Benign tumors of jaw

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radio-graphical description of benign tumors of jaw

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Benign tumors of jaw

  1. 1. Benign tumors ofjawMODERATED BY:DR. SUNITA GUPTADR. SHALINIDR. DEEPMALADR. VIPULDR. ANSHUMBy:VARUN SURYA1
  2. 2. The term " benign " implies mildand non-progressiveA benign tumor is a tumor thatlacks all three of the malignantproperties of a cancer.Thus, by definition, a benigntumor does not grow in anunlimited , aggressive manner,does not invade surroundingtissues , and does not2
  3. 3. Features Benign tumor Malignant tumorPeriphery Smooth, well defined,Encapsulated,corticatedIll defined border, lackof cortication , absenceof encapsulationSurrounding tissue Compressed InvadedSize Usually small Often largerGrowth rate Slow RapidSpread Direct extension MetastasisLocation Specific anatomic site AnywhereInternal structure RO , RL , mixed RadiolucentEffect on surrounding Resorb teeth root ,displace teeth in abodily fashion withoutcausing loose teethDestroy supportingalveolar bone so thatteeth may appearfloating in space3
  4. 4. Benign tumors represent a new uncoordinatedgrowthBenign tumors are slow-growing and spread bydirect extension and not by metastases.They tend to resemble the tissue of originhistologically.It is thought that benign tumors have unlimitedgrowth potential.Often hamartomas are included in the category ofbenign tumors.However, hamartomas are overgrowths ofdisorganizednormal tissue that have a limited growthpotentialFor example, an odontoma is a hamartoma of 4
  5. 5. Clinical FeaturesBenign tumors typically have an insidious onsetandgrow slowly.These tumors usually are painless ,do notmetastasize, and are not life threateningunless theyinterfere with a vital organ by direct extension.Benign tumors are usually detected clinically byenlargement of the jaws or are found during aradiographic examination.5
  6. 6. Radiologic featuresLOCATIONMany tumors have a specific anatomic predilectionodontogeniclesionsoccur in the alveolarprocessesAbove the inferior alveolar nervecanal, where tooth formationoccursVascular andneural lesionsmay originate inside themandibular canal, arising fromthe Neurovascular tissuesCartilaginoustumorsoccur in jaw locations whereresidual cartilaginous cells lie, suchas around the mandibular condyle.6
  7. 7. PERIPHERY AND SHAPEBenign tumors enlarge slowly by formation of additionalinternal tissue.Because of this, the radiographic borders of benigntumors appear relatively smooth, well defined, andsometimes corticated.If the tumor produces a calcified product-forexample, abnormal tooth material or abnormal bone-the most mature part of the tumor will be in thecentral region with the most immature aspect at theperiphery.This sometimes results in a radiolucent band of softtissue or capsule at the periphery where the calcifiedproduct has not yet formed; this band separates themore mature internalradiopaque portion from the surrounding normalbone. 7
  8. 8. INTERNAL STRUCTUREIt may be completely radiolucent or radiopaqueor maybe a mixture of radiolucent andradiopaque tissues.If the lesion contains radiopaque elements,these structures usually represent eitherresidual bone or a calcified material that isbeing produced by the tumor.For instance curved septa that arecharacteristic in ameloblastoma representresidual bone trapped inside the tumor that hasremodeled into curved septa.The ameloblastoma does not produce bone..8
  9. 9. EFFECTS ON SURROUNDING STRUCTURESa benign tumor exerts pressure on neighboringstructures, resulting in the displacement of teeth orbony cortices.If the growth is slow enough, there will be adequatetime for the outer cortex to remodel in response to thepressure,resulting in an appearance that the cortex hasbeen displaced by the tumor .This is caused by simultaneous resorption of bonealong the inner surface (endosteal) of the cortex anddeposition of bone along the outer cortical surface bythe periosteumThrough this remodeling process, the cortexmaintains its integrity and resists perforation.Benign tumors may also cause bodilydisplacement of near by teeth .9
