Agressive jaw tumours in children.powerpoint.ppt

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1. AGGRESSIVE JAW TUMORS AGGRESSIVE JAW TUMORS
IN CHILDRENIN CHILDREN
PRESENTER : PRESENTER :
DR. AKSHAY SHETTY DR. AKSHAY SHETTY
DEPT OF ORAL&MAXILLOFACIAL SURGERYDEPT OF ORAL&MAXILLOFACIAL SURGERY

2. AGGRESSIVE LESIONSAGGRESSIVE LESIONS
•KABAN IN HIS PAPER ON “AGGRESSIVE KABAN IN HIS PAPER ON “AGGRESSIVE
JAW TUMORS IN CHILDREN” LISTED JAW TUMORS IN CHILDREN” LISTED
TUMORS WITH HIGH PREDILECTION TUMORS WITH HIGH PREDILECTION
FOR CHILDREN AND TUMORS MORE FOR CHILDREN AND TUMORS MORE
COMMON IN ADULT AGE GROUPS,BUT COMMON IN ADULT AGE GROUPS,BUT
WHICH COULD OCCUR IN CHILDRENWHICH COULD OCCUR IN CHILDREN
(OMFSCNA,MAY93)(OMFSCNA,MAY93)

3. ““AGGRESSIVE LESIONS”AGGRESSIVE LESIONS”

4. AGGRESSIVE JAW TUMORS IN AGGRESSIVE JAW TUMORS IN
CHILDRENCHILDREN
•CENTRAL GIANT CELL LESIONSCENTRAL GIANT CELL LESIONS
•HEMATOPOIETIC AND R.E. TUMORSHEMATOPOIETIC AND R.E. TUMORS
•MALIGNANT MESENCHYMAL TUMORSMALIGNANT MESENCHYMAL TUMORS
•FIBRO-OSSEOUS LESIONSFIBRO-OSSEOUS LESIONS
•NEUROGENIC TUMORSNEUROGENIC TUMORS

5. CENTRAL GIANT CELL LESIONSCENTRAL GIANT CELL LESIONS
•CENTRAL GIANT CELL GRANULOMACENTRAL GIANT CELL GRANULOMA
•HYPERPARATHYROIDISMHYPERPARATHYROIDISM

6. Central giant cell granulomaCentral giant cell granuloma
•Benign lesion exclusive to jaw boneBenign lesion exclusive to jaw bone
•Jaffe termed it giant cell reparative granulomaJaffe termed it giant cell reparative granuloma
•Anterior to first molar, mandible more frequentlyAnterior to first molar, mandible more frequently
•Children and young adultsChildren and young adults
•Females more than malesFemales more than males
•Swelling accompanying pain and tenderness on Swelling accompanying pain and tenderness on
palpationpalpation
•Mandible:Maxilla-3:1Mandible:Maxilla-3:1

7. Central giant cell granulomaCentral giant cell granuloma

Aggressive CGCG: Aggressive CGCG:
•Rapid growth. Rapid growth.
•High recurrent rate. High recurrent rate.
•Common root resorption & cortical perforation. Common root resorption & cortical perforation.
•Likelihood of being painful & paresthesia.Likelihood of being painful & paresthesia.

8. Central giant cell granulomaCentral giant cell granuloma
•ReparativeReparative
•NeoplasmicNeoplasmic
•Developmental AnomalyDevelopmental Anomaly

9. Central giant cell granulomaCentral giant cell granuloma
HistopathologyHistopathology
•Proliferating endothelial Proliferating endothelial
cells. cells.
•Multinucleated giant Multinucleated giant
cells. (char. of osteoclast) cells. (char. of osteoclast)
•Mitotic figures of Mitotic figures of
mononuclear cells. mononuclear cells.
•Active fibroblast & Active fibroblast &
myofibroblast. myofibroblast.
•Extravasated RBC. Extravasated RBC.

10. •RADIOLUCENCY:INDISTIRADIOLUCENCY:INDISTI
NCT LINE OF NCT LINE OF
DEMARCATIONDEMARCATION
•COMPLETE CORTICAL COMPLETE CORTICAL
BONE LOSSBONE LOSS
•RESORPTION OF ROOTSRESORPTION OF ROOTS
•BUCCAL AND LINGUAL BUCCAL AND LINGUAL
EXPANSIONEXPANSION
•UNILOCULAR/UNILOCULAR/
MULTILOCULARMULTILOCULAR

11. Central giant cell granulomaCentral giant cell granuloma

12. Central giant cell granulomaCentral giant cell granuloma
SURGICALSURGICAL
•CurettageCurettage
•Block resectionBlock resection
NON-SURGICALNON-SURGICAL
•CalcitoninCalcitonin
•CorticosteroidsCorticosteroids
•Interferon alphaInterferon alpha

13. Central giant cell granulomaCentral giant cell granuloma
• Developed by Harris at 1993. Developed by Harris at 1993.
• Finding of unidentified, circulating Finding of unidentified, circulating
PTH-like hormone. PTH-like hormone.
•Regimen: Salmon calcitonin Regimen: Salmon calcitonin
• Nasal spray, 200 units per day (1.0 Nasal spray, 200 units per day (1.0
mg) mg)
• Arrest the growth of lesion, until Arrest the growth of lesion, until
they can heal spontaneously. they can heal spontaneously.

