BENIGN NON ODONTOGENIC TUMORS Histopathology

• fibrous CT with well defined spindled fibroblasts

• uniform cellularity
Ossifying Fibroma • collagen fibers are arranged haphazardly, although a
whorled, storiform pattern may be evident

• (+) bony spheroids, trabeculae, islands in the stroma

• bone is immature, and often is surrounded by
OSTEOBLASTS
• osteoclasts are infrequently seen

Juvenile (Aggressive) Ossifying Fibroma

• benign neoplasm of bone that has the potential for 1. Juvenile Trabecular
excessive growth, bone destruction and recurrence • children and adolescents
• clinically and microscopically similar to Cementifying • 20% occur in older than 15
Fibroma • exclusively in maxilla and mandible
• progressive and rapid growth but rarely pain
Radiographically,
Etiology and Pathogenesis • defined border
• undetermined cause • may range from radiodense to radiolucent
• (+) excision, rare recurrence

2. Juvenile Psammomatoid Ossifying Fibroma (JTOF)

• extragnathic craniofacial bones, esp.
paranasal sinuses and periorbital bones
• older populations
• microscopically, formed relatively cellular
stroma containing small, rounded
calcifications (psammomatoid)
• surgical excision, 30% show recurrences
Clinical Features

Cementifying Fibroma and Cemento-ossifying fibroma

• terms used when the bony islands are round and
spheroidal
• similar age groups and locations, exhibit comparable
clinical characteristics, and same biological behavior
• 3rd and 4th decades of life • same lesion as Ossifying fibroma
• women > men
• slow-growing, asymptomatic, expansile lesion Differential Diagnosis
• jaw and craniofacial bones • Ossifying Fibroma - mature lamellar bone
• tooth bearing regions - mandibular premolar-molar • Fibrous Dysplasia - woven bone
• expansion, and thinning of Bu and Li cortical plates • Osteoblastoma - younger age group, pain, osseous
trabeculae are rimmed by abundant plump
• perforation and mucosal ulceration are rare osteoblasts, central nidus
• solitary • Focal Cemento-osseous Dysplasia - biopsy, same in the
radiograph
Radiographically • Focal Osteomyelitis - associated with a source of
• well circumscribed, sharply defined borders inflammation, pain, swelling
• unilocular or multilocular radiolucencies
• mixed radiolucent-radiopaquue image is seen when
islands of tumor bone are densely calcified Treatment and Prognosis
• mixed radiolucent-radiopaquue image is seen when • Surgical removal
islands of tumor bone are densely calcified • Curettage
• Enucleation
• easily separated from surrounding bone
• recurrence is rare

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Fibrous Dysplasia Radiographically

• radiolucent to uniformly radiopaque mass

• “ground glass” or “peau d’orange” effect (not
pathognomonic)
• unilocular or multilocular, esp in long bones
• mottled radiolucent and radiopaque appearance
(long standing)
• fingerprint bone pattern
• superior displacement of the mandibular canal in the
• normal medullary bone is replaced by an abnormal mandibular lesions
fibrous CT proliferation • poorly defined radiographic and clinical margins of the
• new, non maturing bone lesions
• blends in the surrounding normal bone without the
Etiology and Pathogenesis evidence of a circumscribed border
• term to indicate that the condition represented a • elliptic in shape rather than spheric
dysplastic growth resulting from deranged
mesenchymal cell activity or a defect in control of Histopathology
bone cell activity • slightly to moderately cellular fibrous CT stroma that
• neoplastic process contains foci of irregularly shaped trabeculae of
• mutations of GNAS I gene encoding for the alpha immature bone
subunit of a transmembrane-signaling G protein • fibrous tissue : bone ratio is characteristic
appear to be present • fibroblasts exhibit a uniform spindle-shaped nuclei
• may ultimately affect the proliferation and • (-) mitotic figures
differentiation of fibroblasts and osteoblasts that make • bony trabeculae assume irregular shapes (Chinese
up this lesion characters) and (-) functional orientation
• bone is women type, arise directly from the
Clinical Features collagenous stroma without osteoblastic activity
• asymptomatic • Mature fibrous dysplasia - (+) lamellar bone
• enlargement of involved bone • (+) capillaries
• single or several bone involvement
• Monostatic Fibrous Dysplasia or Polyostotic Fibrous
Dysplasia

