+ Face!Mouth!&!Jaw!Surgery:!International!Trainee!Journal!of!Oral!&!Maxillofacial!Surgery.!2013;3(2):29D32! + + + CASE+ REPORT+
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! Carotid!Body!Paraganglioma:!! !A!Case!Report! Abstract+
L"M"Dunphy" W"S"Hislop" Department!of!Oral!and!Maxillofacial!Surgery,! Monklands!Hospital,!Airdrie,!Scotland! ! Email:
[email protected]! ! Keywords:! carotid! body! tumour,! paraganglioma,! zellballen! !
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Case!Report!
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! A! 55! year! old! female! presented! with! an! asymptomatic,! enlarging! right! lateral! neck! swelling! of! 4! months! duration.! Her! medical! history! included! Multiple! Endocrine! Neoplasia! Type!II.!She!had!a!vagal!paraganglioma!excised! from! the! right! carotid! bifurcation! in! 2004.! A! paraganglioma!was!resected!from!her!abdomen! and! right! adrenal! gland! in! 2009.! The! patient! and!her!brother!were!diagnosed!with!a!genetic! syndrome! causing! multiple! paragangliomas! in! the! form! of! succinate! dehydrogenase! gene! mutation.! She! smoked! 20! cigarettes! daily! and! did!not!consume!alcohol.!! ! Examination!revealed!a!firm!swelling!3x2cm!in! size! at! right! level! III! and! a! scar! along! the! anterior! margin! of! the! sternocleidomastoid!
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Paragangliomas+ are+ highly+ vascular+ tumours+ that+ develop+from+neural+crest+tissue1.+They+may+occur+ at+ any+ point+ along+ the+ pathway+ of+ embryologic+ migration+ extending+ from+ the+ skull+ base+ to+ the+ pelvic+ floor2.+ The+ carotid+ body+ represents+ the+ most+ common+ site+ of+ localization+in+ the+ head+ and+ neck,+ followed+ by+ the+ jugular+ bulb,+ the+ tympanic+ plexus+ and+ the+ vagus+ nerve3.+ We+ present+ a+ recurrent+ paraganglioma+ in+ a+ 55+ year+ old+ female+ who+presented+with+a+right+lateral+neck+mass.+ ! Face!Mouth!Jaw!Surg!2013;3(2):29D32!
muscle.! Haematological! investigations! revealed! no! abnormalities.! A! contrast! enhanced! CT! revealed! a! hypervascular! region! compressing! the! internal! jugular! vein! and! displacing! the! carotid! vessels! anteriorly! (figure! 1).! A! provisional! diagnosis! of! recurrent! paraganglioma!was!made.! ! Selective! angiography! was! performed! in! the! right!superior!thyroid!and!within!the!ascending! pharyngeal! artery.! Selective! embolization! was! not! possible! due! to! scarring! from! previous! surgery!and!feeder!vessel!diameter.!The!tumour! was! resected! from! the! carotid! bulb! in! a! subadventitial! plane! under! General! Anaesthesia.! ! Histopathological! examination! revealed! a! nodular,! well! circumscribed! 30x18x9mm! mass!
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Carotid!Body!Paraganglioma:!A!Case!Report!
!
Discussion!
of! pale! firm! tissue! with! cuboidal! cells! and! intervening!fibrotic!stroma!(figure!2).!! ! Immunocytochemistry! revealed! strong! tumour! cell! positivity! for! neuroendocrine! markers,! including! CD56,! synaptophysin! and! neuron! specific! enolase.! The! appearances! were! consistent! with! the! diagnosis! of! a! carotid! body! paraganglioma.!Her!recovery!was!uneventful.! !