  10. 10. The roots of teeth maybe resorbed by either benignor malignant tumors, but root resorption morecommonly is associated with benign processes.The benign tumors especially likely to resorbroots are ameloblastomas, ossifying fibromas, andcentral giant cell granulomas.Benign tumors tend to resorb the adjacent rootSurfaces in a smooth fashion.When root resorption is associated with malignanttumors, the resorption is usually in smallerquantities causing thinning of the root into a"spiked" shape.10
  11. 11. 11
  12. 12. 12
  13. 13. Benign tumorsThe benign neoplasias are separated into twomajorgroups: odontogenic tumors and non-odontogenictumors.ODONTOGENICTUMORSOdontogenic tumors arise from the tissues of theodontogenic apparatusAccording to (WHO),13
  14. 14. 1). Odontogenic epithelium(i). Ameloblastoma(ii). Squamous odontogenic tumor(iii).Calcifying epithelialodontogenic tumor (Pindborg’s tumor)(iv).Clear cell odontogenic tumor2). Odontogenic epithelium with odontogenicectomesenchyme(i). Ameloblastic fibroma(ii). Ameloblastic fibro dentinoma andameloblastic fibro odontoma(iii). Odontoameloblastoma(iv). Adenomatoid OdontogenicTumor(v). Calclifying odontogenic cyst(vi). Complex odontoma(vii). Compound odontoma14
  15. 15. 3). Odontogenic ectomesenchyme(i). Odontogenic fibroma(ii). Myxoma / Odontogenic myxofibroma(iii).Benign cementoblastoma( True Cementoblastoma)15
  16. 16. Odontogenicepithelial tumors16
  17. 17. AMELOBLASTOMASynonymsAdamantinoma , adamantoblastoma, andepithelial odontomaDefinitionA true neoplasm of enamel organ type whichdoesn’t undergo differentiation to the point ofenamel formation.robinson Defined it asA tumor that is -Unicentric,non functional,intermittent in growth,anatomically benignand clinically persistent17
  18. 18. 18
  19. 19. Clinical featuresAge - 20 to 40yrsSite - mandible > maxillaslow growing, painless, bony expansioninitially Tennis ball like consistency“Egg shell” like crackingJaw bone enlargement & parasthesia19
  20. 20. Radiographic FeaturesLocation. Most (80%) develop in themolar-ramus region of the mandible, but theymayextend to the symphyseal area.Periphery. is usually well definedand frequently delineated by a cortical border.Theborder is often curved and in small lesions theborderand shape may be indistinguishable from a cystThe periphery of lesions in the maxilla is usually-more ill defined.20
  21. 21. 21
  22. 22. Internal structure. The internal structure variesfrom totally radiolucent to mixed with thepresence of bony septa creating internalcompartments.These septa are usually coarse and curved andoriginate from normal bone that has beentrappedwithin the tumor.Because this tumor frequently has internal cysticcomponents, these septa are often remodeledinto curved shapes providing a honeycomb(numerous small compartments or loculations) orsoap bubble (larger compartments of variablesize)patterns .Generally the loculations are larger in the 22
  23. 23. 23
  24. 24. Egg shell24
  25. 25. Effects on surrounding structures. There ispronounced tendency for ameloblastomas to causeextensive root resorption . Tooth displacement iscommon.Because a common point of origin is occlusalto a tooth, some teeth may be displaced apically.An occlusal radiograph may demonstrate cyst likeexpan-sion and thinning of an adjacent cortical plate,leavinga thin "egg-shell" of bone .Actual perforation of bone into the surroundingsoft tissues or anatomic spaces is a late feature ofameloblastomaUnicystic types of ameloblastoma may causeextreme expansion of the mandibular ramus25
  26. 26. Differential DiagnosisThe odontogenic keratocyst may contain curvedsepta but usually the keratocyst tends to growalong the bone without marked expansion,which is Characteristic of ameloblastomas.26
  27. 27. Giant cell granulomas generally occur anterior tothe molars, occur in a younger age group, andhave more granular and ill defined septa27
  28. 28. Odontogenic myxomas may have similarappearing septa; however , there are usually one ortwo thin, sharp, straight septa, characteristicof the myxoma.Even the presence of one such septum mayindicate a myxoma. Also myxomas are not asexpansile as ameloblastomas and tend to growalong the bone.28
  29. 29. The septa in ossifying fibroma are usuallywide, granular, and ill defined. Also, look forthe presence of small irregular trabeculae.29