• McCune-Albright syndrome
o consists of polyostotic fibrous dysplasia,
cutaneous melanotic pigmentation (café-au-
lait macules) and endocrine abnormalities
o acromegaly, hyperthyroidism, Differential Diagnosis
heperparathyroidism, hyperprolactinemia
• Jaffe-Lichtenstein Syndrome Fibrous Dysplasia Ossifying Fibroma
o multiple bone lesions of fibrous dysplasia and 1st and 2nd decade 3rd and 4th decade
skin pigmentations Maxilla > Mandible Mandible > Maxilla
o jaw involvement is common, other bones Diffuse Opacity Circumscribed
affected - ribs and femur maxilla > mandible Self Limited Continuous growth
o maxillary lesions - maxillary sinus, zygoma, One or more bones One bone
sphenoid bone, floor of the orbit Vascular matrix Cellular fibrous matrix
• Craniofacial Fibrous Dysplasia Woven bone trabeculae Bony islands and trabeculae
o mandible - body of the mandible Stabilizes at puberty Not hormone related
o mandible - body of the mandible Recontour for cosmetics Excise
o (+) facial asymmetry
o dental arch - maintained, (+) displacement of • Chronic Osteomyelitis - inflammation, tenderness, pain,
teeth, malocclusion, interference with tooth drainage
eruption o Periostitis - radiographic feature of
o (-) tooth mobility osteomyelitis
o 1st or 2nd decade • slowly progressive, asymptomatic nature of fibrous
o Mono - equal gender distribution dysplasia allows differentiation from malignant tumors
o Poly - females > males of bone

Treatment and Prognosis

• stabilizes after pubertal growth
• small lesions - no treatment other than bx confirmation
and follow up
• large lesions - surgical recontouring, deferred until
stabilisation of the disease process
• En bloc resection are impractical since it is relatively
large and poor;y delineated
• Bisphosphonates - improve symptoms of pain and bone
density
• Malignant transformation is rare, usually in Polyostotic
type

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Cemento-osseous Dysplasia

Radiographically
• well circumscribed
• unknown cause • mixed lancetopaque pattern
• describes a spectrum of disorders that include PA • thin radiolucency may be surrounding a variably
cements-osseous dysplasia, Focal cements-osseous calcified central tumor
dysplasia, and Florid cemento-osseous dysplasia
• same disease process • sclerosis of precessional bone - constant feature of
• distinguished on the basis of extent of involvement of osteoid osteoma (may be absent in Osteoblastoma)
affected portions of the jaw • peripheral “sun-ray” pattern of new bone production
• generic microscopic term represent a diverse group of may mimic osteosarcoma
reactive, dysplastic and neoplastic lesions
characterized microscopically by replacement of Histopathology
normal bone with collagenous matrix containing
trabeculae of immature bone and sometimes,
cementum-like material
• Dx is based on clinical pathologic correlation
Fibro-osseous Jaw Lesions
• Cemento-osseous dysplasia (PA, Focal, Florid)
• Ossifying Fibroma
• Fibrous Dysplasia • irregular trabeculae of osteoid and immature bone
• Chronic Osteomyelitis within a stroma containing a prominent vascular
network
Osteoblastoma and Osteoid Osteoma • various degrees of calcification
• remodelling of osseous tissue in form of basophilic
reversal lines

