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Dr. Vibhuti Kaul
The Origin and Location of Bone Cells
Bone Remodelling
Four phases of remodelling:
1. Activation of Osteoclasts
2. Resorption of Bone
3. Reversal Phase
4. Forma...
Jaw Bone Lesions
Related to
Dentition
Bone
Proper
Related to
Dentition
Cysts
Odontogenic
Tumors
“It is an epithelium-lined sac filled with fluid or semi-fluid
material.” (Killey and Kay, 1966)
“A cyst is a pathologic c...
Classification of cysts by Shear
EPITHELIAL
Odontogenic
Developmental
 Dentigerous Cyst (follicular)
 Eruption cyst
 Pr...
Non-Odontogenic
 Nasopalatine duct (incisive canal) cyst
 Median palatine, median alveolar and median mandibular
cysts
...
DENTIGEROUS CYST
 Also called as “follicular” or “pericoronal” cyst
 Surround the crown of an unerupted tooth
 Usually ...
ODONTOGENIC KERATOCYST
 OKCs possess destructive potential, with a high
recurrence rate after resection
 develop from th...
Figure 5. OKCs in a 22-year-old man.
Dunfee B L et al. Radiographics 2006;26:1751-1768
©2006 by Radiological Society of No...
• Multiple OKCs in a young patient should raise the possibility of basal
cell nevus syndrome (Gorlin-Goltz syndrome).
• As...
PRIMORDIAL CYST
 An odontogenic cyst developing from the stellate
reticulum.
 Formed in place of the tooth i.e. the toot...
LATERAL PERIODONTAL CYST
 Located between the roots of vital teeth
 Lined by a thin, non-keratinizing squamous/cuboidal
...
CALCIFYING EPITHELIAL
ODONTOGENIC CYST
 1st described by Gorlin in 1962
 Unusual and unique lesion with characteristics ...
GLANDULAR ODONTOGENIC CYST
 Also called as “Sialo odontogenic cyst” or
“mucoepidermoid cyst”
 Has both secretory element...
RADICULAR, RESIDUAL and
PARADENTAL CYST
 Radicular cysts are located at the root tips of teeth with
necrotic pulp tissue....
NASOPALATINE DUCT (INCISIVE
CANAL) CYST
 Derived from the epithelial remnants of the naso-
palatine duct.
 Lies in the a...
MEDIAN PALATINE CYST
GLOBULOMAXILLARY CYST
 First described by Thoma
 Fissural cyst
 Formed at the junction of the globular portion of the
m...
SIMPLE BONE CYST
 Also known as “Traumatic bone cyst”, “Hemorrhagic
cyst”, “Intraosseous hematoma”, “Idiopathic bone cyst...
ANEURYSMAL BONE CYST
 Pseudocyst first described by Jaffe and Lichtenstein in
1942
 Current opinion is that it is an exa...
STAFNE’S BONE CYST
 Also known as “Static bone cyst”, “Lingual mandibular
bone cavity”
 Stafne bone cyst of the mandible...
Odontogenic tumors (modified
WHO classification)
A. Benign
I. Odontogenic epithelium without odontogenic
ectomesenchyme
1....
II. Odontogenic epithelium with odontogenic
ectomesenchyme with or without hard tissue
formation
1. Ameloblastic fibroma
2...
III. Odontogenic ectomesenchyme with or without
included odontogenic epithelium
1. Odontogenic fibroma
2. Myxoma (myxofibr...
B. Malignant
I. Odontogenic carcinomas
1. Malignant ameloblastoma
2. Primary intraosseous carcinoma
3. Clear cell odontoge...
AMELOBLASTOMA
 The ameloblastoma is the most common clinically
significant
 It may develop from cell rests of the enamel...
 Ameloblastomas occur in 3 different clinico-
radiographic situations requiring different therapeutic
considerations and ...
SQUAMOUS ODONTOGENIC
TUMOR
 SOTs occur with about equal frequency in maxilla and
mandible. They are more common in the an...
CALCIFYING EPITHELIAL ODONTOGENIC
TUMOR (PINDBORG TUMOR)
 Pindborg tumor accounts for < 1 % of all odontogenic
tumors.
 ...
ADENOMATOID ODONTOGENIC
TUMOR
 Formerly called an adenoameloblastoma, a somewhat
deceptive term that should be discarded,...
AMELOBLASTIC FIBROMA
 This true mixed odontogenic tumor is more common
in patients in the first and second decades of lif...
AMELOBLASTIC FIBRO-ODONTOMA
 This lesion is defined as a tumor with general features
of an ameloblastic fibroma but conta...
ODONTOAMELOBLASTOMA
 Extremely rare tumor, thus there is little reliable
information.
 Patient Age: Has been seen in you...
ODONTOMA
 The odontoma is the most common odontogenic
tumor.
 It is not a true neoplasm but rather is considered
to be a...
ODONTOGENIC FIBROMA
 Fewer than 70 cases have been reported in the English
literature.
 Sixty percent occur in the maxil...
ODONTOGENIC MYXOMA
 Slow-growing expansile lesion, often associated with
missing or impacted teeth.
 Prominent mucoid in...
CEMENTOBLASTOMA
 The cementoblastoma is a slow-growing lesion that may
cause local expansion of the jaw.
 Patient Age: T...
MALIGNANT AMELOBLASTOMA
 Less than 1 % of the ameloblastomas show malignant
behavior with the development of metastases.
...
CLEAR CELL ODONTOGENIC
CARCINOMA
 First reported in 1985 with few cases thus far.
 Tumor appears to be of odontogenic or...
GHOST CELL ODONTOGENIC
CARCINOMA
 “Is the Gorlin cyst a cyst or is it a tumor that is
frequently cystic?”
