Primary Hodgkin lymphoma of the nasopharynx: Case report

Case report/Kazuistyka

Primary Hodgkin lymphoma of the nasopharynx:

Case report

Justyna Grela-Kowalik

*, Dariusz Zatoński

, Tomasz Zatoński

1DepartmentofOtorhinolaryngologyHospital,Zgorzelec,Poland

2DepartmentofOtolaryngology–HeadandNeckSurgeryMedicalUniversityofWroclaw,Poland

Introduction

Primary extranodal location of Hodgkin lymphoma is uncommon. Hodgkin lymphoma formerly was called a cancer of the lymphatic system. Relates primarily adiseaseof thelymphnodes,extranodalsitesarerareand usuallyconcernlung, liver,bonemarrow, kidneyand skin.

Veryrarelyfigureoutsideofnodesisthefirstmanifestation ofthedisease(0.25%ofcases).

Lymphomas occupy the second place among head and neck cancer after squamous cell carcinoma. Hodgkin's lymphomaisapprox.4%totalcancerheadandneckinmost cases, it involves lymph nodes [1]. The figure outside of nodes within the head and neck is very rare and applies lymphatic Waldeyer ring consisting of tonsils, throat, lan- guage,trumpetand throatfollicles,lymphbandof therear wallthepharynx.

Theetiology is notknown todoend can playa role of EBV infection. Stands out 2 types of Hodgkin lymphoma:

classic disease(with4subtypes)anddiseaseof domination lymphocytes.

Hodgkinlymphomaofthe nasopharynxislessthan1%

totalHodgkinlymphoma.Mostofthereportedcasesinthe literatureconcernthestageIandIIEAandaftertreatment with chemotherapy and radiation showed good results [2,4].

In Poland every year recorded 1000 cases of Hodgkin lymphomamostpeopleinthe20–30years ofageand after 50 years of age. The symptoms of lymphoma are non- specific, it is characteristic swollen glands in the neck, armpit, groin, as well as persistent fever, night sweats, fatigue,weightloss,pruritus,cough.

Diagnosis is based on histopathological examination, however, it is important to also interview and imaging studies, increasingly important PET, both in the diagnosis andmonitoringofdisease[1].

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article info

Articlehistory:

Received:16.01.2017 Accepted:14.04.2017 Availableonline:25.05.2017

Keywords:

 Hodgkinlymphoma

 Nasopharyngealtumor

 Extranodal

 Primarylocation

abstract

PrimaryextranodallocationofHodgkinlymphomaisuncommon.Wepresenta caseof a43-yearoldmanwithisolatedHodgkindiseaselocalizedinthenasopharynxwhopre- sentedwithacomplaintofbilateralnasalcongestion.PrimarylocationofHodgkinlymp- homa(HL)inthenon-lymphoidtissueisveryuncommonandtheprimaryisolatedinvol- vementofthenasopharynxhasbeendescribedin35casesintheworldwideliterature.

©2017PolskieTowarzystwoHematologówiTransfuzjologów,InstytutHematologiii Transfuzjologii.PublishedbyElsevierSp.zo.o.Allrightsreserved.

*Correspondingauthorat:DepartmentofOtorhinolaryngologyHospital,Zgorzelec,Poland.

E-mailaddress:justynagrela@yahoo.com(J.Grela-Kowalik).

ContentslistsavailableatScienceDirect

Acta Haematologica Polonica

journalhomepage:www.elsevier.com/locate/achaem

http://dx.doi.org/10.1016/j.achaem.2017.04.001

0001-5814/©2017PolskieTowarzystwoHematologówiTransfuzjologów,InstytutHematologiiiTransfuzjologii.PublishedbyElsevierSp.

zo.o.Allrightsreserved.

Case report

43-Year-oldpatientreportedtothedepartmentbecauseofthe severity of symptoms in the form of nasal obstruction and impaired speech “nasal”. The first symptoms occurred three years earlier. The lymphatic tissue from the back wall of oropharynxwasbiopsiedandshowedanycancerouschanges (histopath.:inflammationchronicmucosaesineneoplasmate).

During this hospitalization endoscopic examination of the nasopharynx, stating vividly red, round tumor, filling almost all of the nasopharynx (Fig. 1) In addition, the laryngological examination there were no deviations from thenorm.ChestX-ray:nofocallesions.

Computertomographyoftheneckwasperformedwhich showed in the field nasopharynx soft tissue mass of polycyclic outline, well defined, with dimensions of 2.323.1cm.Lymphnodesoftheneckandsubmandibu- lar area are in diameter to 1cm. In the mediastinum withoutlymphadenopathy(Figs.2and3).

