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Parapharynge al space tumors:
conside rations in 26 case s
Head and Neck Service, Department of Surgery, Faculdade de Ciências Médicas,
Universidade Estadual de Campinas (UNICAMP), Campinas, Brazil
ABSTRACT
Contex t: Parapharyngeal space tumo rs co mprise less than 0 .5 % o f all head and neck neo plasms.1 The majo rity o f these tumo rs are benign, but surgery is usually required to establish the diagno sis and treat the patients. W e present 2 6 patients treated surg ically fo r tumo rs arising in the parapharyng eal space (PPS) at the State University o f Campinas Ho spital – UN ICAMP.
Ca ses Serie: O f these, 1 7 (6 5 .5 %) had benig n and 9 (3 4 .6 %) malig nant neo plasms. The surg ical and
patho lo g ical data relevant to these cases are hig hlig hted, o bserving any lo cal recurrence, surg ical co mplicatio ns and the five-year survival. N euro g enic tumo rs and so ft tissue sarco mas were, respectively, the mo st frequent benig n (3 5 .3 %) and malig nant neo plasms (4 4 .5 %). Benig n tumo rs acco unted fo r the majo rity o f the cases and invo lved minimal surg ical mo rbidity with no recurrence during a median fo llo w-up o f five years. Malig nant tumo rs had a hig h rate o f recurrence and mo rtality. Surg ery is the treatment o f cho ice fo r PPS tumo rs. A kno wledg e o f the anato my o f this site is essential fo r the safe perfo rmance o f surg ical pro cedures. Malig nant neo plasms have a po o r pro g no sis. Fine needle aspiratio n was helpful in diag no sis o f all tumo rs.
Key W ords: Parapharyng eal Space Tumo rs. Head and N eck Tumo rs. Parapharyng eal N eo plasms. Head and N eck Surg ery.
INTRODUCTION
Parapharyngeal space (PPS) tumors are rare.1,2 They co mprise less than 0 .5 % o f all head and neck neoplasms.1 The majority of these tumors are benign, but surg ery is usually required to establish the diagnosis and treat the patients. This retrospective review focuses on the histologic distribution of these tumors, the clinical findings and some aspects of anatomy and surgical approaches.
CASES SERIE
From 1986 to 1996, we submitted 26 patients with PPS tumors to surgery in the State University of Campinas Hospital - UNICAMP. Eighteen (70%) were male with ages varying from 12 to 76 years (median: 53). The surgical approach was dependent on the tumor’s pathological type, size and area of invasion into anatomical structures. They were transcervico-submandibular, transcervical, transmandibular and c ra nio fa c ia l. All p a tie nts w e re sub mitte d to complementary diagnosis by imaging and pathological examination.
The presence o f a mass in the neck o r in the o ro pharynx (Figure 1 ) was the mo st co mmo nly enco untered sympto m (6 8 %) fo llo wed by cervical p a in (1 2 %) a nd d ysp ne a (4 %). In 1 6 % w e
Alfio José Tincani, Antonio Santos Martins Albina Altemani, Rui Carlos Scanavini Jr. Gilson Barreto, Henriette de Toledo Lage João Batista Valério, Giulianno Molina
Case R eport
REVISTA PA ULISTA DE M EDIC IN A
Medical Journal
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o b served no specific sympto ms. Fa cia l nerve dysfunctio n was present in 7 .5 % o f the patients, all o f them with malignant tumo rs.
A ll p a tie nts und e rw e nt c o mp ute riz e d tomography (CT), which correctly located the tumor sites (Figure 2 ). Angiography was performed in five patients with pulsatile masses (19.2%) and magnetic resonance imaging (MRI) was used in two (7 .7 %). As seen in Table 1 , benign tumo rs were mo st frequently enco untered (6 5 .5 %). The types were peripheric neuro genic tumo r (3 5 .3 %), pleo mo rphic adeno ma o f the paro tid (2 9 .4 %), paraganglio ma o f the caro tid bo dy (2 9 .4 %) and o ne case o f aneurysm o f the internal caro tid artery (5 .9 %). Ma lig na nc y wa s seen in 3 4 .6 %. So ft tissue sa rc o ma s a c c o unte d fo r 4 4 . 5 % , me ta sta tic squamous cell carcinoma 3 3 .3 %, and 1 1 .1 % were lympho ma and muco epidermo id carcino ma, bo th fro m the paro tid gland (Table 2 ).