 Fejerskov and...
 Currently, WHO classifies the lesion as a benign tumor
and calls it calcifying cystic odontogenic tumor. This is
now def...
Ameloblastic fibrosarcoma
 This lesion is considered the malignant counterpart of
the ameloblastic fibroma in which the m...
Reactive
Bone
Diseases
Fibro-
osseous
lesions
Giant
cell
lesions
Bone
tumors
Others
REACTIVE BONE
LESIONS
TORI
MAXILLARY MANDIBULAR
INFLAMMATORY
DISEASES
(OSTEOMYELITIS)
NECROSIS SCLEROSIS
TORUS PALATINUS
TORUS MANDIBULARIS
OSTEOMYELITIS
Figure 22a. Acute suppurative osteomyelitis in a 44-year-old woman.
Dunfee B L et al. Radiographics 2006;26:1751-1768
©200...
Figure 23. Chronic osteomyelitis in a 47-year-old man.
Dunfee B L et al. Radiographics 2006;26:1751-1768
©2006 by Radiolog...
Figure 24. Sclerosing osteomyelitis in a 10-year-old boy.
Dunfee B L et al. Radiographics 2006;26:1751-1768
©2006 by Radio...
Fibro-osseous
lesions
Fibrous
Dysplasia
Monostotic
Polyostotic
Albright’s Syndrome
Osseous
Dysplasia
Periapical
Focal
Flor...
FIBRO-OSSEOUS LESIONS
Classification by Waldron CA:
1. Fibrous dysplasia
2. Reactive
a. Periapical cemento-osseous dysplas...
FIBROUS DYSPLASIA
 Asymptomatic, self-limiting developmental regional alteration
of bone in which the normal architecture...
MONOSTOTIC FIBROUS DYSPLASIA
• 80 – 85% of cases; Jaws most commonly affected
• Painless enlargement of the affected bone
...
POLYOSTOTIC FIBROUS DYSPLASIA
 McCune Albright Syndrome: Associated with skin
pigmentation and endocrine dysfunction
 Mu...
CEMENTO-OSSEOUS DYSPLASIA
• Most common fibro-osseous lesion of the jaws
• Occurs in tooth bearing areas
• 3 types: focal;...
Focal Cemento-osseous dysplasia
• Single site involvement
• 90% of cases occur in females
• 3-6th decade
• More common in ...
Florid cemento-osseous dysplasia
• Multifocal involvement
• Both anterior and posterior mandible
• Middle-aged African-Ame...
OSSIFYING FIBROMA
 Montgomery, 1927, first used the term.
 Composed of fibrous tissue that contains woven as
well as lam...
JUVENILE TRABECULAR OSSIFYING
FIBROMA
 Shows bands of cellular osteoid together with slender
trabeculae of plexiform bone...
JUVENILE PSAMMOMATOID
OSSIFYING FIBROMA
 Characterized by small ossicles resembling
psammoma bodies, hence its name.
 Th...
CGCG
Cherubism
CENTRAL GIANT CELL GRANULOMA
• Intraosseous destructive lesions of the jaws
• Far less common than peripheral giant cell
g...
Central giant cell granuloma in a 34-year-old man. CT scan (bone windowing)
demonstrates a cystic lesion (arrows) within t...
Central giant cell granuloma in a 34-year-old man.
Photograph of the gross resected specimen shows multiple
cystic cavitie...
CHERUBISM
• Autosomal dominant
• Facial appearance similar to “cherub”-like
• 2 – 5 yrs of age
• The clinical alterations ...
METASTATIC CARCINOMA
• Most common form of cancer involving bone
• Breast and prostate carcinomas are most common
• Lung a...
PRIMARY INTRA-OSSEOUS
CARCINOMA
 Squamous cell carcinoma arising within the jawbones
which has no initial connection with...
MULTIPLE MYELOMA
 Rare malignant plasma-cell disorder.
 The malignancy is more common in men over 50 years
of age, and t...
OSTEOSARCOMA
• Malignancy of mesenchymal cells that produce osteoid or
immature bone
• Can arise following radiation - * I...
CHONDROSARCOMA
• Half as common as osteosarcomas
• 1% to 3% arise in head and neck area
• Extragnathic lesions: older pati...
EWING’S SARCOMA
 Described under small round cell tumors.
 Occurs predominantly in children & young adults
between 5-25 ...
LEUKEMIA
 Destructive lesions of bone are reported in some cases
of chronic leukemia, and these may result in
pathologic ...
PRIMARY LYMPHOMA OF BONE
 Rare malignant neoplastic disorder of skeleton.
 Described as distinct clinical condition by P...
BURKITT’S LYMPHOMA
 High-grade malignant B-lymphocyte lymphoma.
 Epstein–Barr virus is closely associated.
 The maligna...
METABOLIC DISORDERS
Eg.:
 Osteoporosis
 Osteomalacia
 Renal dystrophy
PAGET’S DISEASE (OSTEITIS
DEFORMANS)
 Uncoordinated increase in bone turnover (osteoblastic and
osteoclastic activity) pr...
LANGERHAN’S CELL HISTIOCYTOSIS
Rare disease involving clonal proliferation of Langerhans
cell. Manifestations range from i...
EOSINOPHILIC GRANULOMA
 Slowly-progressing disease
 Can be monostotic or polyostotic
 No extra-skeletal involvement
HAND-SCHüLLER-CHRISTIAN DISEASE
 Seen mostly in children
 Characterized by fever, bone lesions & diffuse euptions
usuall...
LETTERER-SIWE DISEASE
 Rapidly progressing disease in which Langerhans cells
proliferate in many tissues
 Mostly seen in...
