Braz. j. . vol.81 número4

BrazJOtorhinolaryngol.2015;81(4):447---450

www.bjorl.org

Brazilian

Journal

of

OTORHINOLARYNGOLOGY

CASE

REPORT

Primary

mantle

cell

lymphoma

of

the

nasopharynx:

a

rare

clinical

entity

夽

Linfoma

primário

de

célula

do

manto

da

nasofaringe:

uma

entidade

clínica

rara

Ji-Hun

Kang

_,

_Young-Dae

_Park

_,

_Chang-Hoon

_Lee

_,

_Kyu-Sup

_Cho

a_{DepartmentofOtorhinolaryngologyandBiomedicalResearchInstitute,PusanNationalUniversityHospital,Busan,}

RepublicofKorea

b_{DepartmentofPathology,PusanNationalUniversitySchoolofMedicine,PusanNationalUniversityHospital,Busan,}

RepublicofKorea

Received24January2015;accepted19February2015 Availableonline9June2015

Introduction

Mostnon-Hodgkin’slymphomas(NHL)intheheadandneck regiondevelop in the extranodallymphatic system of the Waldeyerring.1_{WithintheWaldeyerring,thenasopharynx}

is thesecond most commonsite ofdisease afterthe ton-sil.Primarynasopharyngeallymphomaismuchlesscommon, occurringinonly8%ofallNHLoftheheadandneck,2_and

diffuselargeB-celllymphoma(DLBCL)isthemostcommon histologictype.3 _{Mantlecelllymphoma(MCL) is adistinct}

subtype of B-cell lymphoma andcomprises approximately 5---10%ofalllymphomas.4_{MCLischaracterizedbyan}

aggres-sive clinical course, and there is a pattern of frequent relapse after conventional chemotherapy.4 _{MCLs involving}

the nasopharynx and oropharynxare extremely rare, and havenotbeenreportedintheliterature,tothebestofthe

夽

Pleasecitethisarticleas:KangJ-H,ParkY-D,LeeC-H,Cho,K-S. Primarymantlecelllymphomaofthenasopharynx:arareclinical entity.BrazJOtorhinolaryngol.2015;81:447---50.

∗_{Correspondingauthor.}

E-mails:choks@pusan.ac.kr,ckssmj@hanmail.net(K.S.Cho).

authors’knowledge.Thiscasereportdescribesarare clini-calpresentationofprimaryMCLarisinginthenasopharynx andextendingtotheoropharynx.Thisstudywasapproved bytheinstitutionalreviewboardofPusanNational Univer-sityHospital.

Case

report

A 66-year-old male with both nasal obstruction and ear fullnessvisitedtheauthors’clinic.Hedeniedfever,chills, and weight loss. The endoscopic examination revealed obstructionofbothposteriorchoanaebyahuge nasopharyn-gealmass,accompanied bynecroticmaterial. Nocervical lymphadenopathies were felt. Paranasal sinus computed tomography (CT) showed a homogenous solid mass with mildenhancementinvolvingbothnasopharyngealwallsand extending to the upperoropharynx (Fig.1). On magnetic resonance (MR) images, the homogenous mass demon-stratedlowsignalintensityonT1-weightedimages(T1WIs), intermediate signal intensity on T2WIs, and moderate enhancement on gadolinium-T1WIs (Fig. 2). A transnasal endoscopicbiopsyofnasopharyngealmasswasperformed. Histopathologicexamination showeddiffuse infiltration of

http://dx.doi.org/10.1016/j.bjorl.2015.02.002

448 KangJ-Hetal.

Figure1 Computedtomography(CT)ofparanasalsinus.Contrast-enhancedCTimages showabilateral,homogenous, mildly-enhancedsolidmassfromnasopharynxextendingtoupperoropharynxonaxial(A),coronal(B),andsagittal(C)view.

smalllymphocyticcellswithmildnuclearatypia(Fig.3A). Immunochemicalstainingrevealedthattheneoplastic lym-phocyteswerestronglypositiveforCD20,CD5,andcyclin D1(Fig.3B---D).ThesefindingswereconsistentwithMCL.An uppergastrointestinalendoscopy,bonescan,andCTscanof thechest,abdomen,andpelviswereallreportedasnormal.

