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REFERENCES
 Odontogenic tumours & Allied lesions- Peter A Reichart , Hans P Philipson
 Textbook of Oral Radiology- White & Pharoah (6th ed.)
 Diagnostic imaging of jaws- Langland & Langlais
 Differential Diagnosis of Oral & Maxillofacial lesions- Wood & Goaz (5th
ed.)
 Principles Practice Oral Radiologic Interpretation- H.M Worth
 Ameloblastic fibroma: A review of published studies with special reference
to its nature and biological behavior Yan Chen, Jing-Ming Wang, Tie-Jun Li
doi:10.1016/j.oraloncology.2007.05.009
 Martin-Granizo Lopez R, Ortega L, Gonzalez Corchon MA et al:
Ameloblastic fibroma of the mandible. Report of two cases, Med Oral
8(2):150-153, 2003
CONTENTS
 Introduction
 Definitions
 Benign tumours
- Clinical features
- Radiographic features
- Differential diagnosis
- Treatment
 Malignant tumours
 Odontogenic
 Non-odontogenic
DEFINITIONS
NEOPLASM : A neoplasm as defined by Willis is “ An abnormal mass of
tissue, the growth of which exceeds and is uncoordinated with that of the
normal tissues and persists in the same excessive manner after the cessation of
the stimuli which evoked the change. ”
BENIGN TUMOUR : Is a new growth, which is limited by a capsule and
grows by local expansion without causing any harm to the host, excepting
its position in a vital organ.
MALIGNANT TUMOUR : Is a new growth which is characterized by rapid
growth, sign of invasion, absence of capsule and last of all dissemination to
other parts of the body usually by hematogenous or lymphatic route or
both.
FEATURES OF BENIGN TUMOR
 Encapsulated
 Slow growing
 Painless
 No metastasis
FEATURES OF MALIGNANT
TUMOR
ODONTOGENIC TUMOURS
Classification
Benign Tumors
1) Odontogenic epithelium with mature, fibrous stroma; odontogenic
mesenchyme not present
 Ameloblastomas
 Calcifying epithelial odontogenic tumor
 Adenomatoid odontogenic tumor
 Squamous odontogenic tumour
Editorial and Consensus Conference -Lyon, France (WHO/ IARC) in July 2003
2) Odontogenic epithelium with odontogenic mesenchyme with or
without dental hard tissue formation
 Ameloblastic fibroma
 Ameloblastic fibro-dentinoma
 Ameloblastic fibro-odontoma
 Complex odontoma
 Compound odontoma
 Odontoameloblastoma
 Calcifying cystic odontogenic tumor
Editorial and Consensus Conference -Lyon, France (WHO/ IARC) in July 2003
3) Mesenchyme and/or odontogenic ectomesenchyme with or without
included odontogenic epithelium
 Odontogenic fibroma
 Odontogenic myxoma or fibromyxoma
 Cementoblastoma
Editorial and Consensus Conference -Lyon, France (WHO/ IARC) in July 2003
Malignant
I Odontogenic Carcinomas
 Metastasizing, malignant ameloblastoma
 Ameloblastic carcinoma
 Primary intraosseuos squamous cell carcinoma
 Clear cell odontogenic carcinoma
 Ghost cell odontogenic carcinoma
II Odontogenic Sarcomas
 Ameloblastic fibrosarcoma
 Ameloblastic fibrodentino and fibro-odontosarcoma
Editorial and Consensus Conference -Lyon, France (WHO/ IARC) in July 2003
AMELOBLASTOMA
WHO Definition -
• Ameloblastoma is a polymorphic neoplasm consisting of proliferating odontogenic
epithelium which usually has a follicular or
plexiform pattern lying in a fibrous stroma
Reichart and Slootweg Definition
• Ameloblastoma is a polymorphous neoplasm consisting of proliferating odontogeni
c epithelium usually occurring in follicular or
plexiform patterns embedded in mature connective tissue stroma.
As stated by Robinson,
Ameloblastoma is usually unicentric, non-functional, intermittent in growth,
anatomically benign and clinically persistent.
HISTORICAL BACKGROUND
 1826 – Guzack – a tumor of jaw
 1868 – Broca – 1st neoplasm of this nature in literature
 1879 – Falkson gave its thorough description.
 1885 – Malassez – ‘Admantinoma’
 1934 – Churchill – ‘Ameloblastoma’
PATHOGENESIS
 Resemblance to odontogenic apparatus – derived from the same.
 Malassez, 1885 – epithelial rests adj. roots of teeth – ‘les debris
épithéliaux’ - proliferate  Adamantine epithelioma
 Origin – varied origin
 Stimulus for initiation - unknown
GENETIC ALTERATION
Overexpressed genes
 Proto-oncogene FOS – most highly
-Fos protein – Activating protein-1 (transcription factor)
-Cell proliferation, differentiation, oncogenic transformation
-Bone development, regulator of osteoclast - macrophage
lineage determination, bone remodelling.
 TNFR 1A – induction of AP-1 activity
-TNF-α – signal transduction
-Inflammation, cell proliferation, differentiation & apoptosis
Heikinheimo et al (2002, J Dent Res)
Underexpressed genes –
 Cadherins, KRT7, NOTCH & TGFB1
 Disturbances in cell adhesion & cell-cell communication
 Sonic hedgehog (SHH) – (expression of PTCH)
 Dysregulation of signalling pathway for ectomecenchymal interactions
Heikinheimo et al (2002, J Dent Res)
VARIANTS
 Classical solid / multicystic ameloblastoma (SMA)
 Unicystic ameloblastoma (UA)
 Peripheral ameloblastoma (PA)
 Desmoplastic ameloblastoma (DA)
SOLID / MULTICYSTIC
AMELOBLASTOMA
Clinical features-
 Early stages – no clinical
signs
 Later –
 Facial deformity – swelling
(bony hard & bulky)
 Loosening of teeth
 Fractures
 Pain – less frequent
(pressure on nerves, sec.
infection)
 Large tumors – thinning of
cortices (egg shell crackling)
 Perforation – late feature
Age –
Mean age – 35.9 yrs (range 9-
92yrs)
Maxillary tumours – 47 yrs
Mandibular tumours – 35.2 yrs
Sex distribution –
Male : female – 1.1 : 1
Reichart et al 1995, biological profile of 3677
cases of ameloblastoma
RADIOGRAPHIC FEATURES
Honeycomb appearance
Spider-like appearance
Soap-bubble appearance
Mother-daughter cell appearance
Unilocular pattern
ROOT RESORPTION PATTERN
Knife edged root resorption Multiplanar root resorption
RADIOGRAPHIC DESCRIPTION OF AMELOBLASTOMA
WORTH (1963)
4 types
 Unilocular
 “Gross caricature of spider”
 Multilocular
 Replacement of normal bone with honeycomb appearance.
CT SCANS
CBCT
MRI
T2WI Gd – T1WI
HISTOPATHOLOGICAL VARIANTS
Islands –
Central polyhedral cells
Surrounded by layer of
cuboidal / Columnar cells
Cystic degenerations –
Within epithelial islands
Follicular
Plexiform
Tumor epithelium in a network
Cyst formation – within stroma
Acanthomatous
0 Extensive squamous metaplasia
0 Keratin formation
Granular
0 Granular transformation of central stellate cells –
round/columnar/cuboidal
0 Acidophillic granules – lysosomal aggregates
Basal
PERIPHERAL AMELOBLASTOMA
 Extraosseous, soft tissue
ameloblastoma
 Histology – similar to SMA
 Tooth bearing region
 Painless, sessile, firm,
 Surface – smooth / pebbly,
granular / papillary, warty
 Pink / red / dark red
 Duration – 2 days – 20 yrs
 2 - 10% of ameloblastomas
 R/F- cupping or saucerization
 Resemblance to –
 Epulis
 Papilloma
 Pyogenic granuloma
UNICYSTIC AMELOBLASTOMA
 ‘UNICYSTIC’ – macro & microscopic appearance – well defined,
monocystic cavity – lined focally / entirely of ameloblastomatous
epithelium.