OSTEOPETROSIS
 Also known as “Marble bone disease”, “Albers-Schönberg
disease”, “osteosclerosis fragilis generalisata”
 ...
OSTEORADIONECROSIS
 Radiation-induced pathologic process characterized
by chronic & painful infection & necrosis accompan...
OSTEOGENESIS IMPERFECTA
ACHONDROPLASIA
 Autosomal dominant disorder that is the most
common cause of short limb dwarfism
 As the skull base form...
HAIR-ON-END SIGN
 Red marrow hyperplasia causes widening of the
diploic space, and the outer table thins or is completely...
ARTERIO-VENOUS MALFORMATIONS
 Abnormal, direct communications between arteries
and veins.
 Rare, may occur within the ra...
AVM in a 28-year-old man. (a) Contrast-enhanced CT scan reveals multiple
dilated and tortuous vessels (arrow) within the r...
PSEUDOTUMOR OF HEMOPHILIA
 First described in 1918 by Starker
 Pseudotumors are found almost exclusively in men
between ...
MELANOTIC NEUROECTODERMAL
TUMOR OF INFANCY
 Rare benign tumor of neural crest origin that was first
described by Krompech...
TRAUMATIC NEUROMA
 Rare disorder that occurs after trauma or surgery &
involves the peripheral nerve.
 Exaggerated respo...
SCHWANNOMA
 Neurilemmoma, a benign neoplasm derived from
Schwann cells was first described by Verocay in 1910.
 The intr...
INTRAOSSEOUS LIPOMA
 Benign tumor, 1% of all lipomas
References:
1. Burket’s Oral Medicine, 8th Ed
2. Burket’s Oral Medicine, 11th Ed
3. Textbook Of Oral Medicine, Arvind Rao ...
7. Cherubism- a case report with review, Indian Journal of Dentistry, Vol. 2/Issue 3, Pages 44-47, April-June 2011
8. A la...
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Jaw bone lesions

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An overview of various pathological processes affecting the Jaw Bones- Maxilla and Mandible including odontogenic cysts and tumours including their radiological findings!

Published in: Health & Medicine
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Jaw bone lesions

  1. 1. Dr. Vibhuti Kaul
  2. 2. The Origin and Location of Bone Cells
  3. 3. Bone Remodelling Four phases of remodelling: 1. Activation of Osteoclasts 2. Resorption of Bone 3. Reversal Phase 4. Formation of Bone Activation of Osteoblasts Mineralization
  4. 4. Jaw Bone Lesions Related to Dentition Bone Proper
  5. 5. Related to Dentition Cysts Odontogenic Tumors
  6. 6. “It is an epithelium-lined sac filled with fluid or semi-fluid material.” (Killey and Kay, 1966) “A cyst is a pathologic cavity having fluid, semifluid, or gaseous contents that are not created by the accumulation of pus; frequently, but not always, it is lined by epithelium.” (Kramer, 1974)
  7. 7. Classification of cysts by Shear EPITHELIAL Odontogenic Developmental  Dentigerous Cyst (follicular)  Eruption cyst  Primordial cyst  Gingival cyst of adults  Lateral Periodontal cyst  Calcifying Odontogenic cyst Inflammatory  Radicular cyst  Residual cyst  Inflammatory collateral cyst  Paradental cyst
  8. 8. Non-Odontogenic  Nasopalatine duct (incisive canal) cyst  Median palatine, median alveolar and median mandibular cysts  Globulomaxillary cyst  Nasolabial cyst NON-EPITHELIAL  Simple bone cyst  Aneurysmal bone cyst CYSTS ASSOCIATED WITH MAXILLARY ANTRUM  Benign mucosal cyst of the maxillary antrum  Surgical ciliated cyst of maxilla CYSTS OF THE SOFT TISSUE OF THE MOUTH, FACE & NECK
  9. 9. DENTIGEROUS CYST  Also called as “follicular” or “pericoronal” cyst  Surround the crown of an unerupted tooth  Usually mandibular 3rd molar and maxillary canine  Cyst wall is attached at the neck of the tooth  Origin is from reduced enamel epithelium, epithelial lining consists of 2-3 layers of cuboidal cells
  10. 10. ODONTOGENIC KERATOCYST  OKCs possess destructive potential, with a high recurrence rate after resection  develop from the dental lamina, which is found throughout the jaw and overlying alveolar mucosa and is lined by stratified keratinizing squamous epithelium  can expand cortical bone and erode the cortex  The lesion is multiloculated, often with daughter cysts that extend to the surrounding bone  Now known as “Keratocystic Odontogenic Tumor”
  11. 11. Figure 5. OKCs in a 22-year-old man. Dunfee B L et al. Radiographics 2006;26:1751-1768 ©2006 by Radiological Society of North America
  12. 12. • Multiple OKCs in a young patient should raise the possibility of basal cell nevus syndrome (Gorlin-Goltz syndrome). • Associated findings with this autosomal dominant disorder include midface hypoplasia, frontal bossing and prognathism, mental retardation, and calcification of the falx cerebri.
  13. 13. PRIMORDIAL CYST  An odontogenic cyst developing from the stellate reticulum.  Formed in place of the tooth i.e. the tooth is missing.
  14. 14. LATERAL PERIODONTAL CYST  Located between the roots of vital teeth  Lined by a thin, non-keratinizing squamous/cuboidal epithelium with focal, plaque-like thickenings that consist of clear cells.  Multilocular variety called as “Botryoid Odontogenic cyst”, term proposed by Waldron.
