Nasopharyngeal Nasopharyngeal Carcinoma Carcinoma
Dec 13, 2015
Nasopharyngeal Nasopharyngeal CarcinomaCarcinoma
IntroductionIntroduction
It is prevalent It is prevalent in Southern China,in Southern China, Southeast Asia, HongKong and Southeast Asia, HongKong and parts of East and North Africa.parts of East and North Africa.
High index of suspicion required for early High index of suspicion required for early diagnosisdiagnosis
AnatomyAnatomy
Anteriorly -- nasal cavityAnteriorly -- nasal cavity Posteriorly -- skull base and vertebral Posteriorly -- skull base and vertebral
bodiesbodies Inferiorly -- oropharynx and soft palateInferiorly -- oropharynx and soft palate Laterally -- Laterally --
Eustachian tubes and toriEustachian tubes and tori Fossa of Rosenmuller - most common Fossa of Rosenmuller - most common
locationlocation
AnatomyAnatomy
Close association with skull base Close association with skull base foramenforamen
Mucosa Mucosa Epithelium - tissue of origin of NPCEpithelium - tissue of origin of NPC
Stratified squamous epitheliumStratified squamous epithelium Pseudostratified columnar epitheliumPseudostratified columnar epithelium
Salivary, Lymphoid structuresSalivary, Lymphoid structures
EpidemiologyEpidemiology
Chinese native > Chinese immigrant > Chinese native > Chinese immigrant > North American nativeNorth American native Both genetic and environmental factorsBoth genetic and environmental factors
GeneticGenetic HLA histocompatibility loci possible markersHLA histocompatibility loci possible markers
EpidemiologyEpidemiology
EnvironmentalEnvironmental VirusesViruses
EBV- well documented viral “fingerprints” in EBV- well documented viral “fingerprints” in tumor cells and also anti-EBV serologiestumor cells and also anti-EBV serologies
Nitrosamines - salted fishNitrosamines - salted fish Others - polycyclic hydrocarbons, chronic Others - polycyclic hydrocarbons, chronic
nasal infection, poor hygiene, poor nasal infection, poor hygiene, poor ventilationventilation
ClassificationClassification
WHO classesWHO classes Based on light microscopy findingsBased on light microscopy findings
Type I - “SCCA”Type I - “SCCA” 25 % of NPC25 % of NPC moderate to well differentiated cells similar moderate to well differentiated cells similar
to other SCCA ( keratin, intercellular to other SCCA ( keratin, intercellular bridges)bridges)
ClassificationClassification
Type II - “non-keratinizing” carcinomaType II - “non-keratinizing” carcinoma 12 % of NPC12 % of NPC variable differentiation of cells ( mature to variable differentiation of cells ( mature to
anaplastic)anaplastic) minimal if any keratin productionminimal if any keratin production may resemble transitional cell carcinoma of may resemble transitional cell carcinoma of
the bladderthe bladder
ClassificationClassification
Type III - “undifferentiated” carcinomaType III - “undifferentiated” carcinoma 60 % of NPC, majority of NPC in young 60 % of NPC, majority of NPC in young
patientspatients Difficult to differentiate from lymphoma by Difficult to differentiate from lymphoma by
light microscopy requiring special stains & light microscopy requiring special stains & markersmarkers
Diverse groupDiverse group Lymphoepitheliomas, spindle cell, clear cell and Lymphoepitheliomas, spindle cell, clear cell and
anaplastic variantsanaplastic variants
ClassificationClassification
Differences between type I and Differences between type I and types II & IIItypes II & III
5 year survival5 year survival Type I - 10% Types II, III - 50%Type I - 10% Types II, III - 50%
Long-term risk of recurrence for types II & IIILong-term risk of recurrence for types II & III Viral associationsViral associations
Type I - HPVType I - HPV Types II, III - EBVTypes II, III - EBV
Clinical PresentationClinical Presentation
Often subtle initial symptomsOften subtle initial symptoms unilateral hearing loss (SOM)unilateral hearing loss (SOM) painless, slowly enlarging neck masspainless, slowly enlarging neck mass
Larger lesionsLarger lesions nasal obstructionnasal obstruction epistaxisepistaxis cranial nerve involvementcranial nerve involvement
Clinical PresentationClinical Presentation
Xerophthalmia - greater sup. petrosal nXerophthalmia - greater sup. petrosal n Facial pain - Trigeminal n.Facial pain - Trigeminal n. Diplopia - CN VIDiplopia - CN VI Ophthalmoplegia - CN III, IV, and VIOphthalmoplegia - CN III, IV, and VI
cavernous sinus or superior orbital fissurecavernous sinus or superior orbital fissure
Horner’s syndrome - cervical sympatheticsHorner’s syndrome - cervical sympathetics CN’s IX, X, XI, XII - extensive skull base CN’s IX, X, XI, XII - extensive skull base