14. Central giant cell granulomaCentral giant cell granuloma
Intralesional glucocorticosteroids: Intralesional glucocorticosteroids:
•Regiment: 2 ml / 2 cm of radiolucency. Regiment: 2 ml / 2 cm of radiolucency.
administer weekly for least 6 weeks. administer weekly for least 6 weeks.
content: Triamcinilone acetonide, content: Triamcinilone acetonide,
Interferon alpha Interferon alpha
• multiple location injection. multiple location injection.
Useful for large lesion, because surgery can Useful for large lesion, because surgery can
cause severe esthetic & functional deficit in cause severe esthetic & functional deficit in
the patientthe patient

15. HEMATOPOIETIC/ HEMATOPOIETIC/
RETICULOENDOTHELIAL TUMORSRETICULOENDOTHELIAL TUMORS
•HISTIOCYTOSIS X:HISTIOCYTOSIS X:
EOSINOPHILIC GRANULOMAEOSINOPHILIC GRANULOMA
HAND-SCHULLER-CHRISTIAN DISEASEHAND-SCHULLER-CHRISTIAN DISEASE
LETTERER-SIWE DISEASELETTERER-SIWE DISEASE
•BURKITT’S LYMPHOMABURKITT’S LYMPHOMA

16. BURKITT’S LYMPHOMABURKITT’S LYMPHOMA
•A malignant proliferation of B-A malignant proliferation of B-
lymphocytes lymphocytes
•Believed to be caused by the Epstein-Believed to be caused by the Epstein-
Barr virus Barr virus
•Two types: African (endemic) and Two types: African (endemic) and
American (non-endemic)American (non-endemic)

17. BURKITT’S LYMPHOMABURKITT’S LYMPHOMA
African Burkitt’s LymphomaAfrican Burkitt’s Lymphoma
•Large Jaw tumor with or without orbital Large Jaw tumor with or without orbital
involvement involvement
•Occurs in children during the first Occurs in children during the first
decade of life decade of life
•2:1 male to female ratio 2:1 male to female ratio
•Affected jaws is pathognomonic for Affected jaws is pathognomonic for
African Burkittís LymphomaAfrican Burkittís Lymphoma

18. BURKITT’S LYMPHOMABURKITT’S LYMPHOMA
American Burkitt’s LymphomaAmerican Burkitt’s Lymphoma
•Tumors most often in the abdomen Tumors most often in the abdomen
•Non-endemic regions in older Non-endemic regions in older
children and young adults children and young adults
•Jaw tumors only occur in 7-18% of Jaw tumors only occur in 7-18% of
casescases

19. BURKITT’S LYMPHOMABURKITT’S LYMPHOMA
Clinical ManifestationsClinical Manifestations
•Firm tumor, may Firm tumor, may
become ulcerated with become ulcerated with
trauma trauma
•Often associated with Often associated with
buccal or lingual buccal or lingual
cortical expansion cortical expansion
•Painless, typically Painless, typically
without sensory deficitswithout sensory deficits

20. BURKITT’S LYMPHOMABURKITT’S LYMPHOMA
Radiographic findingsRadiographic findings
•Massive lesions undergoing osseous Massive lesions undergoing osseous
resorption resorption
•Irregular margins Irregular margins
•Tooth displacement and root Tooth displacement and root
resorption are commonresorption are common

21. BURKITT’S LYMPHOMABURKITT’S LYMPHOMA
Histological findingsHistological findings
•Medium sized round Medium sized round
lymphoblasts lymphoblasts
•Lacunae with macrophages Lacunae with macrophages
containing phagocytized containing phagocytized
cellular debrisÖThis is cellular debrisÖThis is
pathognomonic for African pathognomonic for African
type type
•Lacunae resemble a Starry Sky Lacunae resemble a Starry Sky
•Tumors may invade PDL and Tumors may invade PDL and
can invade the pulp of can invade the pulp of
developing teethdeveloping teeth

22. BURKITT’S LYMPHOMABURKITT’S LYMPHOMA
TreatmentTreatment
•Radiation: 2000c Gy total radiation Radiation: 2000c Gy total radiation
•CHEMOTHEREPY for 4 months CHEMOTHEREPY for 4 months
–Cyclophosphamide , Ifosfamide, Cyclophosphamide , Ifosfamide,
Prednisone, Dexamethazone, Vincristine, Prednisone, Dexamethazone, Vincristine,
Methotrexate, ARA-C VM-26, AdriamycinMethotrexate, ARA-C VM-26, Adriamycin