  15. 15. CALCIFYING EPITHELIAL ODONTOGENIC CYST  1st described by Gorlin in 1962  Unusual and unique lesion with characteristics of a solid neoplasm and a cyst  Also known as “Keratinizing and/or calcifying epithelial odontogenic cyst”, “Gorlin cyst”, “cystic keratinizing tumor”  The neoplasm is more aptly termed as “dentinogenic ghost cell tumor”
  16. 16. GLANDULAR ODONTOGENIC CYST  Also called as “Sialo odontogenic cyst” or “mucoepidermoid cyst”  Has both secretory elements and stratified squamous epithelium. (Sadeghi et al., 1991)  Slight predilection for mandibular anterior region.  Recurrence is common after treatment.
  17. 17. RADICULAR, RESIDUAL and PARADENTAL CYST  Radicular cysts are located at the root tips of teeth with necrotic pulp tissue.  Origin from cell rests of Malassez.  Radicular cyst left behind after removal of the associated tooth is called residual cyst.  Similar cyst located at the lateral side of the tooth at the border between enamel and root cementum are called paradental.
  18. 18. NASOPALATINE DUCT (INCISIVE CANAL) CYST  Derived from the epithelial remnants of the naso- palatine duct.  Lies in the anterior palate just behind the central incisor teeth.
  19. 19. MEDIAN PALATINE CYST
  20. 20. GLOBULOMAXILLARY CYST  First described by Thoma  Fissural cyst  Formed at the junction of the globular portion of the medial nasal process and the maxillary process
  21. 21. SIMPLE BONE CYST  Also known as “Traumatic bone cyst”, “Hemorrhagic cyst”, “Intraosseous hematoma”, “Idiopathic bone cyst”, “Extravasation bone cyst”  Not a true cyst as lacks lining.  Probable etiology is trauma.
  22. 22. ANEURYSMAL BONE CYST  Pseudocyst first described by Jaffe and Lichtenstein in 1942  Current opinion is that it is an exaggerated localized proliferative response of vascular tissue  Contains giant cells which represent an attempt at repair of a hematoma of bone  Biesecker and his associates postulated etiology being secondary to primary lesion of bone
  23. 23. STAFNE’S BONE CYST  Also known as “Static bone cyst”, “Lingual mandibular bone cavity”  Stafne bone cyst of the mandible is the only described destructive bone lesion that is highly localized, nonprogressive, but nonhealing.  An indentation on the lingual surface of the mandible within which a portion of the submandibular gland lies.
  24. 24. Odontogenic tumors (modified WHO classification) A. Benign I. Odontogenic epithelium without odontogenic ectomesenchyme 1. Ameloblastoma 2. Squamous odontogenic tumor 3. Calcifying epithelial odontogenic tumor 4. Adenomatoid odontogenic tumor
  25. 25. II. Odontogenic epithelium with odontogenic ectomesenchyme with or without hard tissue formation 1. Ameloblastic fibroma 2. Ameloblastic fibrodentinoma 3. Ameloblastic fibro-odontoma 4. Odontoameloblastoma 5. Calcifying odontogenic cyst 6. Complex odontoma 7. Compound odontoma
  26. 26. III. Odontogenic ectomesenchyme with or without included odontogenic epithelium 1. Odontogenic fibroma 2. Myxoma (myxofibroma) 3. Cementoblastoma (benign cementoblastoma, true cementoma)
  27. 27. B. Malignant I. Odontogenic carcinomas 1. Malignant ameloblastoma 2. Primary intraosseous carcinoma 3. Clear cell odontogenic carcinoma 4. Ghost cell odontogenic carcinoma II. Odontogenic sarcomas 1. Ameloblastic fibrosarcoma 2. Ameloblastic fibrodentinosarcoma 3. Ameloblastic fibro-odontosarcoma
  28. 28. AMELOBLASTOMA  The ameloblastoma is the most common clinically significant  It may develop from cell rests of the enamel organ; from the developing enamel organ; from the lining of odontogenic cysts or from the basal cells of the oral mucosa.  It is typically slow-growing, locally invasive and runs a benign course.  H.G.B. Robinson described it as being a benign tumor that is “usually unicentric, non-functional, intermittent in growth, anatomically benign and clinically persistent.”
  29. 29.  Ameloblastomas occur in 3 different clinico- radiographic situations requiring different therapeutic considerations and having different prognoses.  Conventional Solid/Multicystic (86 % of all cases)  Unicystic (13 % of all cases)  Peripheral or Extraosseous (1 % of all cases)  Several microscopic subtypes: follicular, plexiform, acanthomatous, granular, desmoplastic, basaloid
  30. 30. SQUAMOUS ODONTOGENIC TUMOR  SOTs occur with about equal frequency in maxilla and mandible. They are more common in the anterior regions of the jaws than in the posterior. The lesions occur in the alveolar process.
  31. 31. CALCIFYING EPITHELIAL ODONTOGENIC TUMOR (PINDBORG TUMOR)  Pindborg tumor accounts for < 1 % of all odontogenic tumors.  It is clearly of odontogenic origin but its histogenesis is uncertain.  The tumor cells are said to resemble cells of the stratum intermedium.
  32. 32. ADENOMATOID ODONTOGENIC TUMOR  Formerly called an adenoameloblastoma, a somewhat deceptive term that should be discarded, the AOT represents about 3-7 % of all odontogenic tumors.  This epithelial tumor has an inductive effect on the odontogenic ectomesenchyme with dentinoid frequently being produced.
  33. 33. AMELOBLASTIC FIBROMA  This true mixed odontogenic tumor is more common in patients in the first and second decades of life with a mean of 14 years.  It is slightly more common in males than females.  Approximately 70 % of the ameloblastic fibromas occur in the posterior mandible.