Inconnection withsuspectedhemangioma nasopharyn- gealpatientwasreferredtoa referencecenterof oncology, wherehehasqualifiedforendoscopicsurgerytoremovethe tumor.Because ofmajorbleeding,biopsyof atumorofthe nasopharynx,and the histopathology showed fragmentsof lymphoid tissue-covered focal normal respiratory epithe- liumwithouttumorlesion.

Dueto thelackof improvement innasal obstruction two monthslater,thepatientwasqualifiedforendoscopicsurgery toremovethetumornasopharynx,uncomplicatedpostopera- tive course. Histopathological research showed the classical Hodgkin'slymphoma(a typeof“lymphocyte–Rich”),immu- nophenotypecellsReed–Sternberg:CD30+,CD15+,MUM1+5 +PAX,Ki-67+,CD3-,CD20-,bcl2-,EBV-,LMP-Ab1-,PD1-,CD4- macroscopically;irregular,beigefragmentsoftissue,including ofdimensions.1.5cm1.3cm0.4cm.

Fig.3–Computertomographyscanofthenecksagittal projection

Fig.1–Endoscopyofthenasopharynx

Fig.2–Computertomographyscanoftheneckcoronary projection

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PET before treatment with adjuvant showed no active infiltrationHL.

TheMRneckrevealednopathological structureswithin thenasopharynxonlya singlelymphnodesina group1a, 2a,2b,3onWed0.5cm.

Ascomplementarytreatmentwasused:2coursesABVD chemiotherapyandradiotherapyIMRTphoto6MeV.

Controldouble-PET within 24-months observationwith- outactiveinfiltrationHL.

Discussion

Hodgkin's lymphoma nasopharyngeal cancer is rare and about 1% of Hodgkin's lymphomas. It occurs in patients young and middle-aged. World literature describes just over 100 cases of Hodgkin's lymphoma and nasopharyn- geal only 35 cases of this form of insulation up to the nasopharynx,withoutnodalmanifestations.Iyengaretal., in a retrospective analysis of 3500 patients with HL recordedin 40years (1967–2007)reported only34 patients with Hodgkin's lymphoma was originally located in the head andneck, andonly 9casesrelatedto thenasophar- ynx[5].

In Poland, is described one case of 35-year-old woman with isolated Hodgkin's lymphoma nasopharyngeal [3].

Eavey and Goodman have identified 2 cases of Hodgkin's lymphoma within the nasopharynx of the 500 cases of Hodgkin's lymphoma localized in the head and neck. In studiesAnselmofrom2150casesofHodgkin'slymphomain theyears1977–2001,Hodgkinlymphomawaslocatedinthe nasopharynxin7patients,andonly1patientwasaformof insulationuptothenasopharynx[7].

Epstein–Barr virus is a known factor in the etiology of Hodgkin's disease, interesting is why the nasopharynx reservoirof EBV isnot acommon location for this tumor.

Moreover, in most cases of Hodgkin lymphoma located in thenasopharynxnotshowthepresenceofEBV[8].

The most common subtype of Hodgkin's lymphoma nasopharyngealis mobile mixed type. Important in differ- entiating types of lymphoma are immunohistochemical studies. In the treatment of Hodgkin's lymphoma departs from the same radiotherapy and therapies used complex chemotherapy (ABVD=Adriamcin, Bleomycin, Vinblastine, Dacarbazine),andradiationtherapyandlymphnodesinthe necknasopharynxdoses20–50Gy.

MostpatientsareyoungmenwithstageI,IIwithagood prognosis[2,4].

Conclusions

Nasopharyngeal lymphoma is a very rare cancer of the nasopharynx,butshouldbeconsideredinthedifferentialin thecaseof tumors of the nasopharynx.Ambiguous results ofimagingtestsandstretchtendoftentoremovethetumor

in its entirety and diagnosis after histopathological exam- ination,includingimmunostaining.

Lymphoma nasopharynx can imitate changes such as mononucleosis, NK-T cell lymphoma, T-cell/B-cell lym- phoma. Accordingly, the immunohistochemical study is veryimportant.Itsobjectiveistocorrectdiagnosisandrule outotherlymphomasinthatarea[6].

Conflict of interest/Konflikt interesu

Nonedeclared.

Financial support/Finansowanie

Nonedeclared.

Ethics/Etyka

The work described in this articlehas been carried out in accordance with The Code of Ethics of the World Medical Association (Declarationof Helsinki)for experiments invol- ving humans; EU Directive 2010/63/EU for animal experi- ments;UniformRequirementsformanuscriptssubmittedto Biomedicaljournals.

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[6] CenciniE,FabbriA,LazziS,BocchiaM.Primary nasopharyngealHodgkinlymphoma.HeadNeckPathol 2015;9(December(4)):536–537[online2015Jun25].

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