23. EOSINOPHILIC GRANULOMAEOSINOPHILIC GRANULOMA
•Eosinophilic Granuloma (EG) Eosinophilic Granuloma (EG)
represents the most benign and represents the most benign and
localized form of the three localized form of the three
Langerhans cell histiocytosis Langerhans cell histiocytosis
syndromes. In bone, it is a tumor-like syndromes. In bone, it is a tumor-like
proliferation of Langerhans cells.proliferation of Langerhans cells.

24. EOSINOPHILIC GRANULOMAEOSINOPHILIC GRANULOMA
•1940- Lichtenstein and Jaffe coin the term 1940- Lichtenstein and Jaffe coin the term
eosinophilic granuloma eosinophilic granuloma
•1953 - Lichtenstein proposes that EG, Hand-1953 - Lichtenstein proposes that EG, Hand-
Schuller-Christian syndrome and Letterer-Schuller-Christian syndrome and Letterer-
Siwe syndrome should be included in Siwe syndrome should be included in
Histiocytosis X Histiocytosis X
•1987 -the Histiocyte Society introduces 1987 -the Histiocyte Society introduces
Langerhans cell histiocytosis as the new Langerhans cell histiocytosis as the new
name for Histiocytosis Xname for Histiocytosis X

25. EOSINOPHILIC GRANULOMAEOSINOPHILIC GRANULOMA
•Intraosseous EG lesions can be solitary Intraosseous EG lesions can be solitary
or multifocal or multifocal
•EG accounts for 60-70% of LCH cases EG accounts for 60-70% of LCH cases
•All bones can be affected except the All bones can be affected except the
hands and feet hands and feet
•10-20% of cases occur in the jaws: two-10-20% of cases occur in the jaws: two-
thirds of these in the mandible thirds of these in the mandible

26. EOSINOPHILIC GRANULOMAEOSINOPHILIC GRANULOMA
•The age of patients with EG varies, but The age of patients with EG varies, but
usually occurs within first three usually occurs within first three
decades decades
•Ratio of men to women is 2:1 Ratio of men to women is 2:1
•EG does not require eosinophils or EG does not require eosinophils or
granulomas for diagnosis granulomas for diagnosis
•Etiology is unknown May be of Etiology is unknown May be of
infection, inflammation or infection, inflammation or
immunological response.immunological response.

27. EOSINOPHILIC GRANULOMAEOSINOPHILIC GRANULOMA
Other signs/symptoms: Other signs/symptoms:
•Persistent oral ulcer Persistent oral ulcer
•Gingivitis Gingivitis
•Loose teeth Loose teeth
•Foul breath and taste Foul breath and taste
•Mastication difficulty Mastication difficulty
•Headaches Headaches
•Sensory disturbancesSensory disturbances

28. EOSINOPHILIC GRANULOMAEOSINOPHILIC GRANULOMA
•NON-SPECIFIC-NOT NON-SPECIFIC-NOT
DIAGNOSTICDIAGNOSTIC
•RADIOLUCENT LYTIC RADIOLUCENT LYTIC
LESIONLESION
•TYPICALLY ROUND TYPICALLY ROUND
OR OVAL/MAY BE OR OVAL/MAY BE
WELL DEFINED OR WELL DEFINED OR
DIFFUSEDIFFUSE


  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  



  
  
  
  
  
  
  
  
  


  
  
  
  
  
  
  
  
  


  
  
  
  
  
  
  
  
  


  
  
  
  
  
  
  
  
  


  
  
  
  
  
  
  
  
  


  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  
  

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29. EOSINOPHILIC GRANULOMAEOSINOPHILIC GRANULOMA
•LANGERHANS CELL LANGERHANS CELL
DISPLAYING DISPLAYING
EOSINOPHILIC,GRAEOSINOPHILIC,GRA
NULAR CYTOPLASM NULAR CYTOPLASM
AND DISTINCT CELL AND DISTINCT CELL
MARGINSMARGINS
•EOSINOPHILS EOSINOPHILS
SEEN,BUT NOT SEEN,BUT NOT
DIAGNOSTICDIAGNOSTIC

30. EOSINOPHILIC GRANULOMAEOSINOPHILIC GRANULOMA
•Immunohistochemical staining and Immunohistochemical staining and
electron microscopy of EGelectron microscopy of EG
•Stains positive for S-100 protein Stains positive for S-100 protein
•Stains positive for CD1 antigen Stains positive for CD1 antigen
•Ultrastructural feature specific to Ultrastructural feature specific to
Langerhans cells - cytoplasmic inclusion Langerhans cells - cytoplasmic inclusion
bodies called Birbeck granules bodies called Birbeck granules