  34. 34. AMELOBLASTIC FIBRO-ODONTOMA  This lesion is defined as a tumor with general features of an ameloblastic fibroma but containing enamel and dentin.  Some investigators believe that this entity is but a stage in the development of an odontoma; however, most agree that progressive destructive tumors are true neoplasms.
  35. 35. ODONTOAMELOBLASTOMA  Extremely rare tumor, thus there is little reliable information.  Patient Age: Has been seen in younger patients.  Gender Predilection: Unknown.  Location: Most cases have been in mandible.  Radiographic Appearance: Lesion is a mixed radiolucent-radiopaque, ill-defined one.
  36. 36. ODONTOMA  The odontoma is the most common odontogenic tumor.  It is not a true neoplasm but rather is considered to be a developmental anomaly (hamartoma).  Two types of odontomas are recognized:  Compound: this type of odontoma is composed of multiple small tooth-like structures.  Complex: this lesion is composed of a conglomerate mass of enamel and dentin, which bears no anatomic resemblance to a tooth.  The compound type is more often in the anterior maxilla while the complex type occurs more often in the posterior regions of either jaw.
  37. 37. ODONTOGENIC FIBROMA  Fewer than 70 cases have been reported in the English literature.  Sixty percent occur in the maxilla where most are located anterior to the first molar. When in the mandible, approximately 50 % occur in the posterior jaw.
  38. 38. ODONTOGENIC MYXOMA  Slow-growing expansile lesion, often associated with missing or impacted teeth.  Prominent mucoid intercellular substance is present.  Hyaluronic acid and chondroitin sulfate are produced by this lesion.  Although benign, behaves aggressively.
  39. 39. CEMENTOBLASTOMA  The cementoblastoma is a slow-growing lesion that may cause local expansion of the jaw.  Patient Age: This lesion is most commonly occurs in the second and third decades.  Gender Predilection: Approximately equal.  Location: The cementoblastoma is associated with the roots of posterior teeth and is more common in the mandible than the maxilla.
  40. 40. MALIGNANT AMELOBLASTOMA  Less than 1 % of the ameloblastomas show malignant behavior with the development of metastases.  Malignant ameloblastoma is a tumor that shows histologic features of the typical (benign) ameloblastoma in both the primary and secondary deposits.  Ameloblastic carcinoma is a tumor that shows cytologic features of malignancy in the primary tumor, in recurrence and any metastases.  Metastases most often occur in the lungs
  41. 41. CLEAR CELL ODONTOGENIC CARCINOMA  First reported in 1985 with few cases thus far.  Tumor appears to be of odontogenic origin but its histogenesis is uncertain.  The term carcinoma is used because most cases have demonstrated aggressive behavior with invasion of contiguous structures.  The clear cells contain small amounts of glycogen.
  42. 42. GHOST CELL ODONTOGENIC CARCINOMA  “Is the Gorlin cyst a cyst or is it a tumor that is frequently cystic?”  Fejerskov and Krogh suggested the term ‘calcifying ghost cell odontogenic tumor’  Freedman and his associates suggested the name ‘cystic calcifying odontogenic tumor’  The calcifying odontogenic cyst can be classified mainly into two type: 1. Cystic lesion 2. Solid neoplastic lesion
  43. 43.  Currently, WHO classifies the lesion as a benign tumor and calls it calcifying cystic odontogenic tumor. This is now defined as “a benign cystic neoplasm of odontogenic origin, characterized by an ameloblastoma-like epithelium with ghost cells that may calcify”.  Dr. Gorlin’s recent paper classifies the lesion into four types. • Type 1. Simple cystic CCOT. Includes pigmented and clear cell variants. • Type 2. Odontoma-associated CCOT. • Type 3. Ameloblastomatous proliferating CCOT. • Type 4. CCOT associated with benign odontogenic tumors other than odontoma.
  44. 44. Ameloblastic fibrosarcoma  This lesion is considered the malignant counterpart of the ameloblastic fibroma in which the mesenchymal portion shows features of malignancy.  The ameloblastic fibrosarcoma may arise de novo or there may be a malignant transformation of an ameloblastic fibroma.