• (+) osteoblast-like cells and multinucleated giant cells

Differential Dx
• Cementoblastoma - arises from tooth root and fused to
it
• Ossifying Fibroma - not painful, no osteoblasts
• uncommon primary lesion of the bone • Fibrous Dysplasia - poorly defined radiographic margins,
• maxilla or mandible no prominent osteoblasts microscopically
• Osteoid osteoma - smaller version • Osteosarcoma
• benign neoplasms of undermined cause, genetic • hyperchromatic, large osteoblasts - both
defect • cytologic atypia, abnormal mitotic figures, delicately
• maybe confused with Osteosarcoma microscopically calcified tumor osteoid and heterogenous pattern

CLinical Features Treatment and Prognosis

• Osteoblastoma - lesions larger than 1.5cm in diameter • conservative surgical approach
• Osteoid osteoma - less than 1.5cm • curettage or local excision
• vertebrae or long bones, less commonly on craniofacial • Aggressive Osteoblastoma
bones
• Posterior tooth-bearing areas of maxilla and mandible
• bony cortices may be expanded and tender to
palpation
• 2nd decade, 90% appears before age 30
• males > females, 2:1
• severe pain in Osteoid osteoma
• localized swelling alone or along with pain
• Aspirin or NSAIDs relieve symptoms, including nocturnal
pain
• duration of signs and symptoms - weeks to years

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Osteoma Differential Diagnosis
• Bony exostoses of the jaw
• Osteblastomas and Osteoid Osteoma - pain
• Odontoma
• Focal Sclerosing Osteomyelitis

Treatment and Prognosis

• Surgical excision
• (-) recurrence

• benign tumors
• consist of mature, compact, or cancellous bone
• Periosteal Osteoma - arise from surface of the bone
• Endosteal Osteoma - develop centrally within bone

Clinical Features
• 2nd to 5th decade
• males > females
• solitary, except in patients with Gardner’s syndrome
• Periosteal Osteoma
• asymtomatic
• slow-growing, bony hard masses
• asymmetry may be noted when enlarged to sufficient
proportions
• Endosteal Osteoma discovered through routine
radiograph examination dense, well-circumscribed
radiopacities
• may arise both in maxilla and mandible
• facial and skull bones and paranasal sinuses
• symptoms : headaches, recurrent sinusitis,
ophthalmologic complaints

• Gardner’s Syndrome
o autosomal dominant disorder
o characterized by intestinal polyposis, multiple
osteomas, fibromas of skin, epidermal and
trichilemmal cysts, impacted permanent and
supernumerary teeth and odontomas

Histopathology
• 2 histologic variants
• 1. relatively dense, compact bone with sparse marrow
tissue
• 2. lamellar trabeculae of cancellous bone with
abundant fibrofatty marrow osteoblasts may be
numerous, but osteoclasts are sparse

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Desmoplastic Fibroma Chondroma

• benign, locally aggressive lesion of bone • benign cartilagenous tumor

• considered the bony counterpart of fibromatosis • cause : unknown
• long bones and pelvis, occasionally the jaws • rarely seen in the jaws
• cause : unknown • painless, slowly progressive swelling Chondroma
• locally aggressive clinical behaviour, suggesting a • gradual expansion = mucosal ulceration
neoplastic process • arise in nasal septum and ethmoid sinuses
• genetic, endocrine, and traumatic factors = exuberant • maxilla = anterior region
reactive proliferation • mandible = body and symphysis, coronoid process and
condyle
Clinical Features • male = female
• < 30 years old, mean = 14 years • before 50 years of age
• no gender predilection Radiographically
• mandible, body-ramus region • irregular radiolucent area
• slowly progressive, asymptomatic • (+) foci of calcification within radiolucent area
• (+) swelling of the jaw • well defined lobules of hyaline cartilage
• diagnostic problem : Chondroma vs. Chondrosarcoma
Radiographically • surgical excision
• unilocular or multilocular • unusual recurrence
• margins : well demarcated or poorly defined
• (+) cortical perforation
• (+) root resorption

