Braz. j. . vol.83 número5

BrazJOtorhinolaryngol.2017;83(5):600---601

www.bjorl.org

Brazilian

Journal

of

OTORHINOLARYNGOLOGY

CASE

REPORT

Postauricular

neurofibroma

---

a

rare

occurrence

夽

Neurofibroma

retro-auricular

---

uma

ocorrência

rara

Tan

Shi

Nee

_,

_Mazita

_Ami

_,

_Kong

_Min

_Han

_,

_Primuharsa

_Putra

_Sabir

_Husin

_Athar

a_{KPJHealthcareUniversityCollege,SchoolofMedicine,DepartmentofOtorhinolaryngology,Head&NeckSurgery,Selangor,}

Malaysia

b_{KPJKlangSpecialistHospital/KPJHealthcareUniversityCollege,Ear,Nose&Throat---Head&NeckConsultantClinic,Selangor,}

Malaysia

c_{UniversityKebangsaanMalaysiaMedicalCenter,DepartmentofOtorhinolaryngology,Head&NeckSurgery,KualaLumpur,}

Malaysia

d_{KPJSerembanSpecialistHospital/KPJHealthcareUniversityCollege,Ear,Nose&Throat---Head&NeckConsultantClinic,}

NegeriSembilan,Malaysia

Received18June2015;accepted14September2015 Availableonline19December2015

Introduction

Neurofibromasareatypeofbenigntumor developedfrom the myelin sheath of the peripheral nervous system and frequentlyseeninneurofibromatosistype1(NF1). Neurofi-broma of the head and neck regions is not uncommon.1

Therewereonlyafewcasesof isolatedexternal ear

neu-rofibromawithoutneurofibromatosis(NF)reported,asthey

rarelyaffecttheexternalear.1_{Itisbelievedthat}

neurofibro-matosisisautosomaldominant;thetype1neurofibromatosis

isduetodisruptivemutationoftheNF1gene,whichcodes

forproteinneurofibromin.

Case

report

A 38-year-old Malay male with no comorbidity came to

our otorhinolaryngology, head and neck clinic with the

夽 _{Pleasecitethisarticleas:ShiNeeT,AmiM,MinHanK,Sabir}

HusinAthar PP. Postauricularneurofibroma--- a rareoccurrence. BrazJOtorhinolaryngol.2017;83:600---1.

∗_{Correspondingauthor.}

E-mail:[email protected](P.P.SabirHusinAthar).

PeerReviewundertheresponsibilityofAssociac¸ãoBrasileirade OtorrinolaringologiaeCirurgiaCérvico-Facial.

complaint of a right postauricular swelling for the past

two years. He was more concerned about the cosmetic

disfiguring. The swelling was insidious at the onset and

progressivelyincreasinginsize.It didnotcauseanypain.

Therewasnohistoryofbleedingfromtheswellingandno

historyofanysuchlesionsinthebody.Nosignificantfamily

history. Examinationoftheswellingshowedpresenceof a

single4cm×5cmmass,firm,withregularsurfaceoverthe

postauricularregion.Subsequently,weperformed a

surgi-calremovalofthemassforthispatientviaapostauricular

approachundergeneralanesthesia.Themasswaspartially

adherenttotheskin.Afterremovingtheexcessskinwhich

wasadherenttoit,theentiremasswascompletelyexcised.

Histopathological report (Fig. 1) revealed that the lesion

wascomposedofspindle-shapedcellswithfusiformnuclei

arrangedin fascicles. No cellularatypia or necrosisseen.

Thesehistologicalfeaturesaresuggestiveofneurofibroma.

Patient has an uneventful recovery, and a good cosmetic

resultwasachieved.Thereis norecurrenceduringfollow

upforthepastyear.

Discussion

Neurofibromas are a benign neoplasm, derived from the

myelin sheathoftheperipheral nerve.Theymayoccur in

thepresenceofneurofibromatosis,ahereditarycondition.2

http://dx.doi.org/10.1016/j.bjorl.2015.09.001

Postauricularneurofibroma---arareoccurrence 601

Figure 1 Microphotograph showing spindle shaped cells arrangedinshortfascicles,whirlingandstoriformpattern.