  30. 30. TreatmentThe most common treatment is surgical resection. Ifthe ameloblastoma is relatively small , it may beremovedcompletely by an intraoral approach and largerlesionsMay require resection of the jaw.The maxilla is usually treated more aggressivelybecause of the tendency of ameloblastoma toinvade adjacent vital structures.Radiation therapy may be used for inoperabletumors,Especially those in the posterior maxilla.30
  31. 31. CALCIFYING EPITHELIUM ODONTOGENICTUMOR( Pindborg’s tumor )Definition:It is a locally aggressive tumor consist of sheets &strands of polyhedral cells in fibrous stroma withno inflammatory component & are oftenaccompanied by spherical calcifications & amyloidstaining hyaline deposits.Origin -Rest of dental lamina-Reduced enamel epithelium1% of all odontogenic tumor31
  32. 32. Clinical featuresCEOTCentral Peripheral(intraosseous) (extraosseous)age - 40yrs site - anterior gingivasite - 2/3rd of appears as superficiallesions in mandible soft tissue swellingslow growing. of gingiva in a toothpainless mass. bearing area oredentulous areaof jaw32
  33. 33. Radiographic features:Location. mandiblemost develop in the premolar-molar area, witha52% association with an unerupted or impactedtooth.Periphery. The border may have a well-definedcyst likecortex. In some tumors the boundary maybeirregularand ill definedInternal structure. The internal aspect mayappear unilocular or multilocular with numerousscattered,radiopaque foci of varying size and density. 33
  34. 34. 34
  35. 35. most characteristic and diagnostic finding is theappearance of radiopacities close to the crown of theembedded tooth35
  36. 36. 36
  37. 37. Effects on surrounding structures. may displacea developing tooth or prevent its eruption.Associated expansion of the jaw with maintenanceof a cortical boundary may also occur.Differential DiagnosisLesions with completely radiolucent internalstructuremay mimic dentigerous cysts or evenameloblastomasOther lesions with radiopaque foci, includingade-nomatoid odontogenic tumor, ameloblasticfibro-odontoma, and calcifying odontogenic cyst , mayhave 37
  38. 38. TreatmentThe treatment of the CEOT is more conservativethanthe ameloblastoma , with local resection38
  39. 39. MIXED TUMORS(OF ODONTOGENICEPITHELIUM ANDODONTOGENICECTOMESENCHYME)39
  40. 40. ODONTOMAMost common type of odontogenic tumorDefinition:a tumor that is radiographically and histologicallycharacterized by the production of mature enamel,dentin, cementum,and pulp tissue.Clinical features:Age- 10 to 20yrsSite - Maxilla > mandibleSlow growing , hard , painless mass40
  41. 41. GARDNER’S Syndrome is associated with it(a). Multiple odontomas(b). Multiple osteomas(c ). Intestinal polyps(d). Epidermoid cyst(e). Dermoid tumor(fibrous)2 Types(1). Complex(2). Compound41
  42. 42. Features Compound complexlocation anterior maxilla inassociation with thecrown of an uneruptedcanine.mandibular first andsecond molar area.Internal structure number of toothlikestructures or denticlesthat look like deformedteethirregular mass ofcalcified tissueFrequency More Less commonSex predilection M=F More in female42
  43. 43. Radiographic FeaturesLocation.compound odontomas (62%) occur in the anteriormaxilla in association with the crown of anunerupted canine.70%of complex odontomas are found in themandibular first and second molar area.Periphery. The borders of odontomas arewell defined and may be smooth or irregular.These lesions have a cortical border andimmediately inside and adjacent to the corticalborder is a soft tissue capsule.43
  44. 44. Internal structure. The contents of theselesions are largely radiopaque.Compound odontomas have a number oftoothlike structures or denticles that look likedeformed teethComplex odontomas contain an irregular mass ofcalcified tissueEffects on surrounding structures. Odontomas,can interfere with the normal eruption ofteeth. Most odontomas (70%) are associatedwith abnormalities such as impaction,malpositioning, diastema, aplasia, malformation,and devitalization of adjacent teeth.Large complex odontomas may cause expansion ofthe44
  45. 45. Complex odontoma45
  46. 46. Compound odontoma46
  47. 47. treatmentComplex and compound odontomas are usuallyRemoved by simple excision. They do not recur and arenot locally invasive47
  48. 48. AMELOBLASTIC FIBROMASynonymsSoft odontoma, soft mixed odontoma, mixedodonto genic tumor, fibroadamantoblastoma,andgranular cellAmeloblasticfibromadefinationAmeloblastic fibromas are benign, mixedodontogenictumors. They are characterized by neoplasticprolifera-tion of maturing and early functionalameloblasts,aswell as the primitive mesenchymal components ofthe 48