31. MANAGEMENTMANAGEMENT
Curettage/surgical resection
Systemic and/or local corticoids
Irradiation
Systemic chemotherapy
 Recurrence is low - 7.3%

32. HAND-SCHULLER-CHRISTIAN HAND-SCHULLER-CHRISTIAN
DISEASEDISEASE
•ALSO KNOWN AS NON-LIPID ALSO KNOWN AS NON-LIPID
RETICULOENDOTHELIOSIS,PULMONARY RETICULOENDOTHELIOSIS,PULMONARY
HISTIOCYTOSIS X, PULMONARY LANGERHANS CELL HISTIOCYTOSIS X, PULMONARY LANGERHANS CELL
GRANULOMATOSISGRANULOMATOSIS
•CHARACTERISED BY BONY CHARACTERISED BY BONY
LESIONS,EXOPHTHALMOS,DIABETES INSIPIDUSLESIONS,EXOPHTHALMOS,DIABETES INSIPIDUS
•ETIOLOGY NOT CLEAR,IMMUNE SYSTEM DISODER?ETIOLOGY NOT CLEAR,IMMUNE SYSTEM DISODER?
•PROLIFERATION OF MONONUCLEAR LANGERHANS PROLIFERATION OF MONONUCLEAR LANGERHANS
HISTIOCYTES WITH SIMULTANEOUS DAMAGE IN HISTIOCYTES WITH SIMULTANEOUS DAMAGE IN
VARIOUS ANATOMIC LOCATIONS.VARIOUS ANATOMIC LOCATIONS.

33. HAND-SCHULLER-CHRISTIAN HAND-SCHULLER-CHRISTIAN
DISEASEDISEASE
•FEATURES OF SEVERE PERIODONTAL FEATURES OF SEVERE PERIODONTAL
DISEASE,TOOTH MOBILITY/TOOTH DISEASE,TOOTH MOBILITY/TOOTH
LOSS,MORE COMMON IN POSTERIOR LOSS,MORE COMMON IN POSTERIOR
MANDIBLEMANDIBLE
•ALVEOLAR BONE ATROPHYALVEOLAR BONE ATROPHY
•MULTIFOCAL DISSEMINATED LESIONSMULTIFOCAL DISSEMINATED LESIONS
•WIDE AGE RANGE,USUALLY UNDER AGE 10WIDE AGE RANGE,USUALLY UNDER AGE 10
•MILD DULL PAINMILD DULL PAIN

34. HAND-SCHULLER-CHRISTIAN HAND-SCHULLER-CHRISTIAN
DISEASEDISEASE
HYPERPLASIA AND PROLIFERATION OF THE
RETICULOENDOTHELIAL SYSTEM
INTRACYTOPLASMIC BIRBECK GRANULES
DENSE INFILTRATION WITH CD1a POSITIVE HISTOCYTES

35. HAND-SCHULLER-CHRISTIAN HAND-SCHULLER-CHRISTIAN
DISEASEDISEASE
•MULTILOCULAR MULTILOCULAR
“PUNCHED OUT” “PUNCHED OUT”
RADIOLUCENCYRADIOLUCENCY
•SHARP/ILL-DEFINED SHARP/ILL-DEFINED
NON-CORTICATED NON-CORTICATED
BORDERSBORDERS
•OSTEOLYTIC LESIONSOSTEOLYTIC LESIONS
•TEETH FLOATING IN TEETH FLOATING IN
SPACESPACE

36. HAND-SCHULLER-CHRISTIAN HAND-SCHULLER-CHRISTIAN
DISEASEDISEASE
DIAGNOSIS:DIAGNOSIS:
-SUSPECTED ON CLINICAL PRESENTATION-SUSPECTED ON CLINICAL PRESENTATION
-BIOPSY,IMMUNOHISTOLOGICAL STAINING -BIOPSY,IMMUNOHISTOLOGICAL STAINING
USING CD1a SURFACE ANTIGENUSING CD1a SURFACE ANTIGEN
DIFFERENTIAL DIAGNOSIS INCLUDES: DIFFERENTIAL DIAGNOSIS INCLUDES:
PERIODONTAL DISEASE,ODONTOGENIC PERIODONTAL DISEASE,ODONTOGENIC
CYST,GIANT CELL GRANULOMA,EWING’S CYST,GIANT CELL GRANULOMA,EWING’S
SARCOMASARCOMA

