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

1. Dr. Vibhuti Kaul

2. The Origin and Location of Bone Cells

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. Jaw Bone Lesions
Related to
Dentition
Bone
Proper

5. Related to
Dentition
Cysts
Odontogenic
Tumors

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. 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. 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. 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

12. 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”

13. 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

14. • 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.

15. PRIMORDIAL CYST
 An odontogenic cyst developing from the stellate
reticulum.
 Formed in place of the tooth i.e. the tooth is missing.

18. 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.

20. 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”

22. 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.

24. 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.

27. 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.

29. MEDIAN PALATINE CYST

30. 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

32. 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.

34. 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

37. 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.

40. 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

41. 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

42. III. Odontogenic ectomesenchyme with or without
included odontogenic epithelium
1. Odontogenic fibroma
2. Myxoma (myxofibroma)
3. Cementoblastoma (benign cementoblastoma,
true cementoma)

43. 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

44. 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.”

45.  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

49. 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.

51. 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.

55. 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.

59. 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.

62. 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.

64. 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.

66. 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.

69. 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.

71. 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.

73. 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.

76. 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

78. 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.

80. 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

81.  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.

83. 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.

86. Reactive
Bone
Diseases
Fibro-
osseous
lesions
Giant
cell
lesions
Bone
tumors
Others

87. REACTIVE BONE
LESIONS
TORI
MAXILLARY MANDIBULAR
INFLAMMATORY
DISEASES
(OSTEOMYELITIS)
NECROSIS SCLEROSIS

88. TORUS PALATINUS

89. TORUS MANDIBULARIS

91. OSTEOMYELITIS

93. 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

94. 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

95. 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

96. Fibro-osseous
lesions
Fibrous
Dysplasia
Monostotic
Polyostotic
Albright’s Syndrome
Osseous
Dysplasia
Periapical
Focal
Florid
Familial gigantiform
cementoma
Ossifying
fibroma
Juvenile trabecular
Juvenile psammomatoid

97. 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

98. 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

100. 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

103. 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

105. 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

108. 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

110. 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

112. 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

114. 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.

119. 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.

122. CGCG
Cherubism

123. 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

125. 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.

126. 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.

128. 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

131. 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

135. 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.

138. 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.

141. 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

144. 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

146. 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.

148. LEUKEMIA
 Destructive lesions of bone are reported in some cases
of chronic leukemia, and these may result in
pathologic fracture or osteomyelitis- Shafer

150. 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.

154. 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.

157. METABOLIC DISORDERS
Eg.:
 Osteoporosis
 Osteomalacia
 Renal dystrophy

158. 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

161. 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

162. EOSINOPHILIC GRANULOMA
 Slowly-progressing disease
 Can be monostotic or polyostotic
 No extra-skeletal involvement

163. 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

165. LETTERER-SIWE DISEASE
 Rapidly progressing disease in which Langerhans cells
proliferate in many tissues
 Mostly seen in children <2 yrs of age
 Poor prognosis

167. 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

169. 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.

171. OSTEOGENESIS IMPERFECTA

173. 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.

176. 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.

179. 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.

180. 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.

181. 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

183. 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.

187. 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.

189. 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.

192. INTRAOSSEOUS LIPOMA
 Benign tumor, 1% of all lipomas

193. 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

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