Figure+1:+Contrast+enhanced+CT+demonstrating+a+ 2cm+ recurrent+ paraganglioma+ around+ the+ right+ carotid+bifurcation.+Note+splaying+of+the+ICA+and+ ECA+(Lyre+sign).+
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+ Figure+2:+Photomicrograph+showing+ haematoxylin+and+eosin+stained+tumour+cells+ arranged+in+small+groups+(Zellballen)+with+ moderate+nuclear+pleomorphism+(x+400).+
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! Paragangliomas! are! rare! in! the! head! and! neck,! representing! 3%! of! all! extraDadrenal! paragangliomas,!(85%!develop!in!the!abdomen,! 12%! in! the! chest).! They! are! divided! according! to! location:! the! carotid! body! tumour! (CBT)! at! the! bifurcation,! the! jugular! foramen! (glomus! jugulare! tumour,! vagal! paraganglioma)! and! the! middle! ear! (glomus! tympanicum! tumour).! These! tumours! may! synthesise! and! secrete! catecholamines,! although! this! is! less! common! than! with! adrenal! paragangliomas! (pheochromocytomas),! which! originate! in! the! chromaffin!cells!of!the!adrenal!medulla.!! ! The!carotid!body,!first!described!by!Von!Haller! in!1743,!is!a!highly!specialized!structure!2D6mm! in!size!located!in!the!adventitia!of!the!common! carotid! artery! bifurcation.! Physiologically,! it! functions! as! a! chemoreceptor! organ! and! is! stimulated! by! hypoxia,! hypercapnia! and! acidosis.! The! Carotid! Body! Tumour! constitutes! 60D70%! of! Head! and! Neck! paragangliomas.! It! arises! from! paraganglionic! cells! of! the! carotid! body,! which! develop! from! both! mesodermal! elements!of!the!third!branchial!arch!and!neural! elements! originating! from! the! neural! crest! ectoderm.! As! the! tumours! enlarge,! they! may! become! adherent,! invade! or! incorporate! the! carotid!arteries.!! ! The! average! age! of! onset! is! 45! years! with! a! female! to! male! ratio! of! 2.7:1.4! The! most! common! presentation! is! a! rubbery,! non! tender! mass! in! the! upper! neck! along! the! anterior! border! of! the! sternocleidomastoid! muscle.! The! lesion! is! more! mobile! laterally! than! vertically! (fontaine’s! sign).5+ Horner’s! syndrome! may! result!from!cervical!sympathetic!chain!invasion.! Carotid! sinus! syndrome! can! occur! spontaneously!or!secondary!to!head!movement! or! pressure! from! the! tumour.! Cranial! nerve! palsies!(V,!VII,!X!and!XII)!are!present!in!10%!of! cases.!Metastatic!malignant!CBT!occurs!in!5%!of! cases! with! metastasis! usually! confined! to! the!
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Face!Mouth!&!Jaw!Surgery:!International!Trainee!Journal!of!Oral!&!Maxillofacial!Surgery.!2013;3(2):29D32!
! neck.! The! differential! diagnosis! includes! medullary! thyroid! carcinoma,! carotid! bulb! ectasia!and!carotid!artery!aneurysm.6! ! On! microscopic! examination,! the! individual! tumour!cells!are!polygonal!to!oval,!arranged!in! distinctive!cell!balls,!called!Zellballen!(figure!2)! surrounded! by! sustentacular! cells.! With! immunohistochemistry,! the! chief! cells! are! positive! for! chromogranin,! synaptophysin,! neuron! specific! enolase! and! neural! cell! adhesion! molecule.7! Familial! paragangliomas! are! inherited! in! an! autosomal! dominant! pattern,! with! bilateral! tumours! seen! in! 30%.! Mutations! in! the! succinate! dehydrogenase! subunit! genes! predispose! carriers! to! paragangliomas.8! Associated! Syndromes! include! Multiple! Endocrine! Neoplasia! Type! II,! Von! HippelDLindau! Syndrome! and! Neurofibromatosis!Type!1.!! ! Carotid!Angiography!remains!the!gold!standard! of!diagnosis!and!should!demonstrate!an!intense! tumour!blush!with!splaying!of!the!ICA!and!ECA! (Lyre! sign,! figure! 1).9! MRI! produces! a! characteristic! “salt! and! pepper”! appearance,! representing! a! combination! of! punctuate! regions!of!haemorrhage!or!slow!flow!(salt)!and! flow!voids!(pepper).9!+! ! Complete! resection! of! the! primary! mass! is! the! treatment! of! choice,! with! the! first! successful! excision! performed! by! Albert! in! 1889.10! In! 1940,! GordonDTaylor! demonstrated! a! safe! subadventitial! plane! of! tumour! dissection.10! Shamblin! et! al+ suggested! surgical! classification! of! CBTs! into! three! groups! and! established! that! the!risk!of!surgical!intervention!depends!mainly! on! the! relationship! of! the! tumour! with! the! carotid! vessels! (Table! 1).11! If! surgical! intervention! is! considered! too! risky,! conventional!radiotherapy!may!be!considered.!! ! ! ! !
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Grade
Definition
I
Localized and easily resected.
