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1、,Nasopharygeal Carcinoma,Introduction,Rare in the US,more common in AsiaHigh index of suspicion required for early diagnosisNasopharyngeal malignanciesSCCA(nasopharyngeal carcinoma)LymphomaSalivary gland tumorsSarcomas,Anatomy,Anteriorly-nasal cavityPosteriorly-skull base and vertebral bodiesInferio
2、rly-oropharynx and soft palateLaterally-Eustachian tubes and toriFossa of Rosenmuller-most common location,Anatomy,Close association with skull base foramenMucosa Epithelium-tissue of origin of NPCStratified squamous epitheliumPseudostratified columnar epitheliumSalivary,Lymphoid structures,Epidemio
3、logy,Chinese native Chinese immigrant North American nativeBoth genetic and environmental factorsGeneticHLA histocompatibility loci possible markers,Epidemiology,EnvironmentalVirusesEBV-well documented viral“fingerprints”in tumor cells and also anti-EBV serologies with WHO type II and III NPCHPV-pos
4、sible factor in WHO type I lesionsNitrosamines-salted fishOthers-polycyclic hydrocarbons,chronic nasal infection,poor hygiene,poor ventilation,Classification,WHO classesBased on light microscopy findingsAll SCCA by EMType I-“SCCA”25%of NPCmoderate to well differentiated cells similar to other SCCA(k
5、eratin,intercellular bridges),Classification,Type II-“non-keratinizing”carcinoma12%of NPCvariable differentiation of cells(mature to anaplastic)minimal if any keratin productionmay resemble transitional cell carcinoma of the bladder,Classification,Type III-“undifferentiated”carcinoma60%of NPC,majori
6、ty of NPC in young patientsDifficult to differentiate from lymphoma by light microscopy requiring special stains&markersDiverse groupLymphoepitheliomas,spindle cell,clear cell and anaplastic variants,Classification,Differences between type I and types II&III5 year survivalType I-10%Types II,III-50%L
7、ong-term risk of recurrence for types II&IIIViral associationsType I-HPVTypes II,III-EBV,Clinical Presentation,Often subtle initial symptomsunilateral HL(SOM)painless,slowly enlarging neck massLarger lesionsnasal obstructionepistaxiscranial nerve involvement,Clinical Presentation,Xerophthalmia-great
8、er sup.petrosal nFacial pain-Trigeminal n.Diplopia-CN VIOphthalmoplegia-CN III,IV,and VIcavernous sinus or superior orbital fissureHorners syndrome-cervical sympatheticsCNs IX,X,XI,XII-extensive skull base,Clinical Presentation,Nasopharyngeal examinationFossa of Rosenmuller most common locationVaria
9、ble appearance-exophytic,submucosal NP may appear normalRegional spreadUsually ipsilateral first but bilateral not uncommonDistant spread-rare(3%),lungs,liver,bones,Radiological evaluation,Contrast CT with bone and soft tissue windowsimaging tool of choice for NPCMRIsoft tissue involvement,recurrenc
10、esCXRChest CT,bone scans,Laboratory evaluation,Special diagnostic tests(for types II&III)IgA antibodies for viral capsid antigen(VCA)IgG antibodies for early antigen(EA)Special prognostic test(for types II&III)antibody-dependent cellular cytotoxicity(ADCC)assayhigher titers indicate a better long-te
11、rm prognosisCBC,chemistry profile,LFTs,Staging,Variety of systems usedAm Jt Comm for Ca StagingInternational Union Against CaHo SystemUnique NPC prognostic factors often not considered and similar prognosis between stages,Staging,Neel and Taylor SystemExtensive primary tumor+0.5Sxs present 2 months
12、before dx-0.5Seven or more sxs+1.0WHO type I+1.0Lower cervical node dx+1.0-ADCC assay titer considered if available,Staging,Stage A=2,Treatment,External beam radiationDose:6500-7000 cGyPrimary,upper cervical nodes,pos.lower nodesConsider 5000 cGy prophylactic tx of clinically negative lower neckAdju
13、vant brachytherapymainly for residual/recurrent disease,Treatment,External beam radiation-complicationsMore severe when repeat treatments requiredIncludexerostomia,tooth decayETD-early(SOM),later(patulous ET)Endocrine disorders-hypopituitarism,hypothyroidism,hypothalamic disfunctionSoft tissue fibro
14、sis including trismusOphthalmologic problemsSkull base necrosis,Treatment Surgical management,Mainly diagnostic-Biopsyconsider clinic bx if cooperative patientmust obtain large biopsyclinically normal NP-OR for panendo and bxSurgical treatmentprimary lesion regional failure with local controlETD,Tre
15、atment Surgical management,Primary lesion consider for residual or recurrent diseaseapproachesinfratemporal fossa transparotid temporal bone approachtransmaxillarytransmandibulartranspalatal,Treatment Surgical management,Regional diseaseNeck dissection may offer improved survival compared to repeat
16、radiation of the neckETDBMT if symptomatic prior to XRTPost XRTobservation period if symptoms not severeamplification may be more appropriate,Treatment,ChemotherapyVariety of agentsChemotherapy+XRT-no proven long term benefitMainly for palliation of distant diseaseImmunotherapyFuture treatment?Vaccine?,Conclusion,Rare in North America,more common in China40%overall survival at 5 yearsComplete H&P,careful otologic,neurologic,cervical and NP examsThree WHO types-all from NP epitheliumTypes II,III-better prognosis,EBV assoc.Treatment is primarily XRT,