Synonyms –
 Cystic ameloblastoma
 Mural ameloblastoma
 Age –
 Dentigerous variant –
younger (mean age – 16.5)
 Non dentigerous – mean age
– 32.2
 Male : female –
 Dentigerous - 1.6 : 1
 Non-dentigerous – 1 : 1.8
 Location – Mandible
 Posterior mandible, Asc.
Ramus
 Dentigerous variety – 3rd
molar
HISTOLOGICAL VARIANTS
(ACKERMAN ET AL, 1988 - SUBTYPES)
1. - Luminal UA
1.2 - Luminal & Intraluminal UA
1.2.3 - Luminal, Intraluminal & intramural
1.3 - Luminal & Intramural
RADIOLOGIC FEATURES
DESMOPLASTIC AMELOBLASTOMA
 Variant of SMA –
extensive stromal
collagenization /
desmoplasia
 Benign, locally infiltrative
 Location - Maxilla =
mandible
 Age – mean – 42.8 yrs
 Male : female – 1 : 0.9
 Origin – similar to SMA
OTHER TYPES
 Clear cell A
 KeratoA & Papilliferous A
 Mucus cell differentiation
 Hemangiomatous Ameloblastoma
 Extragnathic Adamantinoma
 Metastasizing Ameloblastoma
DIFFERENTIAL DIAGNOSIS
 OKC
 Odontogenic Myxoma
 Aneurysmal bone cyst
 Central gaint cell granuloma
 Central hemangioma
TREATMENT
 Treatment depends on the type of lesion –
 SMA – radical surgical intervention
 UA – conservative surgical enucleation
 Peripheral ameloblastoma – more conservative surgery
 Radiotherapy –
 1982 – Reynolds et al – effects of radiation on ameloblastoma –
usefulness
 Not used as 1st line of treatment
PROGNOSIS
 SMA –
 ↑ Recurrence
 Maxilla – spreads faster - ↑ morbidity & mortality
- Posterior maxilla – poorest prognosis
 Mandible – restricted by thick cortex
 Follow up – 5-10 yrs post-surgery
 DA – same rate of recurrence as SMA
 Peripheral ameloblastoma – low recurrence (16-19%)
 UA – 10.7%
CALCIFYING EPITHELIAL ODONTOGENIC
TUMOUR
 Also called as Pindborg tumor,
first described by
J.J PINDBORG in 1956
 Benign, odontogenic neoplasm
exclusively epithelial in origin
 1% of odontogenic tumors
 ORIGIN – remnants of
primitive dental lamina
CLINICAL FEATURES
 Age ; Sex – 4th decade ;
Males
 Site – Mandible : Maxilla = 2
:1
 Malignant - Locally invasive
with high recurrence rate
RADIOGRAPHIC FEATURES
 Expanded cortices in all dimensions
 Radiolucent ; poorly defined, non-
corticated borders
 Unilocular, multilocular or “moth-
eaten”
 “ Driven-snow ” appearance from
multiple radiopaque foci
 Root divergence / resorption ;
impacted tooth
 Histology
 Islands of eosinophilic
epithelial cells
 Cells infiltrate bony trabeculae
 Nuclear hyperchromatism and
pleomorphism
 Psammoma-like calcifications
(Liesegang rings)
DIFFERENTIAL DIAGNOSIS
 Dentigerous cyst
 Adematoid odontogenic tumor
 Ameloblastic fibro-odontoma
TREATMENT
 Behaves like ameloblastoma
 Low recurrence rate
 En bloc resection, hemimandibulectomy/partial maxillectomy.
 Recurrence rate
- Franklin & Pindborg (1976) : 14%
ADENOMATOID ODONTOGENIC TUMOUR
 Adenoameloblastoma , Ameloblastic adenomatoid tumor
 First reported by - STAFNE
 Term coined by PHILIPSEN & BIRN (1969)
 ORIGIN – Dental epithelium
 TYPES-
 Peripheral
 Central - Follicular & Extra-follicular
CLINICAL FEATURES
 Age - 2nd decade
 Male : Female – 1 : 2
 Slow growing expansile lesion
 Location : Anterior maxilla
 Associated with unerupted tooth
RADIOGRAPHIC FEATURES
 Well-corticated, non-scalloped
margin.
 Medial growth in maxilla
 Snowflake, animal prints, foot
prints
 Radiolucent band
HISTOLOGY
Thick fibrous capsule, clusters of spindle cells, columnar
cells (rosettes, ductal) throughout
DIFFERENTIAL DIAGNOSIS
 Dentigerous cyst
 Ameloblastic fibroma
 CEOT
Treatment – enucleation, recurrence is rare
Recurrence rate - Low : 0.2%
SQUAMOUS ODONTOGENIC TUMOUR
 Benign Epithelial Odontogenic Tumor
 Hamartomatous proliferation
 Origin
CLINICAL FEATURES
 2nd and 3rd decade
 1:1
 Tooth mobility
 Tenderness on percussion
RADIOGRAPHIC FEATURES
HISTOLOGY
 Oval nest of squamous epithelium in mature collagen stroma.
DIFFERENTIAL DIAGNOSIS
 Dentigerous & Radicular cyst
 Treatment – Conservative enucleation & thorough curettage ; low
recurrence rate.
II. ODONTOGENIC EPITHELIUM
WITH ODONTOGENIC
MESENCHYME WITH OR WITHOUT
DENTAL HARD TISSUE
FORMATION
AMELOBLASTIC FIBROMA
 AF – True mixed odontogenic tumor
 Consists of both epithelial and ectomesenchymal components are
neoplastic.
 1946, Thoma and Goldman were the first to classify this tumour as a
separate entity
CLINICAL FEATURES
 Hard swelling
 Mandibular posteriors
 2nd and 3rd decade
 Tumour size - 1 - 10 cm
 Recurrence rate - 33.3%
 Malignant transformation - 11.4%
Yan Chen, Jing-Ming Wang, Tie-Jun Li . Ameloblastic fibroma : A review of published studies
with special reference to its nature and biological behavior.
RADIOGRAPHIC FEATURES
Martin-Granizo Lopez R, Ortega L, Gonzalez Corchon MA et al: Ameloblastic fibroma of the
mandible. Report of two cases, Med Oral 8(2):150-153, 2003.
DIFFERENTIAL DIAGNOSIS
 Dentigerous cyst
 Ameloblastoma
TREATMENT
Conservative surgery
 Excision
 Enucleation
 Curettage
Radical surgery
 Marginal resection
 Segmental resection
 Hemisection
AMELOBLASTIC FIBRO-ODONTOMA
 Hooker -1967
 Ameloblastic fibroma + odontome
 2% incidence- Regezi et al
FEATURES
C/F
 83%- unerupted tooth
 2nd decade of life
 M : F - 1.4 : 1
 Posterior mandible - 53.2%
R/F-
 Expansile, smooth- well defined cortication
 Radiopaque flecks - 1 – 2 mm to 1cm
 Tooth displacement
DIFFERENTIAL DIAGNOSIS
 Odontome
TREATMENT
 Conservative enucleation
 Resection with removal of impacted teeth.
ODONTOME
 Benign Hamartoma
 1971 - Pindborg et al.
