An approach to trans-spatial and multi-spatial processes ......• Plexiform neurofibroma...

An approach to trans-spatial and multi-spatial processes

in the head and neck RemyLobo,MDNeuroradiology

UniversityofMichiganUniversityofUtah

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•  Specialthanks:• AshokSrinivasan• KristineMosier•  EdwardP.Quigley• RichardWiggins

Up front definitions

•  Transspatiallesionsoccupyorinvolvemultiplecontiguousspaces

• Multispatiallesionsinvolvemultiplespaces,thoughinaNON-contiguousfashion

Transspatial categories

• Congenital•  Formpriortofascialclosure(e.g.veno/lymphaticmalformation)

•  Inflammatory/Infectious•  Cellulitis/phlegmon/abscesscanaffectsurgicalmanagement

• Benigntumors•  Nerveorigin(schwannoma)orvascular(hemangioma)

• Malignanttumors•  Pharyngealmucosalsurfacesquamouscellcarcinoma(SCCa)caninvademultiplecontiguousspaces(bewareperineuraltumorspread)

Multispatial categories

• Congenital•  Syndromicpresentations(PHACES,NF,etc…)

•  Infectious/Inflammatory•  Suppurativeortuberculousnodes

• Malignanttumors•  SCCaorlymphomaoftheaerodigestivetract,andnodalburden

Case 1

•  24yoMwithfacialswelling

Transspatial or Multispatial?

Transspatial

•  T2hyperintensefromsubmucosalsurfaceoforopharynxtothesublingualandsubmandibularspace•  Extensiontothemasseter(masticatorspace)andelevationoftheSMAS•  SMAS=superficialmusculatureaponeurosissystem,facialexpression

T2 hyperintense

•  Initiallybelievetobealymphatic(orvenolymphatic)malformation

• U/Sguidedaspirationwasperformedinclinic

What biochemical or cytological analysis can be performed upon aspiration of the lesion to prove it is a ranula (i.e. salivary in origin)?

•  Lipase• Amylase• Protein• Cellcount•  alpha-fetoprotein

What biochemical or cytological analysis can be performed upon aspiration of the lesion to prove it is a ranula (i.e. salivary in origin)?

•  Lipase• Amylase• Protein• Cellcount•  alpha-fetoprotein

Ranula

•  Sublingualspace/glandmucousretentioncyst•  Simple–unilocularsublingualspacecyst•  Unilateral=oval•  Bilateral=horseshoe

• Diving–ruptureintothesubmandibularspace

Ranula

• OftenmatchesCSF(T2FSbestsequence)• Peripherymayenhance

• DDx:lymphaticmalformation,dermoid(fat),epidermoid(DWI),BCC,nodes,abscess,mucocele(SMG)orsialocele(true/false)

•  Treatmentiscontroversial

Case 2

•  3yoMwithneckmass

Transspatial or Multispatial? Transspatial

•  Fatattenuating/intensitysignalfrompleuralmarginintoneuralforamina,superiorlyontovisceralspace,parapharyngealspaceandposteriorcervicalspace• Vascularandneuronalencasement• Minimalseptaeassociated

What is the most likely diagnosis for this transspatial fat mass in a 3 year old male?

•  Lipoblastoma•  Liposarcoma•  Lymphangioma• Ganglioneuroma• Rhabdomyosarcoma

What is the most likely diagnosis for this transspatial fat mass in a 3 year old male?

•  Lipoblastoma•  Liposarcoma•  Lymphangioma• Ganglioneuroma• Rhabdomyosarcoma

Lipoblatoma/Lipoblastomatosis

• Raretumorofinfancy/childhood• Postnatallipoblastproliferation(whitefat)•  80-90%diagnosedby3years,rapidlygrowingneckmassinamale(3:1)•  Lipoblastoma–focal/circumscribedclassicallysuperficial•  Lipoblastomatosis–diffuseinfiltrativeformindeepertissue(pronetorecur)

Lipoblatoma/Lipoblastomatosis

• DDx:liposarcoma(uncommon),lymphangioma(vascular/lymphatic),ganglioneuroma(neuronaltissue),rhabdomyosarcoma(mesoderm)

•  Someproposethatlipoblastomamaydifferentiateintomaturelipoma(ifgiventhechance)

Lipoblatoma/Lipoblastomatosis

• Variableadipocytes,mesenchymalcells,myxoidmatrix,welldefinedseptaeandfinevascularnetwork• Keytodistinguishfrommyxoidvariantliposarcoma,uncommonindemographic

• Completeexcisionisideal•  12-25%canrecur

Case 3

•  56yoFwithfaceswelling

Transspatial or Multispatial?

Transspatial

• MixedgasandfluidattenuationthroughtheSMAS,SMS,SLS,MS,PS,PPS,RPSontotheCS

•  Lotsofspaces!!Buttheyalltouch

•  Infectioncanspreadquickly

What is the most likely cause of this transspatial infectous process?

•  Trauma• Dental• Hematogenous•  Iatrogenic• Neoplastic

What is the most likely cause of this transspatial infectous process?

