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.
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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
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)
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
21. RADIOGRAPHIC DESCRIPTION OF AMELOBLASTOMA
WORTH (1963)
4 types
Unilocular
“Gross caricature of spider”
Multilocular
Replacement of normal bone with honeycomb appearance.
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
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.
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
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.
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.
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
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
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
Significant of all OT – high incidence, clinical behavior & high recurrences
Amelogenin gene –Tsujigiwa et al (2005, Oral Oncology)
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
This resembles a dentigerous cyst – varied thickness of expansion of buccal cortex – more in favor of a tumor
Coronal CT in a bone window – expansile lesion of the rt ramus of mandible – thinning & perforation in areas
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.
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
Shows mixed radiolucent-radiopaque appearance similar to fibro-osseous lesions
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
This fibroma is often associated with an impacted tooth.
No root resorption, no hydraulic effect
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.
washer like appearance
Mild expansion- odontome
Large expansion- cystic odontome or ameloblastic odontome
48% unerupted tooth
Tennis racket- osteoporotic
Sun burst- break out
Among all the reviewed cases, 27% presented at first histopathologic Diagnosis as a follicular Or Plexiform Ameloblastoma And Secondarily as an ameloblastic carcinoma
Bays within bays- margins with large eroded areas within which smaller areas of bone destruction are present
The degree of malignancy of the mesenchymal component is decided by the cellularity, palisading pattern and cellular atypia (mitosis, shape and size).
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