2 types - Compound and complex
 Pathogenesis
 Local trauma
 Infection
 Family history
 Genetic mutation
CLINICAL FEATURES
 Slow growing , expansile lesion
 Compound – 2nd decade of life ; before 20yrs of age
 Complex - 2nd decade of life ; before 30yrs of age
 M : F - 1.5 : 1
Location –
 Compound – anterior maxilla ; posterior mandible – 2 : 1
 Complex – anterior maxilla ; posterior mandible – 1 : 2
 Displacement of tooth
RADIOGRAPHIC FEATURES
Compound odontome Complex odontome
VARIANTS
 Cystic odontome
 28% incidence
 Amelobalstic odontome
Treatment : Surgical enucleation
Differential diagnosis
 Ameloblastic fibro-odontome
III. Mesenchyme or odontogenic
ectomesenchyme with or without included
odontogenic epithelium
ODONTOGENIC MYXOMA
 Benign non-encapsulated odontogenic neoplasm.
 In 1947- Thoma and Goldman
Pathogenesis
 Arises from mesenchymal portion of the tooth germ, dental papilla, the
follicle or the periodontal ligament.
CLINICAL FEATURES
 1-3%
 2nd and 4th decade
 M:F- 1:1.6
 Mand posteriors
RADIOGRAPHIC FEATURES
Two pattern depending on evolution of tumor:
 Osteoporotic appearance
 Breakout/destructive phase
HISTOLOGICAL FEATURE
DIFFERENTIAL DIAGNOSIS
 Ameloblatoma
 Central giant cell granuloma
 Central hemangioma
 Osteosarcoma
 Treatment : As infiltrative local recurrence rate - 33%
 Resection with 1-1.5 cm of bone
ODONTOGENIC FIBROMA
 Two variants can be distinguished: an intraosseous or central type
(COF) and an extraosseous or peripheral type (POF)
 Clinical variants- 1) hyperplastic, 2) simple, 3) WHO type
 C/F - age- 3rd decade
 M:F- 1:2.8
 Maxilla : mandible – 1 : 6.8
Siar C H et al ,Clinicopathological study of peripheral and central odontogenic fibromas (WHO –
type in Malaysians ( 1967 - 9 5 ). B r J Oral Maxillofac Su rg 2000 ;38: 19- 22 .
RADIOGRAPHIC FEATURES
BENIGN CEMENTOBLASTOMA
 True cementoma, Attached cementoma
 First reported by NORBERG (1930)
 Term given by KRAMER
 < 1% of all odontogenic tumors
 Unique in two ways:
 True neoplasm of cementoblasts
 Attached routinely to an involved tooth
 Origin - PDL
CLINICAL FEATURES
 2nd and 3rd decade
 Mand 1st premolar and molar
 M : F – 1 : 1.2
 SYMPTOMS
 Pain – 53% of cases
 SIGN
 Swelling-73% cases
RADIOGRAPHIC FEATURES
 Three radiologically distinct
stages:
 Uncalcified matrix stage
 Calcified blastic stage
 Mature stage
HISTOLOGY
DIFFERENTIAL DIAGNOSIS
 Periapical cemental dysplasia
 Enostosis
 Hypercementosis
 Sclerosing osteitis
TREATMENT
 Surgical extraction of involved tooth
 Tumor can be amputated from tooth & tooth endodontically
treated
 Pattern of flecks on CEOT
 According to histological criteria by Franklin and Pindborg, scattered
flecks are seen with central radiolucency
 Histopathologically, features of unilocular or multilocular ameloblastoma
are similar.
 Lichen planus of jaw – Odontogenic myxoma ; Traumatic bone cyst
Metastasizing malignant ameloblastoma
 Criteria for identifying metastatic diseases of the jaws –
1. Lesion must be a true metastasis localized to bone tissue, as distinguished from direct
invasion by a primary tumour of contiguous structure
2. Lesion must be verified microscopically as carcinoma
3. Primary site of the lesion must be known
 Ameloblastoma – metastasizes in spite of its benign histologic appearance.
 Associated with hypercalcemia
 Age – Third decade (5 - 74 yrs)
 Gender – M : F = 1 : 1.2
 Location : Posterior mandible
 Clinical features –
 Paresthesia / anesthesia
 Loosening of teeth
 Periodontal abscess
 Facial paralysis
 Pathologic fracture
 Radiographic features
 Frank destruction of bone without new bone formation
within the lesion or adjacent bone.
 Islands of bone with irregular margins
Ameloblastic Carcinoma
 Corio and associates – Any ameloblastoma in which there is histologic evidence
of malignant disease in the primary or recurrent tumour, independent of the
presence of metastasis.
 Most ameloblastic carcinomas presumably arise de novo, and less than 1%
of ameloblastomas undergo malignant transformation.
 Ameloblastic carcinoma, secondary type, is defined as a malignantly
transformed tumor within a pre-existing benign ameloblastoma, regardless of
the presence of metastasis.
Yukio Yoshioka, Shigeaki Toratani, Ikuko Ogawa and Tetsuji Okamoto. Ameloblastic Carcinoma, Secondary Type, of the
Mandible: A Case Report. J Oral Maxillofac Surg 71:e58-e62, 2013.
Clinical Features
 Age – 4th decade
 Female : Male - 1 : 2.7
 Swelling (61.5%), bleeding, ulceration and fistula (15.4%).
 Progressive types : cortical bone perforation, soft tissue invasion,
recurrences and metastases.
Radiographic Features
 Unilocular or multilocular
 Ragged borders
 Root resorption
 Cortical perforation
Histopathology
Kar, et al.:Ameloblastic carcinoma: A clinicopathologic dilemma – Report of two cases with total review of literature from 1984 to
2012. Annals of Maxillofacial Surgery | January - June 2014 | Volume 4 | Issue 1
Differential Diagnosis
 Metastatic carcinoma
 Squamous odontogenic tumour
TREATMENT
 Radical surgery with neck dissection
 Hemimandibulectomy / maxillectomy
 Postive margins : adjuvant radiotherapy - 5 fractions of 1.8 Gy each for a
period of 5 weeks (45 Gy).
Primary Intraosseous Squamous Cell
Carcinoma
WHO defines:
 “ Odontogenic carcinoma consisting of a squamous cell carcinoma arising within
the jaws having no initial connection with the oral mucosa and presumably
developing from residues of the odontogenic epithelium or from an odontogenic
cyst or tumour”.
Elzay 1982:
 Arising from odontogenic cysts
 Arising from ameloblastoma( malignant or AC)
 De novo
CLINICAL FEATURES
 Age : 6th and 7th decade
 M : F- 3 : 1
 Location : Posterior mandible
 Tooth mobility
 Paraesthesia
 Regional LN involvement
 Exfoliation
 Non-healing Extraction sockets (50-
60%)
Mitsuyoshi Iino et al. Solid type primary intraosseous squamous cell carcinoma in the maxilla: report of a new
case. Ear, Nose and Throat Disorders 2013, 13:13.
Radiographic Features
 Alveolar bone destruction
 Cortical perforations
 Pathologic fractures
 “Floating tooth”
 “ Bays within bays ”
Huang et al.: Primary Intraosseous Squamous Cell Carcinoma. Arch Pathol Lab Med—Vol 133, November 2009
HISTOPATHOLOGY
Differential diagnosis
 Gingival carcinomas
 Ameloblastic carcinoma
Treatment
 Partial or total maxillectomy or mandibulectomy.
 Radiotherapy - Fractionated in seven weeks each session of 50 Gy.
 Adjuvant chemotherapy
 Cisplatin 100 mg/m2 IV or 40-50 mg/m2 IV weekly for 6-7wk.
 Cetuximab 400 mg/m2 IV loading dose 1wk before the start of radiation therapy, then 250
mg/m2 weekly (premedicate with diphenhydramine and ranitidine)
.