Histopathology
• interlacing bundles and whorled aggregates of densely
collagenous tissue that contains spindled and
elongated fibroblasts
• hypercellularity with plumper fibroblasts
• (-) cytologic aytpia, (-) mitotic figures
• bone is not produced

Differential Diagnosis
• Odontogenic Cysts
• Odontogenic Tumors
• Non Odontogenic Lesions
• Malignancy (due to aggressive features such as
cortical perforation)
• Fibrosarcoma

Treatment and Prognosis

• Surgical Resection
• Curettage : recur

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Central Giant Cell Granuloma

Histopathology
• uniform fibroblasts in a stroma containing various
• or Giant Cell Lesion amounts of collagen
• benign proliferation of fibroblasts and multinucleated • (+) Hemosiderin-laden macrophages and extravasated
giant cells that occurs almost exclusively within the jaws erythrocytes
• solitary radiolucent lesion of the maxilla or mandible • (+) multinucleated giant cells (patches or evenly
distributed)
Etiology and Pathogenesis • (+) foci of osteoid around the peripheral margins
• thought to represent a reparative response to intrabony
haemorrhage and inflammation Differential Dx
• once regarded as “reactive lesion” • Solitary or Multilocular CGCG
• best classified as benign neoplasm o Ameloblastoma
• primary tumor cells : FIBROBLASTS o Odontogenic Myxoma
• secondary cells : MULTINUCLEATED GIANT CELLS o Odontogenic Keratocyst
• accessory cells : macrophages, factor XIIIa dendritic • Young Age
cells, endothelial cells o Ameloblastic Fibroma
• Fibroblasts: o Ossifying Fibroma
o express proteins that are indicative of cells in o AOT
the cell cycle • Microscopic appearance
o responsible for the recruitment and retention • Giant cell lesion of Hyperparathyroidism -biochemical
of monocytes and transformation into test, elevated parathyroid hormone
multinucleated giant cells • Giant cell tumor of long bone
• Aneurysmal Bone Cyst - sinusoidal blood spaces within
Clinical Features the tumor mass
• children and young adults • Cherubism - cherubism gene SH3BP2
• 75% before age 30 • Noonan-like / Multiple Giant Cell Lesion Syndrome
• females > males 2:1 • mutations in PYTPN11 gene
• occurs almost exclusively in the maxilla and mandible Treatment and Prognosis
• isolated cases in facial bones and small bones of hands • Surgical Management
and feet • Excision or curettage of tumor mass
• mandible > maxilla • removal of peripheral bony margins
• anterior to the permanent molar teeth • good prognosis
• occasional extension across the midline • low recurrence rate
• rarely : mandibular ramus and condyle • Intralesional Injections of Corticosteroids
• painless expansion and swelling • Exogenous calcitonin
• cortical plates are thinned • Interferon alpha - antiangiogenic mode of action
• perforation and extension to soft tissues : uncommon • Bisphosphanates

Radiographically
• multilocular or unilocular radiolucency of bone
• margins : well demarcated , scalloped border
• “aggressive” CGCG
• pain or paresthesia
• rapid growth
• root resorption
• perforation of cortical bone
• higher recurrence rate

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Giant Cell Tumor Hemangioma of Bone

• true neoplasms • rare intraosseous vascular malformations that, when

• arise most commonly in long bones especially in area seen in the jaws, mimic both ODONTOGENIC and NON
of the knee joint ODONTOGENIC lesions
• rare in the jaws • “difficult-to-control haemorrhage” - notable
• head and neck : sphenoid, ethmoid, temporal bones complication of surgical intervention
• 3rd and 4th decade of life
• slow growth and bone expansion OR Clinical Features
• rapid growth, pain, paresthesia • more than half occur in the mandible
• posterior region
Radiographically • female > male
• radiolucent image • peak age of discovery : 2nd decade of life
• Features firm, slow-grow, asymmetric expansion of the
jaw (most common pt complaint)
• spontaneous gingival bleeding
• paresthesia or pain
• vertical mobility of involved teeth
• (+) bruits or pulsation upon auscultation or palpation of
thinned cortical plates

