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Show '" 1986 Raven Press, New York Squamous Cell Carcinoma of the Pterygopalatine Fossa (Retroantral Space) Michael L. Slavin, M.D. and Allan L. Abramson, M.D. A patient devdoped sensory disturbance and pain in the distribution of the maxillary nerve several months after removal of a statedly benign cutaneous malar lesion. One year later, abducens palsy devdoped, and computed tomography showed a mass of the pterygopalatine fossa abutting on the superior nasopharynx. Results of examination and multiple biopsies of the nasopharynx were normal. Direct biopsy of the pterygopalatine fossa via a transmaxillary sinus approach revealed squamous cell carcinoma. Extension of malignancy from the adjacent nasopharynx (not detected on biopsy) or sphenoid sinus and perineural spread of an undiagnosed cutaneous squamous cell carcinoma along the maxillary nerve were considered as possibilities. The anatomy of the pterygopalatine fossa and its environs is reviewed with respect to clinical signs of second division trigeminal neuropathy, abducens palsy and diminished ipsilateral tearing. From the Division of Neuro-Ophthalmology. Dt'partnwnt of Ophthalmology (M.L.S.), and the Department of Otolar\'ngology and Communicativl' Disorders (A. L.A.). Long Island )l'wish Medical Centl'r, New Hyde Park; and School of Medicine, Health Sciences CentL'r. State UniVL'rsit\' of Nl'w York at Stony Brook, Stony Brook, New York. . Address correspondenn' 'lI1d reprint rl'qUt'sts to Michael L. Slavin, M.D. at thl' DiVision of Neuro-Ophthalmologv. Department of Ophthalmology. Long Island )l'wish Medical Cenll'r, New Hyde Park, NY 11ll42. USA. 254 Squamous cell carcinoma of the pterygopalatine fossa is usually secondary to direct extension of malignancy from adjacent structures, especially the nasopharynx, maxillary or sphenoid sinus. Perineural spread of carcinoma along the maxillary nerve (second division of the trigeminal nerve) from a primary cutaneous malignancy of the malar area could possibly result in a lesion in the pterygopalatine fossa. Dissemination of squamous cell carcinoma to this location from remote foci via the hematogenous or lymphatic routes is unlikely. Primary squamous cell carcinoma of the pterygopalatine fossa has not been described, and indeed this location is typically devoid of squamous epithelial tissue. We report a patient with a lesion of the pterygopalatine fossa whose classic signs and symptoms coincidentally began several months after removal of a statedly benign facial dermatologic lesion. After multiple nasopharyngeal biopsies were taken and diagnosed as unremarkable, surgical access to the pterygopalatine fossa through the maxillary sinus detected squamous cell carcinoma. CASE REPORT A 61-year-old woman presented to the dermatologist in August 1981 with a I-em erythematous papule on the right medial malar area of her face. A shave excision showed seborrheic keratosis. Su~sequentlyshe developed a small papule at the interior margin of the excision, which was removed and showed solar keratosis. Two months later, she noted a gradual numbing sensation of the right cheek along with neuritic-type pain. ~omputed tomography and paranasal sinus x-ray hlms were normal, and the patient was given carbamazepine for tic douloureaux. Her symptoms were unchanged until December 24, 1982, when she noted horizontal diplopia, worse on right CARCINOMA OF PTU<.YCUI'IILATINE FOSSA 255 gaze. ~he had no other associated neurologic or constitutional symptoms. On examination, vist"ll acuity and Goldmann perimetry were normal. A 20'lr, abd uction defect of the right e~'t' was notl'd. Ex,lmin,ltion of each anterior segmt.'nt reveall'd no ClJrl1l'al st,lining and there Wt're normallacrim,ll 1,1kes. Pupils and fundi were normal. Thert' was decre,lsed sl'nsation to pin prick of the right cheek. Corne,ll rt'i1l'xes were s"mmetrical. Nl) skin ll'sions in thl' millar area were l'vidt.'nt. Cr,lni,ll l1l'rves were otherwise norn1.11. Results l)f Tensillln test wen.' unremarkable. No cen'ic,ll l1r submandibular nodes were palpable. Hearing W,lS gross I" normill and symmetric on t'ach side. Nasl1pharyngeal, oral and middle ear examinations were normal. Computed tomograph~' (Fig. 1) demonstrated a 3 x 1.5 cm soH tissue mass posterior to the right maxillary sinus, with erosion of the right pterygoid processes and sphenoid bone. The mass was abutting on the superior nasopharynx and had eroded the anterolateral wall of the sphenoid sinus. There was no radiologic evidence of intracranial