Therearetwotypes ofneurofibromatosis:type1(NF1)

andtype2(NF2).

NF1 is an autosomal dominant type, which commonly

presents with peripheral nerve sheath tumors called the

neurofibromas.3_{NF1iscausedbyachangeinageneonthe}

chromosome17.NF2usuallygrowsintothespinalcordor

brainandiscausedbyachangeinchromosome22.Bilateral

vestibular schwannomas of the acoustic nerve and

multi-plemeningiomasarecharacteristic,rarely withcutaneous

manifestations.3,4

There are two types of neurofibromas: dermal and

plexiform. Dermal neurofibroma is also called discreteor

cutaneousneurofibroma.Theyusuallydevelopduring

ado-lescenceandadulthood.Theytendtoinvolvetheterminal

nervesandmaybenumerous,butthereisnoapparentrisk

ofmalignanttransformation.However,despitetheirbenign

nature,theymaycausesignificantcosmeticproblems and

occasionallyrequireremoval.

Conversely,plexiformneurofibromasareusually

congen-ital.Theydevelopedinchildhoodandareoftenextending

deeplyalongthenervesandmayinvolvealllevelsofskin,

fascia, muscle, bone and evenviscera. They can become

verylargeandmaycausefunctionalimpairment,withrisk

ofmalignanttransformationin6%inthesetumors.4,5

ChangeinthegeneticmaterialthatcausesNF1orNF2can

beinheritedfromaparent,thusreferred toasautosomal

dominant,ormayoccur duetospontaneousmutation.1 _In

ourcasereport,therewasnofamilyhistory.Solitarylesions

are not usually associated with the presence of systemic

manifestations,unlikemultiplelesions,whicharecommonly

seeninpatientswithNForvonRecklinghausen’sdisease.4

In ourcase report,the patientdid nothave any other

featuresofNF1.MosttumorscausedbyNFrequireno

treat-ment; however, when these tumors cause pain, growing

rapidly, disfiguring or impairing function, they may need

treatment.

GhoshS.Ketal.(2008)reportedacaseofneurofibroma

oftheexternalearwhereheremovedtheentirelesionalong

withexcessskin,andagoodcosmeticresultwasachieved.1

ShaidaAMetal.(2007)alsoreportedacaseof

neurofi-broma of the pinna in which surgical excision provided a

satisfactoryoutcomeandresultedinanexcellentfunctional

andcosmeticoutcome.5

Inourcase,swellingoverthepostauricularregioncausing

cosmeticdeformitywasthechiefcomplaint,andwewere

abletoobtaina satisfactory resultwithnorecurrenceon

followupforayear.

Final

comments

Neurofibromas are commonly seen in a patient with a

historyofneurofibromatosis.Inrarecasesofsolitary

neurofi-broma,dependingontheirsizeandsiteoflesion,complete

excision is possible with any significant functional and

cosmeticdeformity.Here,wereportedararecaseof

neu-rofibroma,anervesheathtumorwhichpresentedtouswith

aswellingbehindtheear,whichcausedcosmetical

disfigu-ring.Completeexcisionviathepostauricularapproachgives

asatisfactoryresultwithnorecurrence,aswasachievedin

ourcase.

Conflicts

of

interest

Theauthorsdeclarenoconflictsofinterest.

References

1.GhoshSK,ChakrabortyD,RanjanR,BarmanD.Neurofibromaof theexternalear---acasereport.IndianJOtolaryngolHeadNeck Surg.2008;60:289---90.

2.SinghS,TandonS,LahiriA,SharmaS.NeurofibromaofthePinna. IJOHNS.2014;3:66.

3.MuirD,NeubauerD,LimIT,YachnisAT,WallaceMR.Tumorigenic properties of neurofibromin-deficient neurofibroma Schwann cells.AmJPathol.2001;158:501---13.

4.Wu J, Williams JP, Rizvi TA, Kordich JJ, Witte D, Meijer D, etal.Plexiformanddermalneurofibromasandpigmentationare causedbyNf1lossindeserthedgehog-expressingcells.Cancer Cell.2008;13:105---16.