Clinical PresentationClinical Presentation
Nasopharyngeal examinationNasopharyngeal examination Fossa of Rosenmuller most common locationFossa of Rosenmuller most common location Variable appearance - exophytic, submucosal Variable appearance - exophytic, submucosal
Regional spreadRegional spread Usually ipsilateral first but bilateral not Usually ipsilateral first but bilateral not
uncommonuncommon
Distant spread - rareDistant spread - rare
Diagnose Diagnose
Biopsy under naso-endoscopy Biopsy under naso-endoscopy
— — Gold standardGold standard
— — sometimes repeated biopsy is sometimes repeated biopsy is needed needed
— — additional immunohistochemistryadditional immunohistochemistry
Radiological evaluationRadiological evaluation
Contrast CT with bone and soft tissue Contrast CT with bone and soft tissue windowswindows imaging tool of choice for NPCimaging tool of choice for NPC
MRIMRI soft tissue involvement, recurrencessoft tissue involvement, recurrences
Chest CT, bone scans Chest CT, bone scans
Nasopharyngeal carcinoma. A: Axial contrast-enhanced computed tomography (CECT) demonstrates enhancing lesion (asterisk) involving the pharyngeal mucosa space, retropharyngeal spaces, and prevertebral space. A tumor abuts the skull base. B: Axial CECT image with bone settings at the level of the skull base demonstrates a lytic destructive lesion involving the anteromedial left petrous bone (asterisk), medial portion of greater sphenoid wing (arrowhead),and adjacent clivus (arrow).
Laboratory evaluationLaboratory evaluation
Special diagnostic testsSpecial diagnostic tests IgA antibodies for viral capsid antigen (VCA)IgA antibodies for viral capsid antigen (VCA)
very popular in China very popular in China IgG antibodies for early antigen (EA)IgG antibodies for early antigen (EA)
StagingStaging
Variety of systems usedVariety of systems used Am Jt Comm for Ca StagingAm Jt Comm for Ca Staging International Union Against CaInternational Union Against Ca Ho SystemHo System
Unique NPC prognostic factors often not Unique NPC prognostic factors often not considered and similar prognosis considered and similar prognosis between stagesbetween stages
TreatmentTreatment
External beam radiation: External beam radiation: first choicefirst choice Dose: 6500-7000 cGyDose: 6500-7000 cGy Primary, upper cervical nodes, pos. lower Primary, upper cervical nodes, pos. lower
nodesnodes Consider 5000 cGy prophylactic tx of Consider 5000 cGy prophylactic tx of
clinically negative lower neckclinically negative lower neck
Adjuvant brachytherapyAdjuvant brachytherapy mainly for residual/recurrent diseasemainly for residual/recurrent disease
TreatmentTreatment External beam radiation - complicationsExternal beam radiation - complications
IncludeInclude xerostomia, tooth decayxerostomia, tooth decay ETD - early (SOM), later (patulous ET)ETD - early (SOM), later (patulous ET) Endocrine disorders - hypopituitarism, Endocrine disorders - hypopituitarism,
hypothyroidism, hypothalamic disfunctionhypothyroidism, hypothalamic disfunction Soft tissue fibrosis including trismusSoft tissue fibrosis including trismus Ophthalmologic problemsOphthalmologic problems Skull base necrosisSkull base necrosis
TreatmentTreatment Surgical managementSurgical management
Primary lesion Primary lesion consider for residual or recurrent diseaseconsider for residual or recurrent disease approachesapproaches
infratemporal fossa infratemporal fossa transparotid temporal bone approachtransparotid temporal bone approach transmaxillarytransmaxillary transmandibulartransmandibular transpalataltranspalatal
TreatmentTreatment Surgical managementSurgical management
Regional diseaseRegional disease Neck dissection may offer improved survival Neck dissection may offer improved survival
compared to repeat radiation of the neckcompared to repeat radiation of the neck
TreatmentTreatment
ChemotherapyChemotherapy Variety of agentsVariety of agents Chemotherapy + XRT - no proven long Chemotherapy + XRT - no proven long
term benefitterm benefit Mainly for palliation of distant diseaseMainly for palliation of distant disease
ImmunotherapyImmunotherapy Future treatment??Future treatment?? Vaccine??Vaccine??
ConclusionConclusion
Prevalent in Southern China, Southeast Prevalent in Southern China, Southeast Asia, HongKong. And rare in North Asia, HongKong. And rare in North America, and EuropeAmerica, and Europe
Biopsy is the gold standard for Biopsy is the gold standard for diagnosis.diagnosis.
Treatment is primarily Radiation, not Treatment is primarily Radiation, not surgery. surgery.