Clear cell odontogenic carcinoma
 4th and 5th decade
 Mandible : Maxilla- 7 : 1
 M : F – 1 : 1.6
 Mandibular posterior region
 Poorly delineated radiolucency
 Radical resection and follow up
 Recurrence rate - 55%
Niharika Swain, Richa Dhariwal et al. Clear cell odontogenic carcinoma of maxilla : A case report and mini review.
J Oral Maxillofac Pathol. 2013 JanApr; 17(1): 89–94.
Ghost cell odontogenic carcinoma
 Gorlin 1962- “Malignant counterpart of calcifying odontogenic cyst”.
 Age – 4th and 5th decade
 Gender – M : F = 2.6 : 1
 Location – Maxilla > Mandible
 Clinical features
 Swelling with or without pain
 Osseous destruction with paresthesia
 Radiographic features
 Cortical destruction, obliteration of maxillary sinus
 Ill-defined radiolucency
 Opacification
Ameloblastic Fibrosarcoma
 It is a rare malignant neoplasm composed of benign , ameloblastomous
epithelium and malignant ectomesenchyme.
 Represents only 2% of all odontogenic tumors.
 Clinical features
 Pain and swelling
 Ulceration and bleeding
 Paresthesia of lower lip
 2nd decade
 Mandible : Maxilla = 2.3 : 1
 M : F - 2 : 1
Radiographic Features
 Ill-defined radiolucency
 Pathological fractures
 Treatment
 Wide local excision
 Partial maxilla or
mandibulectomy
 Post surgical radiotherapy /
chemotherapy
 Recurrence rate - 20%
Daniela Otero Pereira et al. Maxillary Ameloblastic Fibroma: A Case Report . Braz Dent J (2011) 22(2): 171-174
Osteosarcoma
 Osteosarcoma - Two types
 An osteoblastic (sclerosing) and
 An osteolytic type.
 Gender : Males > Females
 Age - 33 years (Mean)
 Location : Mandible > Maxilla
 Arise due to –
 Radiation induced
 Trauma
Clinical Features
 Pain, swelling, paraesthesia and ulceration.
 Affected teeth may be loosened and displaced
 Age- 4th & 5th decade
 M : F - 1.1 : 1
Radiographic Features
 Earliest radiographic sign - widening of the periodontal
ligament space or a radiolucency around one or more teeth.
 Later on, the lesion assumes an osteolytic radiolucent form,
an osteoblastic radiopaque form or a mixed radiolucent
image with radiopaque foci.
 Ill-defined borders.
 Expansion and destruction of the cortical plates.
 Characteristic sunray , sunburst or fan-shaped appearance
- thin spicules of new bone extend outwards away from the
bone cortex.
Chittaranjan B, Tejasvi MA, Babu BB, Geetha P. Intramedullary Osteosarcoma of the
Mandible: A Clinicoradiologic Perspective. J Clin Imaging Sci 2014;4, Suppl S1:6
TREATMENT
 The cornerstone of primary jaw osteosarcoma treatment is adequate surgical
resection.
 Radiotherapy or chemotherapy can be used in association with surgical resection
or alone as a palliative treatment in advanced cases.
 Literature review has shown that patients treated initially by aggressive local or
even radical procedures such as hemimandibulectomy fared better.
 Introduction of multi-agent chemotherapy has improved the survival rates, with
60-70% patients surviving after treatment.
CHONDROSARCOMA
 Malignant tumor of cartilaginous tissue
 Age : 3rd to 5th decade.
 Gender : Males > females.
 Painless swelling leading to the expansion of the
buccal and lingual cortical plates
 Regional lymphadenopathy is very rare
 Jaw lesions are rare.
 The teeth adjacent to the lesion may be resorbed,
loosened, or exfoliated.
SanChita kundu et al. Clinicopathologic correlation of chondrosarcoma of mandible with a case report. Contemporary Clinical
Dentistry | Oct-Dec 2011 | Vol 2| Issue 4.
 Single/ multiple radiolucencies with
poorly defined borders
 Moth-eaten radiolucencies to diffusely
opaque lesions.
 Localized widening of the periodontal
ligament space may be observed.
 In some cases, a sunray appearance
may be seen.
TREATMENT
 Wide surgical excision is the mainstay of treatment for
 These tumors are radioresistant and chemotherapy can be used as an adjuvant
therapy after wide surgical excision is made
 Prognosis is poor.
 Local recurrence occurs and may indicate subsequent metastasis; hence
adequate treatment and long-term follow-up, including periodic systemic
evaluation, are required.
EWING’S SARCOMA
 Malignant tumor of bone derived from mesenchymal
connective tissue of the bone marrow.
 Rarely occurs in the jaws
 Rapidly growing, highly invasive tumor
 Pain and swelling are the most common manifestations.
 Age : Children ; young adults
 Gender : M : F = 2 : 1
 The radiographic appearance is that of an ill-defined destructive
radiolucent lesion which may be unilocular or multilocular.
 Stimulates the periosteum to produce thin layers of bone, resulting in
an "onion skin" effect.
 Advanced cases may exhibit a sunburst appearance.
 Chemotherapy, radiation therapy and surgery, has led to an improvement in
prognosis.
 Poor prognostic factors are patients below 10 years of age, presence of
metastasis, presence of systemic symptoms, large tumor volume.
 ES of the mandible has got better prognosis than long bones since facial sites
are diagnosed earlier.
TREATMENT
BURKITT'S LYMPHOMA
 Characteristic form of non-Hodgkin's lymphoma that is endemic in Africa and
occurs sporadically in North America.
 The Epstein-Barr virus has been implicated in the etiology.
 Age : Peak : 5 years
 Primarily a tumor of childhood, with occasional cases seen in young adults.
 Location : Maxilla > Mandible.
 Gender : M : F = 2 : 1
Vasudevan V, Mohandas U, Manjunath V. Burkitt's lymphoma in leukemic phase in an Indian boy. Indian J
Dent Res 2011;22:340-4
 Clinical features –
 Loosening of teeth
 Bilateral jaw tenderness
 Swollen gingiva
 Cervical lymphadenopathy
 Radiographic features
 Ill defined radiolucency extending into tooth crypt
 Displacement of developing and erupting teeth
 Floating in air appearance
 Sunray spicules
TREATMENT
 Good response to chemotherapy, particularly cyclophosphamide.
 The tumor also has been shown to be sensitive to methotrexate, vincristine, and
cytarabine.
 Combinations of drugs have achieved remissions in more than 90% of patients.
Unfortunately, most of the cases recur and patients ultimately succumb to the
disease.
 Recent clinical trials with intensive, multiagent chemotherapeutic protocols have
shown a fair remission rate.
 Individuals who do not receive treatment are not likely to survive longer than 3 to
6 months.
Benign and Malignant Tumours

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Benign and Malignant Tumours

  • 1.
  • 2. REFERENCES  Odontogenic tumours & Allied lesions- Peter A Reichart , Hans P Philipson  Textbook of Oral Radiology- White & Pharoah (6th ed.)  Diagnostic imaging of jaws- Langland & Langlais  Differential Diagnosis of Oral & Maxillofacial lesions- Wood & Goaz (5th ed.)  Principles Practice Oral Radiologic Interpretation- H.M Worth  Ameloblastic fibroma: A review of published studies with special reference to its nature and biological behavior Yan Chen, Jing-Ming Wang, Tie-Jun Li doi:10.1016/j.oraloncology.2007.05.009  Martin-Granizo Lopez R, Ortega L, Gonzalez Corchon MA et al: Ameloblastic fibroma of the mandible. Report of two cases, Med Oral 8(2):150-153, 2003
  • 3. CONTENTS  Introduction  Definitions  Benign tumours - Clinical features - Radiographic features - Differential diagnosis - Treatment  Malignant tumours  Odontogenic  Non-odontogenic
  • 4. DEFINITIONS NEOPLASM : A neoplasm as defined by Willis is “ An abnormal mass of tissue, the growth of which exceeds and is uncoordinated with that of the normal tissues and persists in the same excessive manner after the cessation of the stimuli which evoked the change. ” BENIGN TUMOUR : Is a new growth, which is limited by a capsule and grows by local expansion without causing any harm to the host, excepting its position in a vital organ. MALIGNANT TUMOUR : Is a new growth which is characterized by rapid growth, sign of invasion, absence of capsule and last of all dissemination to other parts of the body usually by hematogenous or lymphatic route or both.