  45. 45. Reactive Bone Diseases Fibro- osseous lesions Giant cell lesions Bone tumors Others
  46. 46. REACTIVE BONE LESIONS TORI MAXILLARY MANDIBULAR INFLAMMATORY DISEASES (OSTEOMYELITIS) NECROSIS SCLEROSIS
  47. 47. TORUS PALATINUS
  48. 48. TORUS MANDIBULARIS
  49. 49. OSTEOMYELITIS
  50. 50. Figure 22a. Acute suppurative osteomyelitis in a 44-year-old woman. Dunfee B L et al. Radiographics 2006;26:1751-1768 ©2006 by Radiological Society of North America
  51. 51. Figure 23. Chronic osteomyelitis in a 47-year-old man. Dunfee B L et al. Radiographics 2006;26:1751-1768 ©2006 by Radiological Society of North America
  52. 52. Figure 24. Sclerosing osteomyelitis in a 10-year-old boy. Dunfee B L et al. Radiographics 2006;26:1751-1768 ©2006 by Radiological Society of North America
  53. 53. Fibro-osseous lesions Fibrous Dysplasia Monostotic Polyostotic Albright’s Syndrome Osseous Dysplasia Periapical Focal Florid Familial gigantiform cementoma Ossifying fibroma Juvenile trabecular Juvenile psammomatoid
  54. 54. FIBRO-OSSEOUS LESIONS Classification by Waldron CA: 1. Fibrous dysplasia 2. Reactive a. Periapical cemento-osseous dysplasia b. Focal cemento-osseous dysplasia c. Florid cemento-osseous dysplasia 3. Fibro-osseous neoplasms i.e. Ossifying fibroma
  55. 55. FIBROUS DYSPLASIA  Asymptomatic, self-limiting developmental regional alteration of bone in which the normal architecture is replaced by fibrous tissue and nonfunctional trabeculae-like osseous tissue.  It is self-limiting (thus it is not a true neoplasm) • Mutation in GNAS 1 gene  Clinical forms of fibrous dysplasia of the jaws • Monostotic: localized to a single bone  Juvenile and aggressive juvenile  Adult • Polystotic: involves several bones  Craniofacial  McCune-Albright syndrome  Jaffe syndrome
  56. 56. MONOSTOTIC FIBROUS DYSPLASIA • 80 – 85% of cases; Jaws most commonly affected • Painless enlargement of the affected bone • Diagnosed during 2nd decade • Maxilla > Mandible; male = females • Growth stops in late teen or early twenties • Maxillary lesions usually involve other adjoining bones – craniofacial FD
  57. 57. POLYOSTOTIC FIBROUS DYSPLASIA  McCune Albright Syndrome: Associated with skin pigmentation and endocrine dysfunction  Multiple bone (particularly the craniofacial bones)  Skin lesions - Café-au-lait spots  Endocrine dysfunction – precocious sexual development, pituitary, thyroid, parathyroid glands  Jaffe Syndrome: Absence of endocrine disturbances  Bone defects dominated by long bone involvement
  58. 58. CEMENTO-OSSEOUS DYSPLASIA • Most common fibro-osseous lesion of the jaws • Occurs in tooth bearing areas • 3 types: focal; periapical; florid  Periapical cemento-osseous dysplasia • Periapical region of anterior mandible • Middle-aged African-American women • 30-50yrs • Associated teeth are vital
  59. 59. Focal Cemento-osseous dysplasia • Single site involvement • 90% of cases occur in females • 3-6th decade • More common in whites • Posterior mandible • Asymptomatic and routine X-rays
  60. 60. Florid cemento-osseous dysplasia • Multifocal involvement • Both anterior and posterior mandible • Middle-aged African-American women • Rarely all 4 quadrants involved • Both dentulous and edentulous areas
  61. 61. OSSIFYING FIBROMA  Montgomery, 1927, first used the term.  Composed of fibrous tissue that contains woven as well as lamellar bone and acellular mineralized material resembling cementum.  Arises from the periodontal membrane.  Classification was given by El-Mofty S., 2002
  62. 62. JUVENILE TRABECULAR OSSIFYING FIBROMA  Shows bands of cellular osteoid together with slender trabeculae of plexiform bone lined by a dense rim of enlarged osteoblasts.  Maybe confused with osteosarcoma  Favors upper jaw.
  63. 63. JUVENILE PSAMMOMATOID OSSIFYING FIBROMA  Characterized by small ossicles resembling psammoma bodies, hence its name.  This type is usually located in the walls of the sinonasal cavities but sometimes can be encountered in the mandible.
  64. 64. CGCG Cherubism
  65. 65. CENTRAL GIANT CELL GRANULOMA • Intraosseous destructive lesions of the jaws • Far less common than peripheral giant cell granuloma • 10-30 yrs of age; Female > Male • Mandible > Maxilla; Ant. > Post. • Mandibular lesions frequently cross the midline • Asymptomatic or painless expansion • Nonaggressive and aggressive lesions • Perforation of the cortical plates and resorption of roots
  66. 66. Central giant cell granuloma in a 34-year-old man. CT scan (bone windowing) demonstrates a cystic lesion (arrows) within the mandible. Note the erosion of the mandibular cortex.
  67. 67. Central giant cell granuloma in a 34-year-old man. Photograph of the gross resected specimen shows multiple cystic cavities (arrows). Photomicrography with H-E stain revealed multinucleated giant cells within the lesion.
  68. 68. CHERUBISM • Autosomal dominant • Facial appearance similar to “cherub”-like • 2 – 5 yrs of age • The clinical alterations typically progress until puberty,  stabilize and slowly regress  Benign, self-limiting fibro-osseous disorder characterized by bilateral expansion of mandible, maxilla or both. • “Eyes upturned to heaven” appearance – due to involvement of the infraorbital rim and orbital floor • Painless bilateral expansion of the post. mandible • Marked widening and distortion of alveolar ridges • Tooth displacement and eruption failure
  69. 69. METASTATIC CARCINOMA • Most common form of cancer involving bone • Breast and prostate carcinomas are most common • Lung and kidney carcinomas also occurs • >80% of jaw metastasis occurs in mandible • Variety of symptoms: pain, swelling, loose teeth, paresthesia • Rarely patients are asymptomatic • Metastasis found in nonhealing extraction • Site from which tooth was removed for local pain or mobility • More often is undifferentiated and does not resemble primary lesion and difficult to tell primary • Immunohistochemistry is important • Prognosis is poor; most patients die within a year
  70. 70. PRIMARY INTRA-OSSEOUS CARCINOMA  Squamous cell carcinoma arising within the jawbones which has no initial connection with the oral mucous membrane, adjoining skin or nasal or antral mucous membranes.  Extremely rare tumor. Diagnosis is often made after metastasis has occurred.  Maybe of three different type (Elzay, 1982): 1. Arising from an odontogenic cyst. 2. Developing from an ameloblastoma. 3. Arising from odontogenic epithelium.
  71. 71. MULTIPLE MYELOMA  Rare malignant plasma-cell disorder.  The malignancy is more common in men over 50 years of age, and the jaws are affected in about 30% of cases.  Clinically, it presents with bone swelling, tooth mobility, pain, and paresthesia.  A painless soft swelling, usually on the alveolar mucosa and gingiva, may develop as part of the overall disease spectrum.