II
Tumour adherent to or partially surrounding the vessels. Resectable with careful subadventitial dissection. Carotids completely encased by tumour. Partial or complete vessel resection and replacement required.
III
Table+1+++Shamblin+et+al10+classification+of+carotid+ body+tumours.+
! ! Although! rare,! carotid! body! paraganglioma! is! still! a! pathology! that! we! encounter! and! should! be! included! in! the! differential! diagnosis! of! a! painless! lateral! neck! mass.! This! case! demonstrates! the! need! for! extra! vigilance! to! enable! early! disease! detection! in! the! familial! setting! of! recurrent! CBT,! in! order! to! decrease! the! surgical! morbidity! associated! with! disease! progression.! ! !
Key!Learning!Points! !
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! 1.!Paragangliomas!are!highly!vascular!tumours! that!develop!from!neural!crest!cells.!They!are! rare!in!the!head!and!neck,!accounting!for!3%!of! all!extra!adrenal!paragangliomas.!! ! 2.!The!most!common!presentation!of!a!carotid! body!paraganglioma!is!a!painless!neck!mass,! occasionally!pulsatile,!demonstrating!a!bruit!or! thrill.! ! 3.!Paragangliomas!commonly!occur!sporadically! but!may!be!familial!as!part!of!Von!HippelGLindau! Syndrome,!Neurofibromatosis!or!MEN!II.!! ! 4.!Complete!resection!of!the!primary!mass!is!the! treatment!of!choice.!!In!familial!or!bilateral! cases,!other!synchronous!and!metachronous! paragangliomas!should!be!excluded.! ! 5.!Paragangliomas!have!a!high!propensity!to! recur!particularly!in!the!gene!mutation! syndromes.!!!!!!!!!!!!!!!!
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References!
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Carotid!Body!Paraganglioma:!A!Case!Report!
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1.
Nitsche! M,! Prinz! M,! Hermann! RM! et! al.! Paraganglioma! of! the! cerebellum:! case! report! and! review! of! the! literature.! Int! J! Clin!Oncol!2005:10:447D452.! 2. Lee! JH,! Barich! F,! Karnell! LH! et! al.! National! cancer! data! base! report! on! malignant! paragangliomas! of! the! head! and! neck.! Cancer!2002:94:730D737.! 3. Thabet! MH,! Kotob! H.! Cervical! paragangliomas:! diagnosis,! management! and! complications.! J! Laryngol! Otol! 2001:115:467D474.! 4. Ridge! BA,! Brewster! DC,! Darling! RC,! Cambria! RP,! LaMuraglia! GM,! Abbott! WM:! Familial! carotid! body! tumours:! incidence! and! implications.! Ann! Vasc! Surg! 1993,! 7:190D194.! 5. Patetsios! P,! Gable! DR,! Garrett! WV,! Lamont! JP,! Kuhn! JA,! Shutze! WP! et! al.! Management! of! carotid! body! paragangliomas! and! reviews! of! 30! years! experience.! Ann! Vasc! Surg.2002,!May,!16(3):331D8.!! 6. Miller! HF,! Lawson! W,! Som! P! et! al.! Glomus! vagale! tumours.! Ann! Otol! Rhinol! Laryngo! 1989,!98:21D26.! 7. Barnes! L,! Tse! L,! Hunt! J,! Michaels! L.! Tumours! of! the! paraganglionic! system.! Introduction.! In:! Delellis! R,! Heitz! P,! Eng! C,! Lloyd! R.! WHO! Classification! of! Tumours.! Pathology!and!Genetics.!2nd!ed.!2004.! 8. Wieneke!J,!Smith!A.!Paraganglioma:!Carotid! Body! Tumour.! J! Head! and! Neck! Pathology! 3(4):303D306.!Dec!2009.! 9. Ayra!S,!Rao!V,!Juvekar!S,!Dcruz!AK.!Carotid! Body!Tumours:!Objective!criteria!to!predict! the! Shamblin! Group! on! MR! Imaging.! Am! J! Neuroradiology!29:1349D1354,!Aug.!2008.! 10. GordonDTaylor! G! (1940).! On! Carotid! Tumours.!Br!J!Surg!28:163D172.! 11. Shamblin! WR,! ReMine! WH,! Sheps! SG! et! al.! Carotid! body! tumour! (chemodectoma).! Clinicopathologic! analysis! of! ninety! cases.! Am!J!Surg!1971;!122:!732D39.!
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