  • 5. FEATURES OF BENIGN TUMOR  Encapsulated  Slow growing  Painless  No metastasis
  • 7. ODONTOGENIC TUMOURS Classification Benign Tumors 1) Odontogenic epithelium with mature, fibrous stroma; odontogenic mesenchyme not present  Ameloblastomas  Calcifying epithelial odontogenic tumor  Adenomatoid odontogenic tumor  Squamous odontogenic tumour Editorial and Consensus Conference -Lyon, France (WHO/ IARC) in July 2003
  • 8. 2) Odontogenic epithelium with odontogenic mesenchyme with or without dental hard tissue formation  Ameloblastic fibroma  Ameloblastic fibro-dentinoma  Ameloblastic fibro-odontoma  Complex odontoma  Compound odontoma  Odontoameloblastoma  Calcifying cystic odontogenic tumor Editorial and Consensus Conference -Lyon, France (WHO/ IARC) in July 2003
  • 9. 3) Mesenchyme and/or odontogenic ectomesenchyme with or without included odontogenic epithelium  Odontogenic fibroma  Odontogenic myxoma or fibromyxoma  Cementoblastoma Editorial and Consensus Conference -Lyon, France (WHO/ IARC) in July 2003
  • 10. Malignant I Odontogenic Carcinomas  Metastasizing, malignant ameloblastoma  Ameloblastic carcinoma  Primary intraosseuos squamous cell carcinoma  Clear cell odontogenic carcinoma  Ghost cell odontogenic carcinoma II Odontogenic Sarcomas  Ameloblastic fibrosarcoma  Ameloblastic fibrodentino and fibro-odontosarcoma Editorial and Consensus Conference -Lyon, France (WHO/ IARC) in July 2003
  • 11. AMELOBLASTOMA WHO Definition - • Ameloblastoma is a polymorphic neoplasm consisting of proliferating odontogenic epithelium which usually has a follicular or plexiform pattern lying in a fibrous stroma Reichart and Slootweg Definition • Ameloblastoma is a polymorphous neoplasm consisting of proliferating odontogeni c epithelium usually occurring in follicular or plexiform patterns embedded in mature connective tissue stroma. As stated by Robinson, Ameloblastoma is usually unicentric, non-functional, intermittent in growth, anatomically benign and clinically persistent.
  • 12. HISTORICAL BACKGROUND  1826 – Guzack – a tumor of jaw  1868 – Broca – 1st neoplasm of this nature in literature  1879 – Falkson gave its thorough description.  1885 – Malassez – ‘Admantinoma’  1934 – Churchill – ‘Ameloblastoma’
  • 13. PATHOGENESIS  Resemblance to odontogenic apparatus – derived from the same.  Malassez, 1885 – epithelial rests adj. roots of teeth – ‘les debris épithéliaux’ - proliferate  Adamantine epithelioma  Origin – varied origin  Stimulus for initiation - unknown
  • 14. GENETIC ALTERATION Overexpressed genes  Proto-oncogene FOS – most highly -Fos protein – Activating protein-1 (transcription factor) -Cell proliferation, differentiation, oncogenic transformation -Bone development, regulator of osteoclast - macrophage lineage determination, bone remodelling.  TNFR 1A – induction of AP-1 activity -TNF-α – signal transduction -Inflammation, cell proliferation, differentiation & apoptosis Heikinheimo et al (2002, J Dent Res)
  • 15. Underexpressed genes –  Cadherins, KRT7, NOTCH & TGFB1  Disturbances in cell adhesion & cell-cell communication  Sonic hedgehog (SHH) – (expression of PTCH)  Dysregulation of signalling pathway for ectomecenchymal interactions Heikinheimo et al (2002, J Dent Res)
  • 16. VARIANTS  Classical solid / multicystic ameloblastoma (SMA)  Unicystic ameloblastoma (UA)  Peripheral ameloblastoma (PA)  Desmoplastic ameloblastoma (DA)
  • 17. SOLID / MULTICYSTIC AMELOBLASTOMA Clinical features-  Early stages – no clinical signs  Later –  Facial deformity – swelling (bony hard & bulky)  Loosening of teeth  Fractures  Pain – less frequent (pressure on nerves, sec. infection)  Large tumors – thinning of cortices (egg shell crackling)  Perforation – late feature Age – Mean age – 35.9 yrs (range 9- 92yrs) Maxillary tumours – 47 yrs Mandibular tumours – 35.2 yrs Sex distribution – Male : female – 1.1 : 1 Reichart et al 1995, biological profile of 3677 cases of ameloblastoma
  • 18. RADIOGRAPHIC FEATURES Honeycomb appearance Spider-like appearance Soap-bubble appearance Mother-daughter cell appearance
  • 20. ROOT RESORPTION PATTERN Knife edged root resorption Multiplanar root resorption
  • 21. RADIOGRAPHIC DESCRIPTION OF AMELOBLASTOMA WORTH (1963) 4 types  Unilocular  “Gross caricature of spider”  Multilocular  Replacement of normal bone with honeycomb appearance.