Fine needle aspiration (FNA) was performed in 3 8 .5 % of all the patients, and correctly provided the diagnosis in 8 8 .9 %. O pen biopsy was required to define malignancy in 3 6 %. In the rest of them (6 4 %) surgery was perfo rmed witho ut previo us
pathologic diagnosis, mainly because of the very suggestive diagnostic impression obtained by CT.
The surgical appro ach to PPS was by the transcervico -submandibular ro ute in 1 7 cases (6 5 . 4 % ), tra nsc e rvic a l in o ne (3 . 8 % ), transmandibular in five (1 9 .2 %), and cranio facial in three patients (1 1 .5 %). Six patients (2 3 .1 %) had radiotherapy in the post-operative period (three with epidermo id carcino ma, two with sarco ma and o ne with muco epidermo id carcino ma).
O ur results with b enig n tumo rs ma y b e co nsidered satisfacto ry, since we had adequate margins in all o f them witho ut early co mplicatio ns, a nd in a me dia n fo llo w-up o f five ye a rs no recurrence has o ccurred. Two patients (7 .7 %) with ne uro g e nic tumo r o f the symp a the tic c ha in develo ped Claude-Bernard-Ho rner syndro me after the surgery. In five patients (1 9 .2 %) with paro tid pleo mo rphic adeno ma, there was tempo rary palsy o f the mandibular branch o f the facial nerve, which receded abo ut fo ur mo nths after the surgery.
O f the patients with malig nant neo plasms, 2 2 % are still alive after a median fo llo w-up o f five years. An adequate surg ical marg in was achieved in o nly o ne case (3 .8 %).
DISCUSSION
Tumors arising in the PPS represent a challenge to the head and neck surgeon. Not only because they are rare, but also because of the wide variety of histological types in this site. Anatomic knowledge is mandatory because of the presence of important structures such as the carotid and jugular vessels and the V, VII, IX, X, XII cranial nerves. Surgery is the mainstay for treatment for these PPS tumors. In o ur experience there are fo ur types o f surg ical a p p ro a c h, g uid e d b a sic a lly b y tumo r siz e , histo lo gical type and po sitio n o f the tumo r with respect to the major vessels and the styloid process. The transcervico -submandibular appro ach pro vides excellent access to the PPS, allo wing dissection of the main trunk of the facial nerve and a de q ua te va sc ula r c o ntro l. If ne c e ssa ry, the submandibular gland may be remo ved and the stylo mandibular lig ament transected fo r better expo sure o f the o perato ry field. W e o btained Ta ble 2 - Pa thologica l Dia gnosis. M a ligna nt
Tumor Type (9 / 2 6 - 3 4 .6 %)
Patho lo g ical Diag no sis n %
So ft Tissue Sarco ma 4 4 4 .5
Squamo us Cell Carcino ma 3 3 3 .3
Muco epidermo id Carcino ma 1 1 1 .1
Lympho ma 1 1 1 .1
Tota l 9 1 0 0 %
Ta ble 1 - Pa thologica l Dia gnosis. Benign Tumor Type (1 7 / 2 6 - 6 5 .5 %)
Patho lo g ic Diag no sis n %
N euro g enic 6 3 5 .3
Vag al 3
Sympathetic Chain 2
Cervical Plexus 1
Parag ang lio ma (Caro tid Bo dy) 5 2 9 .4
Mixed Tumo r o f the Paro tid 5 2 9 .4
Internal Caro tid Aneurysm 1 5 .9
Tota l 1 7 1 0 0 %
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adequate margins and no majo r co mplicatio ns occurred during the operations of the benign cases. This approach was extensively used by Myers and Carrau 3 with success.
The transcervical differs fro m the transcervico -submandibular approach in that the -submandibular triangle is no t entered.
The transmandibular appro ach was used fo r malignant tumors and always as part of a composite resectio n. Mandibulo to my is perfo rmed to achieve adequate expo sure. W hen the mandible was co mpro mised, a segmental mandibulecto my was perfo rmed fo llo wed by a tracheo sto my to avo id re sp ira to ry p ro b le ms d uring p o st-o p e ra tive reco very. The o steo synthesis was perfo rmed using a minip la te . W e use d this a p p ro a c h fo r a ll squamous cell carcinomas, for one case of sarcoma and o ne muco epidermo id carcino ma.