  72. 72. OSTEOSARCOMA • Malignancy of mesenchymal cells that produce osteoid or immature bone • Can arise following radiation - * Intramedullary  * Juxtacortical  * Extraskeletal • Osteosarcomas of jaws: 6% to 8% of all osteosarcomas 3rd to 4th decade (roughly 10-15 yrs older than for long bones) • Mandible=Maxilla • Swelling, pain, loosening of teeth, paresthesia and nasal obstruction
  73. 73. CHONDROSARCOMA • Half as common as osteosarcomas • 1% to 3% arise in head and neck area • Extragnathic lesions: older patients • Jaw lesions: Younger (<20yrs; mean=41.6 yrs) • Maxilla > mandible • Painless mass
  74. 74. EWING’S SARCOMA  Described under small round cell tumors.  Occurs predominantly in children & young adults between 5-25 yrs, median age of occurrence 13yrs.  Males > females  Episode of trauma often preceeds development.  Pain & bone swelling- earliest sign & symptoms  Jaws were involved in 13% of series of 126 cases reported by Geschickter and Copeland.
  75. 75. LEUKEMIA  Destructive lesions of bone are reported in some cases of chronic leukemia, and these may result in pathologic fracture or osteomyelitis- Shafer
  76. 76. PRIMARY LYMPHOMA OF BONE  Rare malignant neoplastic disorder of skeleton.  Described as distinct clinical condition by Parker and Jackson, 1939.  Pain, palpable swelling & pathologic fracture can be presenting feature.  Diagnostic criteria, (Coley, et al., 1950) by WHO are:  A primary focus in a single bone  Histologic confirmation  At the time of Dx, no evidence of distant soft tissue or distant lymph node involvement.
  77. 77. BURKITT’S LYMPHOMA  High-grade malignant B-lymphocyte lymphoma.  Epstein–Barr virus is closely associated.  The malignancy is prevalent in central Africa (the endemic form), and usually affects children 2–12 years of age.  Cases have also been observed in other countries (the nonendemic form), and recently in patients with AIDS.  The jaws are the most common site of lymphoma (60– 70%).  Clinically, it presents as a rapidly growing hard swelling that causes bone destruction, tooth loss, and facial deformity. Pain, paresthesia and large ulcerating or non- ulcerating masses may also be seen.
  78. 78. METABOLIC DISORDERS Eg.:  Osteoporosis  Osteomalacia  Renal dystrophy
  79. 79. PAGET’S DISEASE (OSTEITIS DEFORMANS)  Uncoordinated increase in bone turnover (osteoblastic and osteoclastic activity) producing large but weak bone, increased serum alkaline phosphatase and urinary hydroxyproline  Patients older than 40 yrs of age • More common in Western countries • Men > Women • Bone pain, fractures and bowing deformity – “simian stance” • Progressive increase in head circumference • Jaw involvement (17% of patients) • Maxilla > mandible – “lionlike facies” • Gradual enlargement of the jaw and generalized spaces between teeth • Compression of the cranial nerves and spinal cord leading to paralysis and loss of hearing and sight
  80. 80. LANGERHAN’S CELL HISTIOCYTOSIS Rare disease involving clonal proliferation of Langerhans cell. Manifestations range from isolated bone lesions to multisystem disease. Traditionally divided into 3 groups:  Unifocal – Eosinophilic granuloma  Multifocal unisystem- Hand-Schüller-Christian triad  Multifocal multisystem- Letterer-Siwe disease
  81. 81. EOSINOPHILIC GRANULOMA  Slowly-progressing disease  Can be monostotic or polyostotic  No extra-skeletal involvement
  82. 82. HAND-SCHüLLER-CHRISTIAN DISEASE  Seen mostly in children  Characterized by fever, bone lesions & diffuse euptions usually on scalp & in ear canals  50% cases involve pituitary stalk-> diabetes insipidus  Triad: diabetes insipidus, exopthalmos & lytic bone lesions
  83. 83. LETTERER-SIWE DISEASE  Rapidly progressing disease in which Langerhans cells proliferate in many tissues  Mostly seen in children <2 yrs of age  Poor prognosis
  84. 84. OSTEOPETROSIS  Also known as “Marble bone disease”, “Albers-Schönberg disease”, “osteosclerosis fragilis generalisata”  Defect in osteoclasts to resorb bone.  3 distinct forms:  adult onset  infantile  intermediate
  85. 85. OSTEORADIONECROSIS  Radiation-induced pathologic process characterized by chronic & painful infection & necrosis accompanied by late sequestration & sometimes permanent deformity.  Mandible > maxilla  Intractable pain, surface ulceration & pathologic fracture are common presenting features.
  86. 86. OSTEOGENESIS IMPERFECTA
  87. 87. ACHONDROPLASIA  Autosomal dominant disorder that is the most common cause of short limb dwarfism  As the skull base forms by endochondral ossification whereas the skull vault by membranous ossification there is a marked discrepancy in relative size as the skull vault, brain and cord grow normally whereas the skull base remains small.
  88. 88. HAIR-ON-END SIGN  Red marrow hyperplasia causes widening of the diploic space, and the outer table thins or is completely obliterated.  When the hyperplastic marrow perforates or destroys the outer table, it proliferates under the invisible periosteum, and new bone spicules are laid down perpendicular to the inner table.  Seen in patients with thalassemia major, iron deficiency anemia, sickle cell disease, and spherocytosis.
  89. 89. ARTERIO-VENOUS MALFORMATIONS  Abnormal, direct communications between arteries and veins.  Rare, may occur within the ramus and posterior mandibular body  Identification is important owing to potential fatal hemorrhage after tooth extraction  Radiography shows multiloculated lesions are cystic in appearance.  Angiography maybe necessary for Dx and Rx planning.