  • 23. CBCT
  • 25. HISTOPATHOLOGICAL VARIANTS Islands – Central polyhedral cells Surrounded by layer of cuboidal / Columnar cells Cystic degenerations – Within epithelial islands Follicular Plexiform Tumor epithelium in a network Cyst formation – within stroma Acanthomatous 0 Extensive squamous metaplasia 0 Keratin formation Granular 0 Granular transformation of central stellate cells – round/columnar/cuboidal 0 Acidophillic granules – lysosomal aggregates Basal
  • 26. PERIPHERAL AMELOBLASTOMA  Extraosseous, soft tissue ameloblastoma  Histology – similar to SMA  Tooth bearing region  Painless, sessile, firm,  Surface – smooth / pebbly, granular / papillary, warty  Pink / red / dark red
  • 27.  Duration – 2 days – 20 yrs  2 - 10% of ameloblastomas  R/F- cupping or saucerization  Resemblance to –  Epulis  Papilloma  Pyogenic granuloma
  • 28. UNICYSTIC AMELOBLASTOMA  ‘UNICYSTIC’ – macro & microscopic appearance – well defined, monocystic cavity – lined focally / entirely of ameloblastomatous epithelium. Synonyms –  Cystic ameloblastoma  Mural ameloblastoma
  • 29.  Age –  Dentigerous variant – younger (mean age – 16.5)  Non dentigerous – mean age – 32.2  Male : female –  Dentigerous - 1.6 : 1  Non-dentigerous – 1 : 1.8  Location – Mandible  Posterior mandible, Asc. Ramus  Dentigerous variety – 3rd molar
  • 30. HISTOLOGICAL VARIANTS (ACKERMAN ET AL, 1988 - SUBTYPES) 1. - Luminal UA 1.2 - Luminal & Intraluminal UA 1.2.3 - Luminal, Intraluminal & intramural 1.3 - Luminal & Intramural
  • 32. DESMOPLASTIC AMELOBLASTOMA  Variant of SMA – extensive stromal collagenization / desmoplasia  Benign, locally infiltrative  Location - Maxilla = mandible  Age – mean – 42.8 yrs  Male : female – 1 : 0.9  Origin – similar to SMA
  • 33. OTHER TYPES  Clear cell A  KeratoA & Papilliferous A  Mucus cell differentiation  Hemangiomatous Ameloblastoma  Extragnathic Adamantinoma  Metastasizing Ameloblastoma
  • 34. DIFFERENTIAL DIAGNOSIS  OKC  Odontogenic Myxoma  Aneurysmal bone cyst  Central gaint cell granuloma  Central hemangioma
  • 35. TREATMENT  Treatment depends on the type of lesion –  SMA – radical surgical intervention  UA – conservative surgical enucleation  Peripheral ameloblastoma – more conservative surgery  Radiotherapy –  1982 – Reynolds et al – effects of radiation on ameloblastoma – usefulness  Not used as 1st line of treatment
  • 36. PROGNOSIS  SMA –  ↑ Recurrence  Maxilla – spreads faster - ↑ morbidity & mortality - Posterior maxilla – poorest prognosis  Mandible – restricted by thick cortex  Follow up – 5-10 yrs post-surgery  DA – same rate of recurrence as SMA  Peripheral ameloblastoma – low recurrence (16-19%)  UA – 10.7%
  • 37. CALCIFYING EPITHELIAL ODONTOGENIC TUMOUR  Also called as Pindborg tumor, first described by J.J PINDBORG in 1956  Benign, odontogenic neoplasm exclusively epithelial in origin  1% of odontogenic tumors  ORIGIN – remnants of primitive dental lamina
  • 38. CLINICAL FEATURES  Age ; Sex – 4th decade ; Males  Site – Mandible : Maxilla = 2 :1  Malignant - Locally invasive with high recurrence rate
  • 39. RADIOGRAPHIC FEATURES  Expanded cortices in all dimensions  Radiolucent ; poorly defined, non- corticated borders  Unilocular, multilocular or “moth- eaten”  “ Driven-snow ” appearance from multiple radiopaque foci  Root divergence / resorption ; impacted tooth
  • 40.  Histology  Islands of eosinophilic epithelial cells  Cells infiltrate bony trabeculae  Nuclear hyperchromatism and pleomorphism  Psammoma-like calcifications (Liesegang rings)
  • 41. DIFFERENTIAL DIAGNOSIS  Dentigerous cyst  Adematoid odontogenic tumor  Ameloblastic fibro-odontoma
  • 42. TREATMENT  Behaves like ameloblastoma  Low recurrence rate  En bloc resection, hemimandibulectomy/partial maxillectomy.  Recurrence rate - Franklin & Pindborg (1976) : 14%
  • 43. ADENOMATOID ODONTOGENIC TUMOUR  Adenoameloblastoma , Ameloblastic adenomatoid tumor  First reported by - STAFNE  Term coined by PHILIPSEN & BIRN (1969)  ORIGIN – Dental epithelium  TYPES-  Peripheral  Central - Follicular & Extra-follicular
  • 44. CLINICAL FEATURES  Age - 2nd decade  Male : Female – 1 : 2  Slow growing expansile lesion  Location : Anterior maxilla  Associated with unerupted tooth
  • 45. RADIOGRAPHIC FEATURES  Well-corticated, non-scalloped margin.  Medial growth in maxilla  Snowflake, animal prints, foot prints  Radiolucent band
  • 46.
  • 47. HISTOLOGY Thick fibrous capsule, clusters of spindle cells, columnar cells (rosettes, ductal) throughout
  • 48. DIFFERENTIAL DIAGNOSIS  Dentigerous cyst  Ameloblastic fibroma  CEOT Treatment – enucleation, recurrence is rare Recurrence rate - Low : 0.2%
  • 49. SQUAMOUS ODONTOGENIC TUMOUR  Benign Epithelial Odontogenic Tumor  Hamartomatous proliferation  Origin CLINICAL FEATURES  2nd and 3rd decade  1:1  Tooth mobility  Tenderness on percussion
  • 51. HISTOLOGY  Oval nest of squamous epithelium in mature collagen stroma.
  • 52. DIFFERENTIAL DIAGNOSIS  Dentigerous & Radicular cyst  Treatment – Conservative enucleation & thorough curettage ; low recurrence rate.
  • 53. II. ODONTOGENIC EPITHELIUM WITH ODONTOGENIC MESENCHYME WITH OR WITHOUT DENTAL HARD TISSUE FORMATION
  • 54. AMELOBLASTIC FIBROMA  AF – True mixed odontogenic tumor  Consists of both epithelial and ectomesenchymal components are neoplastic.  1946, Thoma and Goldman were the first to classify this tumour as a separate entity
  • 55. CLINICAL FEATURES  Hard swelling  Mandibular posteriors  2nd and 3rd decade  Tumour size - 1 - 10 cm  Recurrence rate - 33.3%  Malignant transformation - 11.4% Yan Chen, Jing-Ming Wang, Tie-Jun Li . Ameloblastic fibroma : A review of published studies with special reference to its nature and biological behavior.
  • 56. RADIOGRAPHIC FEATURES Martin-Granizo Lopez R, Ortega L, Gonzalez Corchon MA et al: Ameloblastic fibroma of the mandible. Report of two cases, Med Oral 8(2):150-153, 2003.
  • 57. DIFFERENTIAL DIAGNOSIS  Dentigerous cyst  Ameloblastoma
  • 58. TREATMENT Conservative surgery  Excision  Enucleation  Curettage Radical surgery  Marginal resection  Segmental resection  Hemisection
  • 59. AMELOBLASTIC FIBRO-ODONTOMA  Hooker -1967  Ameloblastic fibroma + odontome  2% incidence- Regezi et al
  • 60. FEATURES C/F  83%- unerupted tooth  2nd decade of life  M : F - 1.4 : 1  Posterior mandible - 53.2% R/F-  Expansile, smooth- well defined cortication  Radiopaque flecks - 1 – 2 mm to 1cm  Tooth displacement
  • 62. TREATMENT  Conservative enucleation  Resection with removal of impacted teeth.
  • 63. ODONTOME  Benign Hamartoma  1971 - Pindborg et al. 2 types - Compound and complex  Pathogenesis  Local trauma  Infection  Family history  Genetic mutation
  • 64. CLINICAL FEATURES  Slow growing , expansile lesion  Compound – 2nd decade of life ; before 20yrs of age  Complex - 2nd decade of life ; before 30yrs of age  M : F - 1.5 : 1 Location –  Compound – anterior maxilla ; posterior mandible – 2 : 1  Complex – anterior maxilla ; posterior mandible – 1 : 2  Displacement of tooth
  • 66. VARIANTS  Cystic odontome  28% incidence  Amelobalstic odontome Treatment : Surgical enucleation Differential diagnosis  Ameloblastic fibro-odontome
  • 67. III. Mesenchyme or odontogenic ectomesenchyme with or without included odontogenic epithelium
  • 68. ODONTOGENIC MYXOMA  Benign non-encapsulated odontogenic neoplasm.  In 1947- Thoma and Goldman Pathogenesis  Arises from mesenchymal portion of the tooth germ, dental papilla, the follicle or the periodontal ligament.
  • 69. CLINICAL FEATURES  1-3%  2nd and 4th decade  M:F- 1:1.6  Mand posteriors
  • 70. RADIOGRAPHIC FEATURES Two pattern depending on evolution of tumor:  Osteoporotic appearance  Breakout/destructive phase
  • 72. DIFFERENTIAL DIAGNOSIS  Ameloblatoma  Central giant cell granuloma  Central hemangioma  Osteosarcoma  Treatment : As infiltrative local recurrence rate - 33%  Resection with 1-1.5 cm of bone
  • 73. ODONTOGENIC FIBROMA  Two variants can be distinguished: an intraosseous or central type (COF) and an extraosseous or peripheral type (POF)  Clinical variants- 1) hyperplastic, 2) simple, 3) WHO type  C/F - age- 3rd decade  M:F- 1:2.8  Maxilla : mandible – 1 : 6.8 Siar C H et al ,Clinicopathological study of peripheral and central odontogenic fibromas (WHO – type in Malaysians ( 1967 - 9 5 ). B r J Oral Maxillofac Su rg 2000 ;38: 19- 22 .