The infratemporal fossa approach (craniofacial surgery) was used for malignant soft tissue sarcomas (in all cases). The infratemporal fossa must be accessed in an attempt to obtain adequate margins. In this appro ach, the presence o f a neuro surg eo n is important so that the structures can be dissected easily. W e agree with So m,4 who defined the limits o f the PPS5 ,6 as fo llo ws: Superio rly, the tempo ral bo ne; medially, the buco pharyng eal facia that covers the outer aspect of the pharyngeal constrictor muscles; laterally, the facia o n the medial aspect o f the masticato r space and the facia o ver the deep surface o f the paro tid gland, bo th o f which are fo rmed by the superficial layer o f the deep cervical
facia; anterio rly the pteryg o mandibular raphe; po sterio rly, the do rsal layers o f the caro tid sheet; inferio rly, the stylo glo ssus muscle.
An important practical aspect is the division into pre- and retrostyloid compartments for which the landmark is the tensor veli palatini. The retrostyloid compartment is posterior to this facia and contains the great vessels, cranial nerves (IX to XII) and lymph no des. The prestylo id co mpartment co ntains the parotid gland and some lymphonodes.
Se ve ra l o the r la rg e re p o rts1 ,3 ,7 -1 0 fo und pleo mo rphic adeno ma to be the mo st co mmo n tumo r in the PPS, fo llo wed by neuro genic tumo rs and parag ang lio ma. In o ur series, neuro g enic benign tumo rs were the mo st frequent fo llo wed by pleo mo rphic adeno ma and caro tid bo dy tumo rs.
O ur clinical findings did not differ from other series.9,11 Pain combined with cranial nerve neuropathy were frequently associated with malignancy (56%).
The C T sc a n w a s e sse ntia l in d e fining diagno sis in mo st o f the cases, and fo r planning surg ical therapy. The advantag es o f MRI were mainly the sagittal plane view and, in so me cases, MRI distinguished the anterio r bo rder o f the tumo rs fro m the surro unding musculature.
Fine needle a spira tio n (FN A) pro vided reliable results, especially with benign tumo rs. All o f the so ft tissue sarco mas required an o pen bio psy fo r co rrect histo lo gical diagno sis and so metimes immuno histo chemistry was required.
As mentio ned ea rlier, mo st tumo rs were b e nig n a nd minima l surg ic a l mo rb id ity w a s
Figure 1 - Patient with pleomorphic adenoma of pa-rotid gland bulging into the oropharynx (arrow).
Figure 2 : CT scan in a coronal section, showing the tumor in the parapharyngeal space (arrow).
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expected. W e fo llo wed certain guidelines to avo id iatro g enic co mplicatio ns related to the surg ical pro cedure. First o f all, as described earlier, the size o f the tumo r, its relatio n to the co mpo nents o f the PPS and the index o f suspected malignancy were the main aspects to surgical planning. In additio n, we always avoided the transoral approach because its limited o perative field increased the rate o f vascular co mplicatio ns and the rate o f recurrence. We perfo rmed angio graphy o n all pulsatile tumo rs. O ther series11-13 have also reported poor results with malignant tumors and as in those reports, we also reco mmend the mo re ag g ressive surg ical procedures which are feasible with radiotherapy.
Surgery is the treatment of choice for PPS tumors. Ima g ing exa mina tio ns a nd FN A c a n pro vide diagnosis in all cases. The transcervico-submandibular approach is better for accessing benign tumors, but when malignancy is present, especially with skull base invasion, the prognosis is poor.
REFERENCES
1. Batsakis JG, Sneign N. Patho lo gy Co nsultatio n: Parapharyngeal and retropharyngeal space diseases. Ann Otol Rhinol Laryngol 1989;98:320-1. 2. Hughes III KV, Olsen KD, McCaffrey TV. Parapharyngeal space neoplasms.
Head Neck Surg 1995;17:124-8.
3. Myers E, Carrau RL. Tumors arising in the parapharyngeal space. Rev Bras Cir Cab Pesc 1994;18:1748-55.
4. So m PM, Curtin HD. Head and Neck Imaging. 3rd Editio n. Mo sby: 1996;915-51.
5. Grodinsky M, Holyoke EA. The facia and facial space of the head, neck and adjacent regions. Am J Anat 1938;63:367-408.