invoh·ement. Multiple superficial and deep biopsies of the right nasopharyngeal wall were performed and failed to reveal an abnormality. Afterwards, the pterygopalatine fossa was approached by removal of the posterior wall of the maxillary antrum. Biopsy of a soft tissue mass in this location revealed squamous cell carcinoma. Radiotherapy (6,000 rad) was subsequently ad-ministered to the upper neck, primary tumor site, and base of the skull. Over the next year, the patient developed a total right sixth nerve palsy, superior division third nerve palsy, and corneal anesthesiil with computed tomographic evidence of extension of the tumor to superior orbital fissure, posterior orbit and temporal lobe. In this followup period, there was no clinical or radiologic involvement of the nasopharynx. There was no recurrence of the cutaneous lesion, and review of all pilthologic sections of the initial dermatologic lesion revealed no evidence of malignancy. The patient died several months later and postmortem examination was not performed. DISCUSSION The pterygopalatine fossa (1,2) is situated directly posterior to each maxillary sinus (Fig. 2). It is bordered posteriorly by the fused medial and lateral pterygoid plates of the sphenoid bone. Inferiorly the fossa narrows and becomes the pterygopalatine canal (which communicates with the oral cavity) while superiorly it is continuous with the inferior orbital and superior orbital fissures. Laterally there is direct communication with the infratemporal fossa and medially the pterygopalatine fossa is bound by the perpendicular plate of the palatine bone, which separates it from the nasal cavity. The sphenopalatine foramen (Fig. 2) is at the upper border of the palatine bone where it articulates with the body of the sphenoid and con- FIG. 1. Computed axial tomography at level of maxillary sinus showing soft tissue mass of the pterygopalatine fossa (straight arrow). (a) Note erosion of the body of the sphenoid bone and antero-Iateral wall of the sphenoid sinus (curved arrows). The mass abuts on the superior nasopharynx (straight open arrow). A normal contralateral pterygopalatine fossa is noted (arrowhead). (b) Erosion of the pterygoid laminae is seen (arrow). M. L. SLA VIN AND A. L. ABRAMSON {a) (b) G nects the nasal cavity and pterygopalatine iossa. In all, eight passageways (3) (foramina and iissures), which connect the pterygopalatine fossa with the orbit, middle cranial fossa, nasopharynx, oral cavity and infratemporal fossa, are known to exist. The pterygopala tine fossa houses the splwnopalatine ganglion and the maxillary artery. The maxillary nerve (second division of the trigeminal nerve) proximally traverses the pterygopalatine space on route to the middle crani.ll iossa via the foramen rotundum. The miljor branch t)i the maxillary nerve is the infrilorbital nerve, which innervates the skin of thL' cheek, side oi the nose ,lnd upper lip. This brilnch pilSSL'S through the infraorbital canal and then through the inil'rior orbitill FIG. 2. Right lateral (a) and right inferolateral (b) views of dried skull, with mandible removed. Key: A = pterygopalatine fossa, B = inferior orbital fissure, C = lateral pterygoid lamina of sphenoid bone, 0 = posterior wall of maxillary sinus (antrum), E = foramen ovale, F = foramen lagerum, G = zygomatic arch, H = naasal cavity. I = sphenopalati ne foramen. iissure to the pter~'gopalatine space to join the maxillary nerve. The sphenopalatine ganglion (-1) is the parasympathetic relay station of secretory neurons to the lacrimal gland, mucous membrane of the superior and middle turbinates of the nose, "nd the hard and soft plate. The preganglionic secretory neurons originate in the pons in an area near the superior salivary nucleus, emerge with the nervus intermedius, and join the facial nerve (cranial nerve VII). These axons separate from the iaciill nerve within the petrous pyramid and proct't.' d to the middle cranial fossa as the greater superiicial petrosal nerve. This nerve enters the Vidian canal at the anterior margin of the foramen lacerum (Fig. 2) extradurally and joins with sympathetic fibers to form the Vidian nerve. The Vid- CAI~ClNOMA or l''l'U~)C(l/'ALA'f'lNL FOSSA 257 ian nL'1 \,' proceeds anteri\lrlv to the ptL'rv~op,ll,ltine fossa to syn,lpse with post~,lnglionicIll'uruns in till' sphenopalatine g,lI1glilln. It is well knllwn th,lt p,lin ,1I1d ,llten'd senS.ltilln in the seCllJ1d divisillJ1 llf the tri~emin,ll Ill'r\'l' m,lY be eMlv signs of n,lsllph,Hvnge,ll m,llign'lnC\' (5-7). This is PWb,lblv dUL' t\l