37. HAND-SCHULLER-CHRISTIAN HAND-SCHULLER-CHRISTIAN
DISEASEDISEASE
•MANAGEMENT:VARIES ACCORDING TO MANAGEMENT:VARIES ACCORDING TO
LOCATION,EXTENSION AND NUMBER LOCATION,EXTENSION AND NUMBER
OF LESIONS-SURGICAL OF LESIONS-SURGICAL
CURETTAGE,LOCAL CURETTAGE,LOCAL
IRRADIATION,CHEMOTHERAPY IRRADIATION,CHEMOTHERAPY
•PROGNOSIS:AGE OF ONSET,ACTUAL PROGNOSIS:AGE OF ONSET,ACTUAL
DISEASE CATEGORY,SEVERITY OF DISEASE CATEGORY,SEVERITY OF
DISEASEDISEASE

38. LETTERER-SIWE DISEASELETTERER-SIWE DISEASE
•Occurs primarily in infants with rapid Occurs primarily in infants with rapid
progression and involvement of progression and involvement of
skin,viscera and bone marrowskin,viscera and bone marrow
•Pancytopenia,lymphadenoppathy,liver and Pancytopenia,lymphadenoppathy,liver and
splenic enlargementsplenic enlargement
•Treatment-chemotherapeutic agents,fatal Treatment-chemotherapeutic agents,fatal
despite intensive treatmentdespite intensive treatment
•Allogenic bone marrow transplant –some Allogenic bone marrow transplant –some
successsuccess

39. FIBRO-OSSEOUS LESIONSFIBRO-OSSEOUS LESIONS
•FIBROUS DYSPLASIAFIBROUS DYSPLASIA
•CHERUBISMCHERUBISM
•OSSIFYING FIBROMAOSSIFYING FIBROMA
•OSTEOBLASTOMAOSTEOBLASTOMA

40. FIBROUS DYSPLASIAFIBROUS DYSPLASIA
Definition: A non-neoplastic primary disorder of bone in Definition: A non-neoplastic primary disorder of bone in
which normal medullary bone is replaced by a variable which normal medullary bone is replaced by a variable
amount of structurally weak fibrous and osseous tissue.amount of structurally weak fibrous and osseous tissue.

41. FIBROUS DYSPLASIAFIBROUS DYSPLASIA
•Structurally weak bone,Structurally weak bone,
increased level of alkaline phosphatase increased level of alkaline phosphatase
•Calcium and Phosphorus normal Calcium and Phosphorus normal
•Signs: Rapid growth, pain, elevated alkaline Signs: Rapid growth, pain, elevated alkaline
phosphatasephosphatase
•Congenital developmental anomaly caused Congenital developmental anomaly caused
byby
disturbances from bone forming disturbances from bone forming
mesenchyme. mesenchyme.

42. FIBROUS DYSPLASIAFIBROUS DYSPLASIA
Clasification:Clasification:
Monostotic Monostotic
Polyostotic Polyostotic
McCune - Albright SyndromeMcCune - Albright Syndrome

43. FIBROUS DYSPLASIAFIBROUS DYSPLASIA
MONOSTOTICMONOSTOTIC
•Most common 80 - 85% Most common 80 - 85%
•No gender predilection No gender predilection
•Often quiesces at puberty Often quiesces at puberty
•25% involve the head and neck region 25% involve the head and neck region
•Most common sites: Most common sites:
femur, tibia, ribs, skullfemur, tibia, ribs, skull

44. FIBROUS DYSPLASIAFIBROUS DYSPLASIA
PolyostoticPolyostotic
•10 - 15% of the cases 10 - 15% of the cases
•Involves 2 or more bones Involves 2 or more bones
•Female predilection Female predilection
•50% have head and neck involvement 50% have head and neck involvement
•This form is less likely to become This form is less likely to become
inactiveinactive

45. FIBROUS DYSPLASIAFIBROUS DYSPLASIA
•3% of all cases 3% of all cases
•Multiple bony lesions Multiple bony lesions
•Hyperpigmentation: Café au lait Hyperpigmentation: Café au lait
spots spots
•Endocrine dysfunction Endocrine dysfunction
• Hyperthyroidism Hyperthyroidism
• Precocious puberty in femalesPrecocious puberty in females

46. FIBROUS DYSPLASIAFIBROUS DYSPLASIA
CLINICAL FEATURES : Head and NeckCLINICAL FEATURES : Head and Neck
•Gross deformity of affected bony Gross deformity of affected bony
structures structures
•Malocclusion due to displacement of the Malocclusion due to displacement of the
roots of teeth roots of teeth
•Cranial nerve impingement Cranial nerve impingement
•> then 95% of cases present as affecting > then 95% of cases present as affecting
the temporal bonethe temporal bone