6. Hollinsead WH. Facia and facial spaces of the head and neck. Chapter 5. In: Anatomy for Surgeons. Vol. 1. The Head and Neck, New York, NY: Hoeber-Harper, 1954;282-305.
7. Work W, Hybens RH. A study of tumors of the parapharyngeal space. Laryngo-scope 1974;84:1748-55.
8. Sm ith PG, Sharke y DE. Exp e rie nc e with the re s e c tio n o f the parapharyngeal cancers via the infratempo ral fo ssa appro ach. Oto laryngo l Head Neck Surg 1986;94:291-301.
9. Carrau RL, Myers EN, Jo hnso n JT. Management o f tumo rs arising in the parapharyngeal space. Laryngo sco pe 1990;100:583-9.
10. Warrington G, Emery PJ, Gregory MM, et al. Pleomorphic salivary gland ad-enoma of the parapharyngeal space. J Laryngol Otol 1981;95:205-18. 11. Hughes KV, Olsen RD, McCafrey TV. Parapharyngeal space neoplasms. Head
and Neck 1995;17(2):124-30.
12. Som PM, Biller HF, Lawson W. Tumors of the parapharyngeal space, preopera-tive evaluation, diagnosis and surgical approaches. Ann Otol Rhinol Laryngol 1981;90 (Suppl.8):3-13.
13. Carrau RL, Jo hnso n JT, Myers EN. Management o f the tumo rs o f the parapharyngeal space. Head Neck 1997; 11(5):633-40.
RESUMO
Contex to: Tumo res do espaço parafaríng eo co mpreendendo meno s de 0 ,5 % do s tumo res de cabeça e pesco ço . A maio ria destes são benig no s, sendo a cirurg ia necessária para tratamento e alg umas vez es para diag nó stico do s pacientes.
Apresentamo s 2 6 pacientes tratado s cirurg icamente no Ho spital das Clínicas da UN ICAMP po r tumo res lo caliz ado s no EPF Série de ca sos: A média de idade fo i de 5 3 ano s, sendo que do s tumo res 1 7 (6 5 ,5 %) eram benig no s e , 9 (3 4 ,6 %) malig no s. Enfatiz aremo s as técnicas cirúrg icas e dado s anato mo pato ló g ico s o bservando ainda as recidivas e so brevida. Tumo res neuro g ênico s (3 5 ,3 %) e sarco mas de partes mo les (4 4 ,5 %) fo ram o s tumo res benig no s e malig no s mais freqüentes. O s tumo res benig no s apresentaram mínima mo rbidade cirúrg ica e bo m pro g nó stico , inversamente ao o co rrido co m o s malig no s. A cirurg ia é o tratamento de esco lha para estes tumo res, sendo que o s malig no s apresentaram pio r pro g nó stico . Co m exames de imag em, punção aspirativa po r ag ulha fina (PAAF) o u bió psia aberta, o btivemo s diag nó stico pré-o perató rio em to do s o s caso s.
Fro m Head and N eck Service, Department o f Surg ery, Faculdade de Ciências Médicas, Universidade Estadual de Campinas (UN ICAMP), Campinas, Braz il.
Authors
Alfio José Tinca ni
Head o f the Head and N eck Service o f UN ICAMP Antonio Sa ntos M a rtins
Assistant Pro fesso r o f the Head and N eck Service o f UN ICAMP
Albina Altem a ni
Assistant Pro fesso r o f Department o f Patho lo g y o f UN ICAMP
Rui Ca rlos Sca na vini Jr.
Resident o f the Head and N eck Service o f UN ICAMP Gilson Ba rreto
Head and N eck Surg eo n – Centro Médico de Campinas Ho spital
Henriette de Toledo La ge
Assistant o f the Head and N eck Service o f UN ICAMP Joã o Ba tista Va lério
Resident o f the Head and N eck Service o f UN ICAMP Giulia nno M olina
Resident o f the Head and N eck Service o f UN ICAMP
Sources of Funding: N o t declared Conflict of interest: N o t declared La st received: 1 6 July 1 9 9 8 Accepted: 3 Aug ust 1 9 9 8 Address for correspondence: Alfio Jo sé Tincani
Rua Pro f. Achille Bassi, 1 4 0 - casa 6 A - Cidade Universitária Campinas - SP - Brasil - CEP: 1 3 0 8 3 -5 3 0 Email: tincani@ turing .unicamp.br