•  Trauma• Dental• Hematogenous•  Iatrogenic• Neoplastic

Dental infection

•  1-4%ofERvisits(non-traumaticdental)• Oftenuninsured,untreated(preventable)chronicinfections

• Apicallucency,disruptsadjacentcortex,directrouteofspreadalongmuscleandfascialplanes

Dental infection

• Over700differentbacteriamakeuptheoralmicrobiome•  100millionorganisms/mLsaliva

•  “themouthisadirtyplace”

•  Lookfordrainablefluidcollections•  Treatmentissurgical,antimicrobial

Case 4

•  56yoFwithgeneticdisorder(unspecified)

Transspatial or Multispatial?

Multispatial

• RightorbitalperipherallyT2hyperintense,centrallyhypointensetarget-likelesion• Additionallesionontheleftscalp

•  Enhancingleftbrachialplexuslesionwithsimilarcharacteristics

Which chromosome is abnormal in this patient?

•  3•  9•  16•  17•  22

Which chromosome is abnormal in this patient?

•  3•  9•  16•  17•  22

Neurofibromatosis (type I)

• Autosomaldominant,mutatedtumorsuppressorprotein(neurofibromin),leadstouncontrolledproliferation

• Plexiformneurofibroma(transspatial)isanNF-1defininglesion

• Historyishelpful!

Multiple additional NF-1 associations

•  FASI–dentatenuclei,GP,brainstem,thalamus,HF•  Lambdoidsuturedefectandsphenoiddysplasia•  ICAstenosis,Moyamoya• Pheo,PTadenomas•  Thoracicmeningoceles• Pseudoarthroses(longbones)

Case 5

•  28yoMwithALL,febrileneutropenia

• d

Transspatial or Multispatial?

• d

Transspatial

•  Ethmoidopacification,nonenhancingmucosa,directextensionintothemedialrightorbit•  Extensiveinflammatorystranding• MayhavehypointenseT2signal

What is the most common causative organism in neutropenic patients like the one shown?

• Aspergillus• Rhizopus• Mucor• Absidia•  Staphaureus

What is the most common causative organism in neutropenic patients like the one shown?

• Aspergillus• Rhizopus• Mucor• Absidia•  Staphaureus

AIFRS (Acute Invasive Fungal RhinoSinusitis)

• Rapidlyprogressivetransmucosalfungalinfection,perivascularspreadcommon• Canbehyperattenuating(mineralization)• VariableT1signal,oftenlowonT2(possiblyrelatedtoMn,othermetalliccompounds)• Nonenhancingmucosa=blacknecroticeschar

• Mortalitycanbe50-80%•  Treatmentissurgical,antimicrobial,andaddressunderlyingcause(PMNLs!)

AIFRS

• Neutropenic=Aspergillus• DMpatients=Zygomycetes

• Poorpredictorsincludeorbitalorintracranialspread,inabilitytoaddresstheunderlyingcause

• Checklist:cavernoussinus,vasculature,cranialnerves,brainparenchyma

Case 6

•  75yoMwithhoarseness

Transspatial or Multispatial?

Transspatial

• Relativelyhyperattenuatingmasscenteredinthesupraglotticlarynx• Aryepiglotticfoldthickening,invasionofthepostcricoidspaceandpiriform• Paraglotticinfiltrationandextensionthroughthethyroidcartilageintotheoverlyingstrapmusculature

What is the most common laryngeal site for squamous cell cancer to present?

•  Supraglottis• Glottis•  Subglottis• Allareequal

What is the most common laryngeal site for squamous cell cancer to present?

•  Supraglottis• Glottis•  Subglottis• Allareequal

Which laryngeal site rarely has positive (metastatic) nodes at presentation?

•  Supraglottis• Glottis•  Subglottis• Allareequal

Which laryngeal site rarely has positive (metastatic) nodes at presentation?

•  Supraglottis• Glottis•  Subglottis• Allareequal

Squamous cell carcinoma (larynx)

• Glotticismostcommon(thiscaseisasupraglotticprimaryhowever)• Glotticisleastlikelytohavenodalinvolvement(limitedlymphatics)•  Supraglottic/subglotticcommonlyhavenodalspreadofdisease

•  SmokingandEtOHaremajorriskfactors,leadto“fieldcancerization”

Squamous cell carcinoma (larynx)

• Accuratestagingrequiresknowledgeofvocalcordfunction(cordparesisheraldsatLEASTaT3category)•  InvasionbeyondthethyroidcartilageindicatesaT4acategory•  T4breservedformediastinal,prevertebralorcarotidinvolvement

•  FeaturesofENE:infiltrativefatplanes,irregularnodalborder,tetheringoftheadjacenttissues

Thank you for your time

• Pleaseemailremylobo@med.umich.eduwithanyquestions,commentsorfeedback

An approach to trans-spatial and multi-spatial processes

in the head and neck RemyLobo,MD

remylobo@med.umich.eduUniversityofMichiganUniversityofUtah