47. FIBROUS DYSPLASIAFIBROUS DYSPLASIA

48. •Fibrous Dysplasia Radiographic:Fibrous Dysplasia Radiographic:
•Pagetoid: (56%) Pagetoid: (56%)
•- Radiopaque/ radiolucent - Radiopaque/ radiolucent
areas, ìground glass appearance areas, ìground glass appearance
•Sclerotic (23%): Sclerotic (23%):
•- Homogenously dense - Homogenously dense
•Cystic (21%): Cystic (21%):

49. FIBROUS DYSPLASIAFIBROUS DYSPLASIA
•Plump fibroblasts adjacent Plump fibroblasts adjacent
to metaplastic immature to metaplastic immature
woven bone woven bone
•Irregular, poorly oriented Irregular, poorly oriented
trabeculae trabeculae
•Lack of osteoblastic Lack of osteoblastic
rimming rimming
•Resembling Chinese Resembling Chinese
Characters Characters

50. FIBROUS DYSPLASIAFIBROUS DYSPLASIA
•Ossifying fibroma Ossifying fibroma
•Pagets disease Pagets disease
•Aneurysmal bone cyst Aneurysmal bone cyst
•Giant cell tumor Giant cell tumor
•Browns tumor Browns tumor
•Chronic osteomyelitis Chronic osteomyelitis
•Odontogenic tumorOdontogenic tumor

51. FIBROUS DYSPLASIAFIBROUS DYSPLASIA
•Conservative surgical excision or Conservative surgical excision or
osteoplastyosteoplasty
•Radical resection and grafting with Radical resection and grafting with
cortical bonecortical bone
•Medullary bone will be replaced by Medullary bone will be replaced by
fibrous bone if used in graftingfibrous bone if used in grafting

52. MALIGNANT MESENCHYMAL MALIGNANT MESENCHYMAL
TUMORSTUMORS
•Osteogenic sarcomaOsteogenic sarcoma
•Ewing’s sarcomaEwing’s sarcoma
•ChondrosarcomaChondrosarcoma
•FibrosarcomaFibrosarcoma

53. Osteogenic SarcomaOsteogenic Sarcoma
•20% OF ALL 20% OF ALL
SARCOMAS,MOST COMMON SARCOMAS,MOST COMMON
PRIMARY BONE TUMOR PRIMARY BONE TUMOR
AFTER PLASMA CELL AFTER PLASMA CELL
MYELOMA,5% JAW MYELOMA,5% JAW
INVOLVEMENTINVOLVEMENT
•MAXILLA(51%),MALES(62%)MAXILLA(51%),MALES(62%)
•HISTOLOGIC TYPES-HISTOLOGIC TYPES-
OSTEOBLASTIC,CHONDROBLOSTEOBLASTIC,CHONDROBL
ASTIC,FIBROBLASTIC,TELANGASTIC,FIBROBLASTIC,TELANG
IECTATICIECTATIC
•SITE OF ORIGIN-SITE OF ORIGIN-
CONVENTIONAL,JUXTACORTICONVENTIONAL,JUXTACORTI
CAL, EXTRASKELETALCAL, EXTRASKELETAL

54. ““SUNBURST APPEARANCE”SUNBURST APPEARANCE”

55. HISTOLOGYHISTOLOGY

•The microscopic appearance The microscopic appearance
of osteosarcoma consists of of osteosarcoma consists of
malignant fibrous connective malignant fibrous connective
tissue stroma producing tissue stroma producing
abnormal bone. In this case, abnormal bone. In this case,
the stroma is loose but the the stroma is loose but the
cells are malignant and there cells are malignant and there
is considerable heavily is considerable heavily
mineralized osseous tissue mineralized osseous tissue
being formed.being formed.

56. •Osteosarcoma Osteosarcoma
showing spindling showing spindling
malignant fibrous malignant fibrous
stroma with formation stroma with formation
of poorly calcified, of poorly calcified,
markedly irregular markedly irregular
bone trabeculae.bone trabeculae.

57. •Some osteosarcomas Some osteosarcomas
display cartilage display cartilage
formation. The cartilage formation. The cartilage
is readily visible but there is readily visible but there
are darkly calcified bone are darkly calcified bone
trabeculae at one corner.trabeculae at one corner.
• Higher magnification Higher magnification
shows that bone is being shows that bone is being
formed directly from formed directly from
malignant stroma; malignant stroma;
therefore, the tumor is therefore, the tumor is
best classified as best classified as
osteosarcoma.osteosarcoma.

58. HISTOLOGYHISTOLOGY

•In some instances, the In some instances, the
tumor is composed of tumor is composed of
densely packed poorly densely packed poorly
differentiated malignant differentiated malignant
cells and a minimal cells and a minimal
quantity of poorly quantity of poorly
calcified bone which is calcified bone which is
sometimes difficult for sometimes difficult for
the pathologist to the pathologist to
detect.detect.