  • 75. BENIGN CEMENTOBLASTOMA  True cementoma, Attached cementoma  First reported by NORBERG (1930)  Term given by KRAMER  < 1% of all odontogenic tumors  Unique in two ways:  True neoplasm of cementoblasts  Attached routinely to an involved tooth  Origin - PDL
  • 76. CLINICAL FEATURES  2nd and 3rd decade  Mand 1st premolar and molar  M : F – 1 : 1.2  SYMPTOMS  Pain – 53% of cases  SIGN  Swelling-73% cases
  • 77. RADIOGRAPHIC FEATURES  Three radiologically distinct stages:  Uncalcified matrix stage  Calcified blastic stage  Mature stage
  • 79. DIFFERENTIAL DIAGNOSIS  Periapical cemental dysplasia  Enostosis  Hypercementosis  Sclerosing osteitis TREATMENT  Surgical extraction of involved tooth  Tumor can be amputated from tooth & tooth endodontically treated
  • 80.  Pattern of flecks on CEOT  According to histological criteria by Franklin and Pindborg, scattered flecks are seen with central radiolucency  Histopathologically, features of unilocular or multilocular ameloblastoma are similar.  Lichen planus of jaw – Odontogenic myxoma ; Traumatic bone cyst
  • 81. Metastasizing malignant ameloblastoma  Criteria for identifying metastatic diseases of the jaws – 1. Lesion must be a true metastasis localized to bone tissue, as distinguished from direct invasion by a primary tumour of contiguous structure 2. Lesion must be verified microscopically as carcinoma 3. Primary site of the lesion must be known  Ameloblastoma – metastasizes in spite of its benign histologic appearance.  Associated with hypercalcemia  Age – Third decade (5 - 74 yrs)  Gender – M : F = 1 : 1.2  Location : Posterior mandible
  • 82.  Clinical features –  Paresthesia / anesthesia  Loosening of teeth  Periodontal abscess  Facial paralysis  Pathologic fracture  Radiographic features  Frank destruction of bone without new bone formation within the lesion or adjacent bone.  Islands of bone with irregular margins
  • 83. Ameloblastic Carcinoma  Corio and associates – Any ameloblastoma in which there is histologic evidence of malignant disease in the primary or recurrent tumour, independent of the presence of metastasis.  Most ameloblastic carcinomas presumably arise de novo, and less than 1% of ameloblastomas undergo malignant transformation.  Ameloblastic carcinoma, secondary type, is defined as a malignantly transformed tumor within a pre-existing benign ameloblastoma, regardless of the presence of metastasis. Yukio Yoshioka, Shigeaki Toratani, Ikuko Ogawa and Tetsuji Okamoto. Ameloblastic Carcinoma, Secondary Type, of the Mandible: A Case Report. J Oral Maxillofac Surg 71:e58-e62, 2013.
  • 84. Clinical Features  Age – 4th decade  Female : Male - 1 : 2.7  Swelling (61.5%), bleeding, ulceration and fistula (15.4%).  Progressive types : cortical bone perforation, soft tissue invasion, recurrences and metastases.
  • 85. Radiographic Features  Unilocular or multilocular  Ragged borders  Root resorption  Cortical perforation
  • 86. Histopathology Kar, et al.:Ameloblastic carcinoma: A clinicopathologic dilemma – Report of two cases with total review of literature from 1984 to 2012. Annals of Maxillofacial Surgery | January - June 2014 | Volume 4 | Issue 1
  • 87. Differential Diagnosis  Metastatic carcinoma  Squamous odontogenic tumour
  • 88. TREATMENT  Radical surgery with neck dissection  Hemimandibulectomy / maxillectomy  Postive margins : adjuvant radiotherapy - 5 fractions of 1.8 Gy each for a period of 5 weeks (45 Gy).
  • 89. Primary Intraosseous Squamous Cell Carcinoma WHO defines:  “ Odontogenic carcinoma consisting of a squamous cell carcinoma arising within the jaws having no initial connection with the oral mucosa and presumably developing from residues of the odontogenic epithelium or from an odontogenic cyst or tumour”. Elzay 1982:  Arising from odontogenic cysts  Arising from ameloblastoma( malignant or AC)  De novo
  • 90. CLINICAL FEATURES  Age : 6th and 7th decade  M : F- 3 : 1  Location : Posterior mandible  Tooth mobility  Paraesthesia  Regional LN involvement  Exfoliation  Non-healing Extraction sockets (50- 60%) Mitsuyoshi Iino et al. Solid type primary intraosseous squamous cell carcinoma in the maxilla: report of a new case. Ear, Nose and Throat Disorders 2013, 13:13.
  • 91. Radiographic Features  Alveolar bone destruction  Cortical perforations  Pathologic fractures  “Floating tooth”  “ Bays within bays ” Huang et al.: Primary Intraosseous Squamous Cell Carcinoma. Arch Pathol Lab Med—Vol 133, November 2009
  • 93. Differential diagnosis  Gingival carcinomas  Ameloblastic carcinoma
  • 94. Treatment  Partial or total maxillectomy or mandibulectomy.  Radiotherapy - Fractionated in seven weeks each session of 50 Gy.  Adjuvant chemotherapy  Cisplatin 100 mg/m2 IV or 40-50 mg/m2 IV weekly for 6-7wk.  Cetuximab 400 mg/m2 IV loading dose 1wk before the start of radiation therapy, then 250 mg/m2 weekly (premedicate with diphenhydramine and ranitidine) .
  • 95. Clear cell odontogenic carcinoma  4th and 5th decade  Mandible : Maxilla- 7 : 1  M : F – 1 : 1.6  Mandibular posterior region  Poorly delineated radiolucency  Radical resection and follow up  Recurrence rate - 55% Niharika Swain, Richa Dhariwal et al. Clear cell odontogenic carcinoma of maxilla : A case report and mini review. J Oral Maxillofac Pathol. 2013 JanApr; 17(1): 89–94.
  • 96. Ghost cell odontogenic carcinoma  Gorlin 1962- “Malignant counterpart of calcifying odontogenic cyst”.  Age – 4th and 5th decade  Gender – M : F = 2.6 : 1  Location – Maxilla > Mandible  Clinical features  Swelling with or without pain  Osseous destruction with paresthesia
  • 97.  Radiographic features  Cortical destruction, obliteration of maxillary sinus  Ill-defined radiolucency  Opacification
  • 98. Ameloblastic Fibrosarcoma  It is a rare malignant neoplasm composed of benign , ameloblastomous epithelium and malignant ectomesenchyme.  Represents only 2% of all odontogenic tumors.  Clinical features  Pain and swelling  Ulceration and bleeding  Paresthesia of lower lip  2nd decade  Mandible : Maxilla = 2.3 : 1  M : F - 2 : 1
  • 99. Radiographic Features  Ill-defined radiolucency  Pathological fractures  Treatment  Wide local excision  Partial maxilla or mandibulectomy  Post surgical radiotherapy / chemotherapy  Recurrence rate - 20% Daniela Otero Pereira et al. Maxillary Ameloblastic Fibroma: A Case Report . Braz Dent J (2011) 22(2): 171-174
  • 100. Osteosarcoma  Osteosarcoma - Two types  An osteoblastic (sclerosing) and  An osteolytic type.  Gender : Males > Females  Age - 33 years (Mean)  Location : Mandible > Maxilla  Arise due to –  Radiation induced  Trauma
  • 101. Clinical Features  Pain, swelling, paraesthesia and ulceration.  Affected teeth may be loosened and displaced  Age- 4th & 5th decade  M : F - 1.1 : 1
  • 102. Radiographic Features  Earliest radiographic sign - widening of the periodontal ligament space or a radiolucency around one or more teeth.  Later on, the lesion assumes an osteolytic radiolucent form, an osteoblastic radiopaque form or a mixed radiolucent image with radiopaque foci.  Ill-defined borders.  Expansion and destruction of the cortical plates.  Characteristic sunray , sunburst or fan-shaped appearance - thin spicules of new bone extend outwards away from the bone cortex. Chittaranjan B, Tejasvi MA, Babu BB, Geetha P. Intramedullary Osteosarcoma of the Mandible: A Clinicoradiologic Perspective. J Clin Imaging Sci 2014;4, Suppl S1:6
  • 103. TREATMENT  The cornerstone of primary jaw osteosarcoma treatment is adequate surgical resection.  Radiotherapy or chemotherapy can be used in association with surgical resection or alone as a palliative treatment in advanced cases.  Literature review has shown that patients treated initially by aggressive local or even radical procedures such as hemimandibulectomy fared better.  Introduction of multi-agent chemotherapy has improved the survival rates, with 60-70% patients surviving after treatment.