  49. 49. Clinical features:Age- 5 to 20yrsSite - Maxilla > mandibleSex- no sex predilectionThey usually produce a painless,slow-growingexpan-sion, and displacement of the involved teethRadiographic FeaturesLocation. in the premolar-molar area of themandiblePeriphery. The borders of an ameloblastic-fibromaarewell defined and often corticated in a mannersimilar49
  50. 50. 50
  51. 51. 51
  52. 52. 52
  53. 53. Internal structure. An ameloblastic fibroma ismore commonly unilocular (totally radiolucent)Effects on surrounding structures. If the lesionis large,there may be expansion with an intact corticalplate.The associated tooth or teeth may be inhibitedfromnormal eruption or may be displaced in anapicaldirection.53
  54. 54. Differential DiagnosisAmeloblastoma—however ,the ameloblasticfibroma occurs at an earlier age and the septa inan ameloblastoma are more defined and coarse.In fact the septa in ameloblastic fibroma areinfrequent and often very fine.Giant cell granulomas --may appear multilocular,but these tumors usually have an epicenter anteriorto the first molar and the septa arecharacteristically granular and ill defined.Odontogenic myxomas-- can appear multilocu-lar but usually a few sharp straight septa can beidenti-fied, which are not characteristic ofameloblasticfibromas and myxomas usually occur in an olderage 54
  55. 55. Treatment. Ameloblastic fibromas are benign, andtherate of recurrence is low. A conservativesurgicalapproach,including enucleation and mechanicalcurettage of the surrounding bone,55
  56. 56. AMELOBLASTIC FIBRO-ODONTOMADefinitionAn ameloblastic fibro-odontoma is a mixed tumorwithall the elements of an ameloblastic fibroma butwithScattered collections of enamel and dentineClinical Features.The clinical features aresimilarto odontomas, often associated with a missingtoothor tooth that has failed to erupt.This tumor appears during the same age asodontomas and ameloblastic fibromas with noparticular sex predilection. 56
  57. 57. Radiographic FeaturesLocation-posterior aspect of the mandible. Theepicenter of the lesion is usually occlusal to adeveloping tooth or toward the alveolar crest.Periphery. This tumor is usually well defined andsome-times corticated.Internal structure. The internal structure ismixed, with the majority of the lesion beingradiolucent. Small lesions may appear as enlargedfollicles with only one or two small,discreteradiopacities. Larger lesions may have a moreextensive calcified internal structure57
  58. 58. Differential DiagnosisDifferentiation from a developing odontoma maybe difficult, but generally these tumors have agreater soft tissue component (radiolucent) thanan odontomaA complex odontoma, which shares acommon location, usually has one mass ofdisorganizedtissue in the center, whereas the ameloblasticfibro-odontoma will usually have multiple scatteredmaturesmall pieces of dental hard tissueTreatmentUsually conservative enucleation is used 58
  59. 59. 59
  60. 60. ADENOMATOID ODONTOGENIC TUMORSynonyms ,Adenoameloblastoma and ameloblastic adenomatoidtumorDefinitionAdenomatoid odontogenic tumors are uncommon,Nonaggressive tumors of odontogenic epitheliumClinical featuresAge - younger patient (10 to 19yrs).Sex - femaleSite - anterior portion of the jawmaxilla > mandibleAsymptomatic, painless, slow growing.large lesions causes expansion of bone.60
  61. 61. Both central and peripheral tumors occur. Thecentral tumors are divided into the folliculartype (those associated with the crown of anembedded tooth) and the extra- follicular type(those with no embedded tooth).Radiographic FeaturesLocation. At least 75% occur in the maxillaThe incisor canine-premolar region, especially thecanine regionPeriphery. The usual radiographic appearance is awell-defined corticated or sclerotic border.Internal structure. Radiographically,radiopacities develop in about two thirds of cases61
  62. 62. Effects on surrounding structures. As thetumor enlarges, adjacent teeth are displaced.Root resorption is rare. This lesion also may inhibiteruption of an involved tooth. Although someexpansion of the jaw may occur, the outer cortex ismaintained.Differential DiagnosisIf the attachment of the radiolucent lesion ismore apical than the cementoenamel junction,a follicular cyst can be discounted.The ameloblastic fibro-odontoma and thecalcifying epithelial odontogenic tumor occur morecommonly in the posterior mandible.62