  90. 90. AVM in a 28-year-old man. (a) Contrast-enhanced CT scan reveals multiple dilated and tortuous vessels (arrow) within the right masseter muscle. Note the abnormal enhancement (arrowhead) within the marrow of the mandible. (b) Axial T1-weighted MR image demonstrates a slightly expansile lesion (arrow) within the right mandibular angle and body. Multiple flow voids are present within the right masseter muscle. Note the loss of normal fatty marrow (arrowhead) within the mandible.
  91. 91. PSEUDOTUMOR OF HEMOPHILIA  First described in 1918 by Starker  Pseudotumors are found almost exclusively in men between 20 and 70 years of age  Encapsulated, chronic, slowly expanding hematoma  Many patients recall sustaining an injury prior to development of the pseudotumor  Pseudotumors that occur in muscles with broad tendon insertions readily progress to cause severe pressure erosion of adjacent bone
  92. 92. MELANOTIC NEUROECTODERMAL TUMOR OF INFANCY  Rare benign tumor of neural crest origin that was first described by Krompecher in 1918  Maxilla > mandible  More common in anterior region  Rapidly growing, non-ulcerated, darkly pigmented lesion. Blue-black in color.  Lab Dx- high urinary level of VMA.
  93. 93. TRAUMATIC NEUROMA  Rare disorder that occurs after trauma or surgery & involves the peripheral nerve.  Exaggerated response to injury consisting of reactive hyperplasia of nerve tissue, usually at the proximal end.  Common intra-oral locations are mental foramen, lower lip & tongue. Intraosseous neuroms are very uncommon.
  94. 94. SCHWANNOMA  Neurilemmoma, a benign neoplasm derived from Schwann cells was first described by Verocay in 1910.  The intrabony lesions account for less than 1% of the central neoplasms.
  95. 95. INTRAOSSEOUS LIPOMA  Benign tumor, 1% of all lipomas
  96. 96. References: 1. Burket’s Oral Medicine, 8th Ed 2. Burket’s Oral Medicine, 11th Ed 3. Textbook Of Oral Medicine, Arvind Rao Ghom, 2nd Ed 4. Shafer’s Textbook of Oral Pathology, 5th Ed 5. Textbook of Dental and Maxillofacial Radiology, Freny R Karjodkar, 2nd Ed 6. Color Atlas of Common Oral Diseases, Langlais, 4th Ed
  97. 97. 7. Cherubism- a case report with review, Indian Journal of Dentistry, Vol. 2/Issue 3, Pages 44-47, April-June 2011 8. A large calcifying lesion of the maxilla in a child, JADA, Vol. 142, No. 9, 1026-1030, September 2011 9. Clinical and radiographic features of Solitary and cemento-osseous dysplasia-associated simple bone cysts, DMFR, Volume 40, Number 4, Pages 230-235, May 2011 10. Odontogenic Myxoma: Report of two cases with review of literature, Journal of Indian Academy of Oral Medicine, Volume 3, Number 2, Pages 143-146, April-June 2011 11. Ossifying fibroma of the jaws: Report of two cases and literature review, Oral Oncology, Volume 47,Issue 9, Pages 804-809, September 2011 12. Keratocystuc Odontogenic Tumor: Case Reports and Review of Literatre, Journal of Indian Academy of Oral Medicine, Volume 3, Number 2, Pages 150-154, April-June 2011 13. Odontogenic tumours manifesting in the first two decades of life in a rural African population sample: a 26 year retrospective analysis, DMFR, Volume 40, Number 6, Pages 331-337, September 2011 14. Ameloblastic fibro-odontosarcoma of the mandible: Imaging findings, DMFR, Volume 40, Number 5, Pages 324-327, July 2011 15. Ledesma-Montes C, Gorlin RJ, Shear M, Prae Torius F, Mosqueda-Taylor A, Altini M, et al. International collaborative study on ghost cell odontogenic tumours: calcifying cystic odontogenic tumour, dentinogenic ghost cell tumour and ghost cell odontogenic carcinoma, J Oral Pathol Med 2008;37(5):302-8. 16. El-Mofty S. Psammomatoid and trabecular juvenile ossifying fibroma of the craniofacial skeleton: Two distinct clinicopathologic entities. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2002;93:296-304 17. Keratinized Primary De Novo Intraosseous Carcinoma of Mandible: Report of a Case and Literature Review, Research Journal of Biological Sciences, Year: 2010, Volume: 5, Issue: 3, Page No.: 233-240 18. Cotran, Ramzi S.; Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Robbins, Stanley L. (2005). Robbins and Cotran pathologic basis of disease. St. Louis, Mo: Elsevier Saunders. pp. 685- Langerhans Cell histiocytosis 19. Primary bone lymphoma: report of a case with multifocal skeletal involvement, K Rahmat*, FRCR, ML Wastie, FRCR, BJJ Abdullah, FRCR, Biomed Imaging Interv J 2007; 3(4):e52 20. The Hair-on-End Sign, November 2001 Radiology, 221, 347-348. 21. Pseudotumor of hemophilia in the mandible of a patient with hemophilia A, Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, Volume 113, Issue 2 , Pages 229-233, February 2012 22. Soft tissue lipoma with the radiographic appearance of a neoplasm within the mandibular canal, Dentomaxillofac Radiol July 1, 2006 vol. 35 no. 4 299-302 23. Patil K, Mahima VG, Srikanth HS, Saikrishna D. Central schwannoma of mandible. J Oral Maxillofac Pathol 2009;13:23-6

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