ceruminous glands of the external ear are extremely rare. These glands are typical apoerine sweat glands and t h e y secrete a w a t e r y fluid. The tumours are made up of tubules or alveoli which m a y become distended to produce the appearance of a cystadenoma. Histologically, the epithelium contains two cell compouents, an inner layer of cuboidal or cylindrical cells and an outer layer of myoepithelial cells. The t m n o u r m a y have a papillary or a cribriform pattern. Clinically the eeruminoma usually presents to the ear, nose and t h r o a t surgeon as a mass in the external auditory meatus with deafness, pain, discharge or tinnitus. I t is extremely rare for it to present as a posterior fossa mass. The first ease to do this was reported b y Berlin (1949) who described a ease with unilateral deafness and facial paralysis combined with radiological evidence of erosion of the petrous temporM bone. At operation a mass of t u m o u r was seen extradurally in the left posterior cranial fossa displaeing the left eerebellar hemisphere downwards and to the right. Histologically this turnout had the typical appearance of a ceruminoma. More recently, Kleinsasser and Seharfetter Acta Neurochirurgica, u

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1R. G. g o s s a t o and W. R. T i m p e r l e y : Posterior Fosse C e r u m i n o m a

(1957) d e s c r i b e d a ease w i t h i n v a s i o n of t h e d u r a a n d c e r e b e l l u m . T h e ease we r e p o r t in t h i s p a p e r is o n l y t h e s e c o n d t o be d e s c r i b e d i a t h e world literature with a transdural iavasion el the pens and cerebellum.

Case Report. J . H. male, aged 62 years, was first seen by an E a r , Nose and T h r o a t c o n s u l t a n t w i t h an isolated left facial palsy of progressive onset some five years prior to admission to t h e R o y a l I n f i r m a r y , Sheffield. Because he failed to a t t e n d for follow up he h a d u n d e r g o n e plastic surgery for his facial palsy at some t i m e during t h a t five y e a r interval. His complaints on referral to the D e p a r t m e n t of N e u r o s u r g e r y were those of difficulty w i t h balance and deafness in the left ear, t h e former being first noticed b y t h e p a t i e n t some m o n t h s prior to admission. On questioning, it a p p e a r e d t h a t he h a d i m p a i r e d taste, also paraesthesiae a n d hypoaesthesia of t h e left side of t h e face, which h a d c o m m e n c e d in t h e forehead. Swallowing was also i m p a i r e d and he h a d n o t e d double vision on left lateral gaze. On e x a m i n a t i o n he was found to h a v e depressed f u n c t i o n of t h e sensory c o m p o n e n t s of t h e trigeminal n e r v e w i t h m o t o r sparing and a complete left facial palsy. There was evidence of palatal dysfunction and there was ipsilateral glossal h e m i a t r o p h y . There was a cerebellar disturbance affecting the f u n c t i o n of the left a r m and leg. No sensory deficit was found in t h e limbs or trunk. T h e t y m p a n i c m e m b r a n e s were n o r m a l and no b r u i t was audible in t h e head. B o n e conduction exceeded air conduction and t h e W e b e r test was lateralised to t h e left ear. R a d i o g r a p h s showed gross erosion of t h e internal a u d i t o r y meatus. T h e apex of t h e petrous t e m p o r a l bone was spared (Figs. 1-3). A t operation, a large k n o b b l y surfaced t u m o u r was found filling the left cerebellopontine angle. B o t h t h e trigeminal and a b d u c e n t nerves were compressed and d i s t o r t e d and t h e internal a u d i t o r y pair were i n c o r p o r a t e d in it. Its lower surface e x t e n d e d to the r e g a l group and it was maeroseopieally " a d h e r e n t " to t h e eerebellar hemisphere. A l t h o u g h vascular, there was no question of it being a glomus t u m o u r and despite t h e presence of a little oily fluid, it did n o t appear to be an epidermoid. I-Iis p o s t - o p e r a t i v e course was poor w i t h o u t r e c o v e r y of the affected cranial nerves. Despite t r a e h e o s t o m y and nasogastrie feeding, he died of cardiorespiratory complications a b o u t three weeks post-operatively. A t p o s t - m o r t e m , r e m a n t s of t u m o u r were still present in t h e region of t h e left eerebello-pontine angle a n d the t u m o u r was seen to be arising from the region of t h e left petrous t e m p o r a l bone. This area was r e m o v e d i n t a c t and decalcified. Serial sections were t h e n cut and t h e t u m o u r t r a c e d to the region of t h e external a u d i t o r y canal (Fig. 4). ttistologieally t h e t u m o u r was basically composed of duets and cords of cells, and in m a n y areas was t w o - l a y e r e d w i t h an outer layer of m y o e p i t h e l i a l cells (Fig. 5). M u c h of the turnout, however, h a d a cribriform structure w i t h m u l t i p l e small cysts containing clear fluid and a m o r p h o u s debris, some of which was P A S positive. I n some areas the cells were m o d e r a t e l y pleomorphie and m i t o t i c figures were fairly easily found. This picture was i n t e r p r e t e d as evidence of a low-grade m a l i g n a n t change. Most of the glands showed evidence of apocrine secretory a c t i v i t y in the superficial epithelial cells (Fig. 6). A further interesting feature was that the tumour invaded through the meninges and was infiltrating the cerebellum, loons, and upper medulla (Figs. 7 and 8).