  63. 63. Pebble –like calcification63
  64. 64. TreatmentConservative surgical excision is adequate becausethetumor is not locally invasive,is wellencapsulated,andis separated easily from the bone64
  65. 65. MESENCHYMAL TUMORS(ODONTOGENIC ECTOMESENCHYME)65
  66. 66. ODONTOGENIC MYXOMASynonymsMyxoma, myxofibroma, and fibromyxomaDefinitionOdontogenic myxomas are uncommon,accountingfor only 3% to 6% of odontogenic tumors. Theyare benign, intraosseous neoplasms that arise fromodon-togenic ectomesenchyme and resemble themesenchy-mal portion of the dental papilla66
  67. 67. Clinical featuresAge - 10 to 30yrs.Sex – femaleThe tumor grows slowly and may or may notcause pain.Eventually it causes swelling and May growquite large if left untreated67
  68. 68. Radiographic FeaturesLocation- mandible , the premolar and molar areasPeriphery--The lesion usually is well defined, and itmayhave a corticated margin but most often ispoorlydefined, especially in the maxillaInternal structure. majority have a mixedradiolucent-radiopaque internal pattern.Residual bone trapped within the tumor willremodelinto curved and straight, course or fine septa.Thepres-ence of these septa gives the tumor amultilocular appearance. 68
  69. 69. 69
  70. 70. 70
  71. 71. The majority of the septa are curved and course,but the finding of one or two of these straightsepta will help in the identification of this tumorEffects on surrounding structures. Whengrowing in a tooth-bearing area, it displaces andloosens teeth,but rarely causes resorption of teeth. The lesional sofrequently scallops between the roots. ofadjacent teeth similar to a simple bone cyst. Thistumor has a tendency to grow along the involvedbone without the same amount of expansion seenwith other benign tumors.71
  72. 72. Differential DiagnosisInclude ameloblastomas, central giant cellgranulomas, and central hemangiomas.The finding of characteristic thin, straight septawith less-than-expected bone expansion is veryuseful in the differential.Careful inspection of this area of expansion willreveal a thin but intact outer cortex that would notbe seen in osteogenic sarcomaTreatmentby resection with a generous amount ofsurrounding bone to ensure removal ofmyxomatous tumor that infiltrates the adjacentmarrow spaces.72
  73. 73. BENIGN CEMENTOBLASTOMASynonymsCementoblastoma and true cementomaDefinitionBenign cementoblastomas are slow-growing ,mesenchymal neoplasms composed principally ofcementumClinical FeaturesAge - 12 to 65Sex - femalesThe tumor usually is a solitary lesion that isslow-growing but that may eventually displaceteeth.The involved tooth is vital and often painful.The pain seems to vary from patient to patientand can be relieved by anti-inflammatory drugs.73
  74. 74. Radiographic FeaturesLocation. mandible (78%) -premolar or firstmolar(90%).Periphery. The lesion is a well-defined radiopacitywitha cortical border and then a well-definedradiolucentband just inside the cortical borderInternal structure. mixed radiolucent-radiopaque lesions in which the majority of theinternal structure is radiopaque.wheel spoke patternThe density of the cemental mass usuallyobscures the outline of the enveloped root.This central radiopaque mass as mentioned issurrounded by a radiolucent band indicating that thetumor is maturing from the central aspect to 74
  75. 75. 75
  76. 76. Effects on surrounding structures.If large enough, this tumor can cause expansion ofthe mandible but with an intact outer cortex.Differential Diagnosisthe radiolucent band around the benigncementoblastoma is usually better definedand more uniform than with cemental dysplasia.Also, in the first molar region thecementoblastoma has a more rounded shapethan cemental dysplasia.TreatmentBenign cementoblastomas are apparently self-limitingand rarely recur after enucleation. Simpleexcisionand extraction of the associated tooth are 76
  77. 77. CENTRAL ODONTOGENIC FIBROMASynonymsSimple odontogenic fibroma and odontogenicfibroma(World Health Organization [WHO] type)DefinitionCentral odontogenic fibromas are rare neoplasmsthatSometimes are divided into two types according tohis-tologic appearance:simple type contains mature fibrous tissue withsparsely scattered odontogenic epithelial rests;WHO type, which is more cellular,77