Abonemarrowbiopsyshowednoabnormalities.Thepatient wasstaged IE according totheAnn Arborstaging system. The patientreceivedfourcyclesof R-CHOPchemotherapy and radiotherapy (total dose, 40Gy). After 24 months of post-therapyfollow-up,thepatientexhibitednoevidence ofresidualorrecurrentdisease.

PrimaryMCLofthenasopharynx 449

Figure3 Histopathologicfindingsofnasopharyngealmass.(A)Microscopicfindingshowsdiffuseinfiltrationofsmalllymphocytic cellswithmildnuclearatypia(H&E,×200).Immunohistochemicalstainingshowsstrongpositivitytoanti-CD20(B),anti-CD5(C),

andanti-cyclinD1(D)antibody(×200).

Discussion

MCL is a subtype of B-cell lymphoma, derived from CD5-positive antigen-naïve pregerminal center B-cells within the mantle zone that surrounds normal germinal center follicles.5_{MCLcellsgenerallyover-expresscyclinD1duetoa}

t(11:14)chromosomaltranslocationinthedeoxyribonucleic acid.5,6_{The causeisunknown andnoinherited}

predisposi-tionhasbeenidentified.Itaccountsfor about5%ofadult NHL inthe UnitedStates, andmoreover, theincidenceof MCL has been increasingover the last decade, especially among elderly patients.7 _{The population most commonly}

affectedconsistsofmenwithamedianageof60years.7

Clinically,MCL usuallypresents withstageIII or IV dis-easeandextensivelymphadenopathy,hepatosplenomegaly, andbonemarrowinvolvement.One-quarterofpatientsare found to also have peripheral blood involvement.5

Extra-nodaldisease occurslessfrequently, butwhen present,it typically may be found in the gastrointestinal tract and Waldeyer’s ring.6 _{In the extremely rare cases when MCL}

involvesthenasopharynx,itpresentswithanasopharyngeal mass.DescribedhereinisthefirstcasereportofMCLarising inthenasopahrynx.

MCL is diagnosed by examination of affected tissue, obtained from a biopsy of a lymph node, tissue, bone marrow,orbloodphenotype,whichshowsthetypical mor-phologyofmonomorphicsmall-tomedium-sizedlymphoid cellswithirregularnuclearcontours.8 _{Immunophenotyping}

is commonly used with MCL cells that are CD20+, CD5+, and positive for cyclin D1, whereas negative for CD10 and Bcl-6.8 _{In most of patients with MCL, t (11:14) and}

othergeneticchangescauseexcessproductionofcyclinD1, which is an early event in MCL.9 _{MCL is presently staged}

by using a modified Ann Arbor system. This patient had stageI disease at presentation, withlesions of the naso-pharynxandoropharynx,whichwassuccessfullytreatedby immunochemotherapywithradiotherapy.

MostMCLpatientsreceivetreatmentfollowingdiagnosis andstaging.Anumberofchemotherapyandrituximab com-binations,suchasR-CHOP,areusedtotreatpatients with MCL.Althoughtheadditionofrituximab,amonoclonal anti-body,has significantly improved theoverall outcome, the five-yearoverallsurvivalisaslowas40%inMCLpatients.5,10

Conclusion

ThefirstcaseofprimaryMCLarisinginthenasopharynxand extendingtotheoropharynxhasbeendescribed,whichwas successfully treated by immunochemotherapy with radio-therapy.This entity shouldbe recognizedand adequately diagnosedbecauseit mayhave a moreaggressive clinical course than other types of NHL in the head and neck. A detailedmorphologicevaluationwiththorough immunophe-notypingisessentialforanaccuratediagnosis.

Conflicts

of

interest

Theauthorsdeclarenoconflictsofinterest.

References

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