59. TREATMENT,PROGNOSISTREATMENT,PROGNOSIS
•5 YEAR SURVIVAL RATE-25 TO40%5 YEAR SURVIVAL RATE-25 TO40%
•MANDIBLE BETTER PROGNOSISMANDIBLE BETTER PROGNOSIS
•BEST TREATED BY RADICAL BEST TREATED BY RADICAL
MAXILLECTOMY/MANDIBULECTOMY,CHEMOTMAXILLECTOMY/MANDIBULECTOMY,CHEMOT
HERAPY/RADIOTHERAPY FOR HERAPY/RADIOTHERAPY FOR
RECURRENCES,METASTATIC DISEASE,SOFT RECURRENCES,METASTATIC DISEASE,SOFT
TISSUE EXTENSIONTISSUE EXTENSION
•RARELY METASTASISE TO LYMPH NODESRARELY METASTASISE TO LYMPH NODES
•MOST COMMON SITES OF METASTASES- LUNG MOST COMMON SITES OF METASTASES- LUNG
AND BRAIN(MEAN SURVIVAL 6 MONTHS)AND BRAIN(MEAN SURVIVAL 6 MONTHS)

60. Ewing’s sarcomaEwing’s sarcoma
•1st described in 1921 by 1st described in 1921 by
Dr. James Ewing Dr. James Ewing
Rare Childhood Rare Childhood
malignancy malignancy
Unknown origin Unknown origin
•Mandible > Maxilla Mandible > Maxilla
Males > FemalesMales > Females
•Caucasian , Caucasian ,
First two decades of life, First two decades of life,
but rarelybut rarely before 5 YOA before 5 YOA
•Painless Mass Painless Mass
•Swelling Swelling
•Fatigue, Anemic-lookingFatigue, Anemic-looking

61. Ewing’s sarcomaEwing’s sarcoma
•Non-oral Non-oral
Appearance Appearance
Onion skin/sunray Onion skin/sunray
•Oral Appearance Oral Appearance
Poorly defined Poorly defined
solitary osteolytic solitary osteolytic
imageimage

62. Ewing’s sarcomaEwing’s sarcoma
•Small, round blue Small, round blue
cell tumor cell tumor
•Mensenchymal or Mensenchymal or
neuroectodermal neuroectodermal
in originin origin

63. Ewing’s sarcomaEwing’s sarcoma
DIFFERENTIAL DIAGNOSIS:DIFFERENTIAL DIAGNOSIS:
•Lymphoma, neuroblastoma, Lymphoma, neuroblastoma,
rhabdomyosarcoma rhabdomyosarcoma
Radiographic Radiographic
•osteomyelitis, chondrosarcoma, osteomyelitis, chondrosarcoma,
•osteogenic sarcomaosteogenic sarcoma

64. Ewing’s sarcomaEwing’s sarcoma
Tests Involved with Definitive Tests Involved with Definitive
DiagnosisDiagnosis
•Immunohistochemical Profile Immunohistochemical Profile
•Radiographic Findings Radiographic Findings
•Histological FindingsHistological Findings

65. Ewing’s sarcomaEwing’s sarcoma
Treatment for Ewingís SarcomaTreatment for Ewingís Sarcoma
•Chemotherapy Chemotherapy
•Surgery Surgery
•Radiotherapy Radiotherapy
Dental Management of Patients with ES
•Caries prevention/Xerostomia
•Opportunistic infections
•Local ulcerations

66. NEUROGENIC TUMORSNEUROGENIC TUMORS
•NEUROFIBROMANEUROFIBROMA
•MELANOTIC NEUROECTODERMAL MELANOTIC NEUROECTODERMAL
TUMOR OF INFANCYTUMOR OF INFANCY
•GANGLIONEUROMAGANGLIONEUROMA
•NEURILEMOMANEURILEMOMA

67. NEUROGENIC TUMORSNEUROGENIC TUMORS
•MOST RARE PRIMARY JAW TUMORSMOST RARE PRIMARY JAW TUMORS
•CHUONG AND KABAN REPORTED CHUONG AND KABAN REPORTED
6CASES,4NEUROFIBROMA,1MNTI, 6CASES,4NEUROFIBROMA,1MNTI,
1GANGLIONEUROMA1GANGLIONEUROMA
•NEUROFIBROMA-MOST COMMONNEUROFIBROMA-MOST COMMON