  78. 78. Clinical FeaturesAge - 11 and 39 yearsSex – femalespatients may be asymptomatic or may have swellingand mobility of the teeth.Radiographic FeaturesLocation. mandible, molar-premolar region.They are also prevalent in the maxilla anterior to thefirst molar.Periphery. The periphery usually is well defined,Internal structure. Smaller lesions usually are unilocu-lar, and larger lesions have a multilocular pattern. Theinternal septa may be fine and straight, as in odonto-genic myxomas,or it maybe granular, resembling thoseSeen in giant cell granulomas. 78
  79. 79. Effects on surrounding structures. A centralodontogenic fibroma may cause expansion withmaintenance of a thin cortical boundary or onoccasion can grow along the bone with minimumexpansion similar to an odontogenic myxoma.Tooth displacement is common, and root resorptionhas been reported.Differential DiagnosisDesmoplastic fibromas are more aggressive andtend to break through the peripheral cortex andinvade surrounding soft tissue79
  80. 80. NONODONTOGENICTUMORS80
  81. 81. BENIGN TUMORS OF NEURAL ORIGINNEURILEMOMASynonymSchwannomaDefinitionA central neurilemoma is a tumor of neuro-ectodermalorigin, arising from the Schwann cells that make uptheinner layer covering the peripheral nerves.Clinical FeaturesAge – second and third decadesSex – equal frequency81
  82. 82. The usual complaint is a swelling.Pain, when present, usually develops at theSite of the tumor; if paresthesia occurs,it is feltanteriorto the tumor.Radiographic FeaturesLocation. mandible, most often located withinan expanded inferior alveolar nerve canal posteriorto the mental foramenPeriphery. In keeping with its slow growth rate,themargins of this tumor are well defined and usuallycor-ticated as it expands the cortical walls of theinferior 82
  83. 83. 83
  84. 84. Internal structure. The internal structure isuniformlyradiolucent. When lesions have a scallopingoutline,this may give a false impression of amultilocularpattern.Effects on surrounding structures. If the tumorreaches either the mandibular foramen or mentalforamen, it can cause enlargement of the foramen.Expansion of the inferior alveolar canal is slowand thus the outer cortex of the canal ismaintained and the expansion of the canal is usuallylocalized with a definite epicenter unless the lesionis large.84
  85. 85. TreatmentExcision is usually the treatment ofchoice85
  86. 86. NEUROFIBROMASynonymNeurinomaDefinitionNeurofibromas are moderately firm, benign,well-circumscribed tumors caused by proliferationofSchwann cells in a disorderly pattern thatincludesportions of nerve fibers, such as peripheralnerves,Axons ,and connective tissue of the sheath ofSchwann.As neurofibromas grow, they incorporate axons.86
  87. 87. Clinical FeaturesThe central lesion of a neurofibroma maybe thesameas the multiple lesions that develop in vonReckling-hausens disease.Neurofibromas can occur at any age but usually arefound in young patients.Neurofibromas associated with the mandibularnerve may produce pain or paresthesia.Neurofibromas also may expand and perforate thecortex; causing swelling that is hard or firm topalpation.87
  88. 88. Radiographic FeaturesLocation. in the mandibular canal, in thecancellous bone, and below the periosteum.Periphery.. the margins of the radiolucency inneurofibromas usually are sharply defined andmay be corticated.However, despite the benign nature and slowgrowth of the neurofibroma, some of these lesionshave indistinct margins.Internal structure. The tumors usually appearunilo-cular but on occasion may have amultilocular appearance.Effects on surrounding structures. Aneurofibroma of the inferior dental nerve shows a 88
  89. 89. 89
  90. 90. Differential DiagnosisDifferentiation from other types of neural lesionsmaynot be possible.This tumor can be differentiated fromVascular lesions because the expansion of thecanal isin a fusiform shape,whereas vascular lesionsenlargethe whole canal and alter its path.TreatmentSolitary central lesions that have been excisedseldomrecur. However, it is wise to re-examine the areaperi- 90