inVlllvenll'nt of the m,l\.illarv nt'rH' in till' pll'rvgllp,ll,ltilll' fllSS,l twm direct sprL',ld fwm till' n,lsupharvl1\, (pllssiblv thwugh till' sphenllp,ll,ltine fur,lIlll'n llr erosilln llf the p,ll,ltiIW blllll'). AbduCl'ns p,llsv is till' secund nlllst Clllllnllln nL'urllp,lthv ,lnd indiC,l!L's intr,lcr, lni,ll e'tt'nsilln lIt dise,lSt' through b,lSil,H fllramin, l (rlltundum, 1,1Ct'rUm, ll\·,lil'). M,lndibular Iwuropatll\' (third di\'isilln trigemin,ll neurop,lthv) ma\' llccur \\'ith innll\'enll'nt llf the fllr,lnll'n llvalt'. Diminisht'd ipsil,ltn,ll tt'aring ,1Ild drying of the nasal mUCllS,l m,l\' fllllllW frum involvt'ment of the sphellllp,ll,ltine g,lI1glil1n in the pkrygopalatint' ftlssa llr llf the \. id ia n llr grea ter su perlici.ll petrosal ner\'e in the middle cr,lnial fossa, although the latter is an unusual c1inic,ll sign. Spread of tumor thwugh the basil.1r foramina mav also give rise to Horner's s.vndwme and ma\.' affect the cranial ner\"es in the cavernLlUS sinus. Perineural spread of cutaneous sljuamLlus llr basal cell carcinoma resulting in cranial neuropathy has been reported (S-IO). Facial pain and dysesthesia are the most common presenting signs and indicate spread of tumllf aillng thl' branches of the trigeminal nerve. In ]lJS], Trobe and associates (10) reported a patient initially diagnosed as having trigeminal neuralgia who was shown on computed tomography to have a mass involving the orbits, superior orbital fissure, and cavernous sinus area. Biopsy of the lesion revealed squamous cell carcinoma. This patient was shown to have had a previous resection llf a squamous carcinoma of the skin uf the I,lkral canthus, with documented perineural invulvl'ment of cutaneous nerves. Our patient presented with sensory disturbance in the distribution of the maxillary nt'rve sl'vt'ral months following resection of a bt'nign skin Iesilln of the malar area. The possibility lli perint'ural spread of squamous cell carcinoma ,dong the maxillary nerve to the pterygopalatine fossa was an early consideration in this case. Although carci-noma may hilve been missed on initial biopsies, nu subseLJut'nt recurrence of cutaneous malignancy was dt'monstrated in the follow-up period oi three years. Extension of squamous cell carcinoma from the nilsopharynx was considered to be a strong possibility, despite the fact that no initial involvement of this art'a was demonstrable either on nasopharyngeal t'xamination or after multiple biupsies in the presumably involved areas. Two years afll'r the initial symptoms, neither computed tomography nor physical examination could pinpoint the nasopharynx as being the source of malignancy. We believe that the squamous cell carcinoma of the pterygopalatine fossa, however, was secondary to extension from a small nest of nasopharyngeal cells not detected on initial biopsies, although we cannot categorically rule out the sphenoid sinus as the source. Successful biopsv of tumor in the pterygopalatine iossa may be achieved via a transmaxillary sinus approach in cases where nasopharyngeal biopsv is unremarkable. REFERENCES Plltler CD. The ptl'ryg"p,ll,ltlnl' 1"",1 ,)nd canal ..\111 I Rd' .1,,1/ IYbLJ;I(l7~20-~. 2. O,hllrn AG. R,ldillingl' III til\' plerl'gllld pl,It .., ,lnd ~'Ier\gllpablilw Il"'a. :\I1III~'ldl'" 147Y;J:\2::l~N-Y4. :l. PI,111 eH. :\"111""1.'1'" II,,· 1,,,,1.1 1/11.1 1I1',k Phil,ld,'lphl,l. \\'B S,llinlic'r'. IY7:l:141-n 4. Ru,,,.. 11 GL. rtw d"tnhlltilln l,1 ,H1tl,nllmIC ~,,"/-g,lngl"'nJ" m'rn' til'er' tll til\' I,lcnm,ll gl,1I1d In nllln"e,·' I ,,\",1! 1471; I(lLJ:224-42. 'l. Thlll1l,l' lE. \V,)It ... AC \;,'uHIIllgll.11 m,lIllil"t,ltl,'n, ,'I n,)"' ph,HYng",11 m,llign,lIlt tunll'r' /..\.\1:\ IYt>'l:!lJ2:'I'l-S h. Glldtlr,',ben E. Ophth"lnwl,'pc ,1Ild Il\'ur,,"'~ic "mptllm, III m,llign,lnt na'llph,H\'/lge,11 tunlllr-: clinic,ll ,tu,h Cl'mprhlllg 4'l4 C,he, WIth 'P,'cl,)1 relt'renc,', t" h"k'~',lthl''''~Y .1nd pp........ibilih· ut e.Hlil'f n'(tl~nltll..'n_ ."\If,1 1\.IIt"JIIl1trtl \t'ld"t l ! IlJ44; Suppl .'4:1-.12.1. 7. Smith II.. Wh,'Ii" 1..\. l )",d.H rn,lIl1ll"t,ltl,'n, "I n,l,,'ph.lr~ ·nhl'.ll tuIlHlr..... J1,1"", -\", .-\llld l )"htftIlJ11/I ,/l )/t1l."-If1J:\II/ )l..)p2: hh:h:;q-64. S. r'!<lllrl' l·~.. Hllyt \\T. \;llrth III I'.lllllul l'f,hth,lll111lf''''g/,1 tullu\\'Ing trl'lltl'd "'qu.lnh1ll ... (.H...:1I1\1In.1 I'l thl' h.lrl'ht..'.hi: l'Thit, ll ,Ip'" inn,ln'llll'nt Ir,lrn cl'ntnf'l'/,11 'f'rl',ld ",1 Ihl' 'llpr, ll'rhil,11 nern'..\ 1,-" I :\/1,1 I<1;-(,: I:1,;7 - <I ll. Morn, ICl. 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