68. NEUROFIBROMANEUROFIBROMA
•CENTRAL NEUROFIBROMA-OCCUR AS SOLITARY CENTRAL NEUROFIBROMA-OCCUR AS SOLITARY
LESIONSLESIONS
•TUMOR AND ADJACENT BONE EXTREMELY VASCULARTUMOR AND ADJACENT BONE EXTREMELY VASCULAR
•LESIONS MORE COMMON IN MANDIBLE-DULL LESIONS MORE COMMON IN MANDIBLE-DULL
ACHING PAIN,PARAESTHESIAACHING PAIN,PARAESTHESIA
•RADIOGRAPHICALLY AS MULTILOCULAR,DIFFUSE RADIOGRAPHICALLY AS MULTILOCULAR,DIFFUSE
RADIOLUCENCY WITHCORTICAL THINNINGRADIOLUCENCY WITHCORTICAL THINNING
•HISTOLOGICALLY COMPOSED OF SCHWANN CELLS HISTOLOGICALLY COMPOSED OF SCHWANN CELLS
AND NEURITESAND NEURITES
•DEVELOPMENT OF SARCOMA IN15% OF CASESDEVELOPMENT OF SARCOMA IN15% OF CASES

69. MANAGEMENTMANAGEMENT
•CLOSE OBSERVATION AND SURGICAL CLOSE OBSERVATION AND SURGICAL
EXCISION OF LESIONEXCISION OF LESION
•SURGICAL GOAL-EXCISE OR DEBULK SURGICAL GOAL-EXCISE OR DEBULK
THE SOFT TISSUE MASS ANDC THE SOFT TISSUE MASS ANDC
RECONTOUR UNDERLYING BONERECONTOUR UNDERLYING BONE
•RECURRENCE RATE HIGHRECURRENCE RATE HIGH

70. DIAGNOSIS IN AGGRESSIVE JAW DIAGNOSIS IN AGGRESSIVE JAW
TUMORSTUMORS
•PRESENTING COMPLAINTSPRESENTING COMPLAINTS
•CLINICAL FEATURESCLINICAL FEATURES
•RADIOGRAPHICFEATURESRADIOGRAPHICFEATURES
•HISTOLOGICFEATURESHISTOLOGICFEATURES
•INVESTIGATIONSINVESTIGATIONS

71. JAW LESIONSJAW LESIONS
•ODONTOGENIC TUMORSODONTOGENIC TUMORS
•BENIGN NON-ODONTOGENICBENIGN NON-ODONTOGENIC
•INFLAMMATORYINFLAMMATORY
•MALIGNANT NON-ODONTOGENIC MALIGNANT NON-ODONTOGENIC
TUMORSTUMORS
•METABOLIC AND GENETICMETABOLIC AND GENETIC

72. Odontogenic TumorsOdontogenic Tumors
•Epithelial tumorsEpithelial tumors
–AmeloblastomaAmeloblastoma
–S.O.TS.O.T
–CEOTCEOT
–AOTAOT
–CCOTCCOT
•Mesenchymal tumorsMesenchymal tumors
–Odontogenic MyxomaOdontogenic Myxoma
–CementoblastomaCementoblastoma
–Periapical Cemental DysplasiaPeriapical Cemental Dysplasia
–Cementifying FibromaCementifying Fibroma
•Mixed TumorsMixed Tumors
–OdontomaOdontoma
–Ameloblastic FibromaAmeloblastic Fibroma
–Ameloblastic Fibro-odontomaAmeloblastic Fibro-odontoma

73. Inflammatory Jaw LesionsInflammatory Jaw Lesions
•Acute OsteomyelitisAcute Osteomyelitis
•Chronic OsteomyelitisChronic Osteomyelitis
–FSOFSO
–DSODSO
–Garre’s Osteomyelitis Garre’s Osteomyelitis

74. Malignant NeoplasmsMalignant Neoplasms
•OsteosarcomaOsteosarcoma
•ChondrosarcomaChondrosarcoma
•Ewing’s SarcomaEwing’s Sarcoma
•Burkitt’S LymphomaBurkitt’S Lymphoma
•Plasma Cell NeoplasmsPlasma Cell Neoplasms
–Multiple MyelomaMultiple Myeloma
–Solitary Plasmacytoma of BoneSolitary Plasmacytoma of Bone
•Metastatic CarcinomasMetastatic Carcinomas

75. Metabolic & GeneticMetabolic & Genetic
•MetabolicMetabolic
–Paget’s DiseasePaget’s Disease
–HyperparathyroidismHyperparathyroidism
–AcromegalyAcromegaly
–Phantom Bone DiseasePhantom Bone Disease
•GeneticGenetic
–CherubismCherubism
–OsteopetrosisOsteopetrosis
•OthersOthers
–Pierre Robin SyndromePierre Robin Syndrome
–Hemifacial HypertrophyHemifacial Hypertrophy
–Osteogenesis ImperfectaOsteogenesis Imperfecta

76. Non-Odontogenic TumorsNon-Odontogenic Tumors
•Ossifying fibromaOssifying fibroma
•Fibrous dysplasiaFibrous dysplasia
•OsteoblastomaOsteoblastoma
•ChondromaChondroma
•OsteomaOsteoma
•Tori & ExostosisTori & Exostosis