  91. 91. NEUROFIBROMATOSISSynonymvon Reckling-hausensdiseaseDefinitionNeurofibromatosis is a syndrome consistingof cafe au-lait spots on the skin, multipleperipheral nerve tumors, and a variety ofother dysplastic abnormalities of the skin,nervous system, bones, endocrine organs,and blood vesselsThe two major classifications areNF-1, a generalized form,NF-2, a central form.Oral lesion may occur as part of NF-1 ormay be solitary and are called segmental orforme fruste manifestations91
  92. 92. Clinical FeaturesNeurofibromatosis is one of the most commongeneticDiseases.The peripheral nerve tumors are of two types,schwannomas and neurofibromas. Mostmanifestations are appear gradually duringchildhood & adult life. Cafe-au-Iait spots becomelarger and more numer-ous with age; most patients eventually havemore than six spots larger than 1.5 cm indiameter.92
  93. 93. Radiographic featuresThe radiographic changes in the jaws withneurofibroatosis can be characteristic. Thesechanges include1. enlargement of the coronoid notch ineither or both the horizontal and verticaldimensions2. an obtuse angle between the body andthe ramus3. deformity of the condylar head4. lengthening of the condylar neck5. lateral bowing and thinning of the ramus6. enlargement of the mandibular canal &mental and mandibular foramina and anincreased incidence of branched mandibular93
  94. 94. 94
  95. 95. TreatmentMost patients live a normal life with Jew or nosymp toms. Small cutaneous and subcutaneousneurofibro-mas can be removed if they are painful, butlargeplexiform neurofibromas should be left alone.Malignant conversion of these lesions has occurredin rare cases.95
  96. 96. MESODERMAL TUMORSOSTEOMADefinitionOsteomas can form from membranous bones of theSkull and face.The cause of the slowly growing osteoma is obscure, butthe tumor may arise from cartilage or embryonalperiosteumIt is not clearwhether osteomas are benign neoplasms orhamartomasStructurally, osteomas can be divided into three-types: composed of compact bone (ivory),composed of cancellous bone,composed of a combination of compact and cancellousbone. 96
  97. 97. 97
  98. 98. 98
  99. 99. Clinical FeaturesAge – above 40 yearsSex – Cortical type = mencancellous type= womenRadiographic FeaturesLocation. mandible -posterior aspect -lingual side ofthe ramus or on the inferior mandibular borderbelow the molarsInternal structure. Osteomas composed solely ofcompact bone are uniformly radiopaque; those con-taining cancellous bone show evidence of internal tra-becular structure.Effects on surrounding structures. Large lesions candis-place adjacent soft tissues , such as muscles , andcausedysfunction. 99
  100. 100. TreatmentUnless the osteoma interferes with normal functionorpresents a cosmetic problem, this lesion maynotrequire treatment. In such casesthe osteoma shouldbekept under observation.Resection of osteoma sis possible and maybedifficult if the osteoma is of the cortical (ivory) type.100
  101. 101. CENTRAL HEMANGIOMADefinitionA hemangioma is a proliferation of blood vesselscreating a mass that resembles a neoplasm , although inmany cases it is actually a hamartomaClinical FeaturesAge – first decadeSex – femaleEnlargement is slow , producing a non-tenderexpansion of the jaw that occurs over severalmonths or years.The swelling may or may not be painful, is nottender, and usually is bony hard.Pain, if present, usually of throbbing type101
  102. 102. Some tumors may be compressible or pulsate, anda bruit may be detected on auscultationAnesthesia of the skin supplied by the mentalnerveMay occur.The lesion may cause loosening and migration ofteeth in the affected area.Bleeding may occur from the gingiva around theneck of the affected teeth.These teeth may demonstrate rebound mobility;thatis,when depressed into their sockets,they reboundtotheir original position within several minutesbecauseof the pressure of the vascular network aroundthe 102
  103. 103. Hemangioma are associated with followingSyndrome—Syndrome featuresRendu-osler-weber-syndromemultiple telangi-ectasias ,occasional GI tract &CNS involvementSturge-weber-dimtri syndromePort-wine stain, leptomeningeal angiomasKasabach –merritt syndromeThrombocytopenic purpura associated withhemangiomas , consumptive coagulpathy ,microangiopathic hemolysis , intralesionalfibrinolysisMaffuccisyndromeHemangiomas of mucous membrane ,dyschondroplasiaVon hippel-lindau syndromeHemangiomas of cerebellum , or retina, cyst ofvisceraKlippel –trenaunay webersyndromePort-wine stain & angiomas of extremities103