Fig. 1. Per-orbital X-ray demonstrating gross erosion of the petrous temporal bone (arrow)

Fig. 2. Towne's view of skull demonstrating the erosion with ~pieal petrous sparing (arrow) 21"

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R . G . l%ossato and W. 1%. Timperley:

Fig. 3. Lateral skull X-ray showing the extent of the erosion of the internal auditory meatus {arrow)

Fig. 4. Section of petrous temporal bone to show dilated ceruminous glands adjacent to external auditory canal (to the left). Decalcified section • 24

Posterior Fossa Ceruminoma

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Fig. 5. Section of tumour within petrous temporal bone to show duets and eribriform glandular structures. Haem~toxylin and eosin stain • 250

Fig. 6. Section of tumour epithelium to show apoerine secretory activity • 600

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1~. G. l~ossato and W. 1%. T i m p e r l e y :

Fig. 7. Section of meninges a n d eerebellar c o r t e x at t h e cerebello-pontino anglo to show invasion b y turnout. The t u m o u r is m o r e pleomorphie in this area • 60

Fig. 8. Section of lateral pons to show area of i n v a s i o n b y t u m o u r

• 60

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Discussion This is only the second time t h a t a ceruminoma has been described penetrating the dura mater and invading the cerebellum, pons and upper medulla. Histologically these tnmours closely resemble sweat gland tumours found in other parts of the body and Johnstone et al. (1957) regard them as mental hidradenomata. They show, to variable degrees, the two-layered epithelial structure resembling t h a t of sweat glands. They also show evidence of the apocrine type of secretory activity. Several cases have shown a cylindromatous pattern, also sometimes seen in sweat gland tumours elsewhere. The tumour described here showed all these features in different areas. I t also showed evidence of malignant transformation. Previously described cases have shown a high incidienee of malignancy and even the eases showing uniformly well-differentiated features show a strong tendency to recur. This tendency is notably different to that of sweat gland tumours of the skin surface where only about 5 ~ behave in a malignant fashion (Johnstone et M. 1957). Occasionally the ceruminoma presents to the neurosurgeon as a posterior fossa mass. Berlin (1949) described such a case in a 26 year old man and he suggested that it arose from aberrant tissue usually found in the external or middle ear. He also postnlated that it could arise b y transpetrosM spread but failed to demonstrate this. This was dearly shown in our ease by serially sectioning the petrous temporal bone. The turnout was seen to extend to the region of the external auditory canal but no surface protrusion was seen which might have been visible by examination of the external ear. Jefferson and Whitehead (1931) described a papilliferous cystic turnout producing a facial nerve palsy within the petrous temporal bone. I t seems possible that this tnmour was, in fact, derived from eeruminons glands. Clinically, the ceruminoma is capable of closely mimicking other cerebello-polltine angle tnmours and it should Mways be considered with other more uncommon tumours presenting in this area such as epidermoid cysts (Jefferson and SmMley 1938, Pennybacker 1944, Tytns and Pennybacker, 1956), ependymomas (Cushing 1917, t t a r d m a n and Jefferson 1938), chordomas (Stroobandt and Corn~llis 1971) and glomus turnouts (Shapiro and Neues 1964). I t is also important to remember that this tumour has a bad prognosis and nearly all the tumours in the literature which have been followed up have recurred. Treatment should be radieM when first detected. Presentation with facial nerve involvement and enlargement without erosion of the internal auditory meatus merits vertebral angiography, especially if the facial involvement takes the form of hemifaciM spasm (Echmann et al. 1971).

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Acknowledgement W e would like to t h a n k Mr. J. H a r d m a n of the D e p a r t m e n t of Neurosurgery, under whose care the patient was a d m i t t e d for permission to publish this case; and Mr. K. I-torten, Chief Technician in the D e p a r t m e n t of Ncuropathology for his technical assistance.

Referenees Berlin, L., Intracranial ceruminous adenoma. J. Neurosurg. 6 (1949), 415--418. Cushing, tI., Experiences with acoustic tumours. Bull. Johns I-Iopkins Hosp. Balt. X X X i (1917), 239--241. Eehmann, P., R. Kramer, P. Altrocchi, tIemifacial Spasm. Arch. Neurol. 25 (1971), 81--87. H a r d m a n , J., and G. Jefferson, Cerebello-pontine angle signs produced b y e p e n d y m o m a t a (cyst, turnout). Zentralbl. Ncurochir. 3 (1938), 137--145. Jefferson, G., and A. Smalley, Progressive facial palsy produced b y intratemporal epidermoids. J. Laryngol. Otol. 53 (1938), 4:17--443. - - and K. Whitehead, Papilliferous cystoma of the petrous bone associated with hypernephroma and cystic pancreas. Brit. J. Surg. 19 (1931), 55--62. Johnstone, J. M., ]3. Lennox, and A. J. Watson, Five cases of Ilidradenoma of the External Auditory Meatus; So-called Ceruminoma. J. Path. Bact. L X X i g i (1957), 421 427.

Kleinsasser, O., G. Scharfetter, Adenoma of Ceruminous Gland with invasion of the dura and cerebellum. Zentralb]. Neurochir. (Leipzig) 17 (1957), 4---12. Pennybacker, J., Cholesteatoma of Petrous bone. Brit. J. Surg. 32, (1944), 75--78. Shapiro, M. S , and D. K. Ncues, Technique for removal of glomus jugulare tumours. Arch. Otolaryng. (Chicago) 79 (1964), 219--224. Stroobandt, G., and G. Corn@lis, Tumcrus de la base du crane h localisation pontoc6rebelleuse. Diagnostic diff6rential radiologique et traitcment chirurgical. Neurochirurgie 17 (1971), 11--24. Tytus, J., and J. Pcrmybacker, Pearly turnouts in relation to the C.N.S. J. Neurol. Neurosurg. Psyehiat. 19, (1956), 241--259. Authors' address: Dr. W. I~. Timperley, Consultant Ncuropathologist, The Royal Infirmary, Sheffield, S 6 3 DA, England.