  • 104. CHONDROSARCOMA  Malignant tumor of cartilaginous tissue  Age : 3rd to 5th decade.  Gender : Males > females.  Painless swelling leading to the expansion of the buccal and lingual cortical plates  Regional lymphadenopathy is very rare  Jaw lesions are rare.  The teeth adjacent to the lesion may be resorbed, loosened, or exfoliated. SanChita kundu et al. Clinicopathologic correlation of chondrosarcoma of mandible with a case report. Contemporary Clinical Dentistry | Oct-Dec 2011 | Vol 2| Issue 4.
  • 105.  Single/ multiple radiolucencies with poorly defined borders  Moth-eaten radiolucencies to diffusely opaque lesions.  Localized widening of the periodontal ligament space may be observed.  In some cases, a sunray appearance may be seen.
  • 106. TREATMENT  Wide surgical excision is the mainstay of treatment for  These tumors are radioresistant and chemotherapy can be used as an adjuvant therapy after wide surgical excision is made  Prognosis is poor.  Local recurrence occurs and may indicate subsequent metastasis; hence adequate treatment and long-term follow-up, including periodic systemic evaluation, are required.
  • 107. EWING’S SARCOMA  Malignant tumor of bone derived from mesenchymal connective tissue of the bone marrow.  Rarely occurs in the jaws  Rapidly growing, highly invasive tumor  Pain and swelling are the most common manifestations.  Age : Children ; young adults  Gender : M : F = 2 : 1
  • 108.  The radiographic appearance is that of an ill-defined destructive radiolucent lesion which may be unilocular or multilocular.  Stimulates the periosteum to produce thin layers of bone, resulting in an "onion skin" effect.  Advanced cases may exhibit a sunburst appearance.
  • 109.  Chemotherapy, radiation therapy and surgery, has led to an improvement in prognosis.  Poor prognostic factors are patients below 10 years of age, presence of metastasis, presence of systemic symptoms, large tumor volume.  ES of the mandible has got better prognosis than long bones since facial sites are diagnosed earlier. TREATMENT
  • 110. BURKITT'S LYMPHOMA  Characteristic form of non-Hodgkin's lymphoma that is endemic in Africa and occurs sporadically in North America.  The Epstein-Barr virus has been implicated in the etiology.  Age : Peak : 5 years  Primarily a tumor of childhood, with occasional cases seen in young adults.  Location : Maxilla > Mandible.  Gender : M : F = 2 : 1 Vasudevan V, Mohandas U, Manjunath V. Burkitt's lymphoma in leukemic phase in an Indian boy. Indian J Dent Res 2011;22:340-4
  • 111.  Clinical features –  Loosening of teeth  Bilateral jaw tenderness  Swollen gingiva  Cervical lymphadenopathy  Radiographic features  Ill defined radiolucency extending into tooth crypt  Displacement of developing and erupting teeth  Floating in air appearance  Sunray spicules
  • 112. TREATMENT  Good response to chemotherapy, particularly cyclophosphamide.  The tumor also has been shown to be sensitive to methotrexate, vincristine, and cytarabine.  Combinations of drugs have achieved remissions in more than 90% of patients. Unfortunately, most of the cases recur and patients ultimately succumb to the disease.  Recent clinical trials with intensive, multiagent chemotherapeutic protocols have shown a fair remission rate.  Individuals who do not receive treatment are not likely to survive longer than 3 to 6 months.

Editor's Notes

  1. Significant of all OT – high incidence, clinical behavior & high recurrences
  2. Amelogenin gene –Tsujigiwa et al (2005, Oral Oncology)
  3. Septae are usually coarse and curved, may be remodelled. Numerous small locules. Also seen are a few larger ones. Root resorption & displacement On a tangential view – margin of the lesion meets the buccal cortex – acute angle – tumor like manner of expansion Unilocular
  4. This resembles a dentigerous cyst – varied thickness of expansion of buccal cortex – more in favor of a tumor
  5. Coronal CT in a bone window – expansile lesion of the rt ramus of mandible – thinning & perforation in areas
  6. MRI – correlates well with histopathology – can diff solid & cystic areas (better than CT). Varied high to low signal intensity on MRI On a T2WI mr – hetrogenous high signal intensity noted in the body of mand. An area of very high signal intensity – cystic areas On a Gd – T1WI mr – enhancement of the lesion (indicating solid nature of lesion) also there is an area of low signal intensty with no enhancement – cystic space.
  7. Currently, t here is general agreement that the PA and t he BCC are es­sentially the same lesion and thus should be regarded as a single entity
  8. Shows mixed radiolucent-radiopaque appearance similar to fibro-osseous lesions
  9. Aneurysmal bone cyst Tooth displacement > tooth resorption. Fluid levels – CT. < 20yrs, Female Unilocular, multilocular (thin wispy trabeculae). Rapid growth & expansion Displacement of tooth. Multiplanar root resorption
  10. Hamartomatous proliferation. Maxillary incisor-canine and mandibular molar. Tooth mobility common complaint Radiology – triangular, localized radiolucency between contiguous teeth. Histology – oval nest of squamous epithelium in mature collagen stroma Treatment – extraction of involved tooth and thorough curettage; maxillary – more extensive resection; recurrences – treat with aggressive resection
  11. This fibroma is often associated with an impacted tooth.
  12. No root resorption, no hydraulic effect
  13. Thus radiographically, it appears as a combined radiolucent/radiopaque lesion and must be differentiated from other similar-appearing lesions such as the ossifying fibroma, the calcifying epithelial odontogenic tumor, the calcifying odontogenic cyst, an odontoma, and an adenomatoid odontogenic tumor.
  14. washer like appearance
  15. Mild expansion- odontome Large expansion- cystic odontome or ameloblastic odontome
  16. 48% unerupted tooth
  17. Tennis racket- osteoporotic Sun burst- break out
  18. Among all the reviewed cases, 27% presented at first histopathologic Diagnosis as a follicular Or Plexiform Ameloblastoma And Secondarily as an ameloblastic carcinoma
  19. Bays within bays- margins with large eroded areas within which smaller areas of bone destruction are present
  20. The degree of malignancy of the mesenchymal component is decided by the cellularity, palisading pattern and cellular atypia (mitosis, shape and size).
  21.  Osteosarcoma of jaw bones have some distinct features such as older age at presentation, longer median survival, rare metastases and local recurrences difficult to control, typically leading to death of the patients