  104. 104. Radiographic FeaturesLocotion. Mandible , posterior body and ramusand within the inferior alveolar canal.Periphery. In some instances the periphery iswelldefined and corticated, and in other cases it maybeilldefined and even simulate the appearance of amalig-nant tumorThe formation of linear spicules of bone ema-nating from the surface of the bone in a sunray-likeappearance can occur when the hemangiomabreaks 104
  105. 105. 105
  106. 106. Internal structure. When there is residualbonetrapped around the blood vessels ,the resultmaybe amultilocular appearance.Small radiolucent locules may resemble enlargedmarrow spaces surrounded by coarse, dense,and well-defined trabeculae .These internal trabeculae may produce ahoneycomb pattern composed of small circularradiolucent spacesthat represent blood vessels oriented in the samedirec-tion of the x-ray beam.106
  107. 107. When the inferior alveolar canalis involved, the whole canal is increased in widthandoften the normal path of the canal is altered intoaserpiginous shapeSome lesions may be totally radiolucent.When the hemangioma involves soft tissue theformation of phleboliths (small areas ofcalcification or concretions found in a vein withslow bloodflow) may occur within surrounding soft tissuesThey develop from thrombi that becomeorganized and mineralized and consist of calciumphosphate 107
  108. 108. 108
  109. 109. Effects on surrounding structures. The roots ofteeth in the region of the vascular lesion often areresorbed or displaced.When the lesion involves the inferior alveolarnerve canal, the canal can be enlarged along itsentire length and its shape may be changed to aserpiginous path. The mandibular and mentalforamen may be enlarged. Hemangiomas caninfluence the growth of bone and teeth.The involved bone may be enlarged and havecoarse, internal trabeculae.Also, developing teeth may be larger and eruptearlier when in an intimate relationship with ahemangioma109
  110. 110. 110
  111. 111. Differential DiagnosisHemangiomas should be considered in thedifferentialDiagnosis of multilocular lesions involving thebody ofthe ramus and body of the mandible.Demonstrationof involvement of the inferior alveolar canal isanimportant indicator of a vascular lesion.Treatment should be treated without delay,because trauma that disrupts the integrity oftheAffected jaw may result in lethal exsanguination.Specifi-cally, embolization (introduction of inert 111
  112. 112. ARTERIOVENOUS FISTULASynonymsA-V defect, A-V shunt, A-V aneurysm, andA-VmalformationDefinitionAn arteriovenous (A-V) fistula, an uncommon lesion, isa direct communication between an artery and aveinthat bypasses the intervening capillary bed.It usually results from trauma .The head and neck are the most commonsites.112
  113. 113. Clinical Featuresvary considerably, depending on the extent ofbone or soft tissue involvement.The lesion may expand bone, and a mass may bepresent in the Extraosseous soft tissue.The soft tissue swelling may have a purplediscoloration. Palpation or auscultation of theswelling may reveal a pulse.On the other hand,neither the bone nor the soft tissue maybeexpanded,and no pulse maybe clinically apparent.Aspiration produces blood.Recognition of the hemorrhagic nature of theselesions is of utmost importance, because extractionof an associated tooth may be immediatelyfollowed by life-threatening bleeding. 113
  114. 114. Radiographic FeaturesLocation.- ramus and retromolar area of the mandibleand involve the mandibular canal.Periphery.The margins usually are well defined andcorticated.Internal structure. A tortuous path of an enlargedvessel in bone may give a multilocular appearance.Otherwise the lesion is radiolucent.Effects on surrounding structures. Both centrallesionsand those in adjacent soft tissue can erode bone, result-ing in well-defined (cystlike) lesions in the bone.Changes in the inferior alveolar canal may occur, asdescribed in hemangiomas.114
  115. 115. 115
  116. 116. TreatmentAn A-V aneurysmis treated surgically.116
  117. 117. REFERENCES1. SHAFER’S ORAL PATHOLOGY2. NEVILLE ORAL PATHOLOGY3. ORAL radiology – white & pharoah4. burket’s ORAL medicine117
  118. 118. 118

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