Radiographically
• multilocular radiolucencies
Microscopically • “soap bubble” appearance
• 2nd form - rounded, radiolucent lesion in which bony
trabecular radiate from the center of the lesion
producing angular loculations.
• cyst-like radiolucency
• (+) resorption of the roots

• (+) numerous multinucleated giant cells dispersed

Histopathology
evenly among monocytemacrophages (reactive cells)
• proliferation of blood
and spindle cells (neoplastic cells)
vessels
• stromal cellularity with minimal collagen production
• cavernous type (large-
• giant cells are larger and contain more nuclei than
caliber vessels)
CGCG
• capillary type (small-
• (+) inflammatory cells (+) areas of necrosis
caliber
• (-) hemorrhage and hemosiderin deposition
• osteoid formation is less often than in CGCG
Treatment and Prognosis
Differential Diagnosis
• surgical excision
• Ameloblastoma
• antiosteoclastogenic drugs : bisphosphonates,
• Odontogenic Myxoma
monoclonal antibody to RANK ligand
• Odontogenic Keratocyst
• greater tendency to recur
• CGCG
• 30% recurrence after curettage in long bones
• Aneurysmal Bone Cyst
• Angiography for dx

Treatment and Prognosis

• life threatening if improperly managed
• extraction of teeth in an area involved by a central
vascular lesion may result to potentially fatal bleeding
• NEEDLE ASPIRATION biopsy
• surgery
• radiation therapy
• sclerosing agents
• cryotherapy
• presurgical techniques

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Langerhans Cell Disease • lesions are occasionally located exclusively in the PA
site where they mimic PA inflammatory jaw lesions
• cervical lymphadenopthy, mastoiditis, and otitis media
are head and neck manifestations that often present
with multifocal involvement

Histopathology
• proliferation of large cells with
abundant cytoplasm
• indistinct cell borders
• formerly known as HISTIOCYTOSIS X and IDIOPATHIC • oval to reniform nuclei
HISTIOCYTOSIS • (+) eosinophils and
• proliferation of cells exhibiting phenotypic inflammatory cells
characteristics of Langerhans cells • (+) multinucleated giant cells
• Clinical manifestations : solitary to multiple bone lesions and foci of necrosis
to disseminated visceral, skin, and bone lesions • ultrastructure of tumor cells shows:
• HISTIOCYTOSIS X was used to encompass 3 disorders • unique, rod shaped cytoplasmic structures
o Eosinophilic Granuloma • identical to Birbeck granules (present in normal
o Hand - Schüller -Christian Syndrome Letterer- Langerhans cells)
Siwe Disease • tumor cells express CD1a antigen, S-100 protein and
• similar microscopic appearance despite the diverse human Leukocyte antigens (HLA)-DR (characteristic or
manner of clinical disease expression normal LH cells)
• Eosinophilic Granuloma
o or Chronic Localized LCD Differential Diagnosis
o solitary or multiple bony lesions only • loosening and premature exfoliation of teeth =
• Hand-Schüller-Christian Syndrome precocious eruption of permanent teeth
o or Chronic Disseminated LCD o Juvenile or Diabetic Periodontitis
o specific triad of o Hypophospatasia
▪ 1. lytic bony lesions o Leukemia
▪ 2. exophthalmos and o Cyclic Neutropenia
▪ 3. diabetes insipidus o Agranulocytosis
o lymphadenopathy, dermatitis, splenomegaly o Primary or Metastatic Malignant Neoplasms
or hepatomegaly • Lesions located in PA site
• Letterer-Siwe Disease o Periapical Cyst or Granuloma
o or acute Disseminated LCD o pulp vitality
o malignant process characterised by a rapidly • Solitary Radiolucent lesions
progressive, often fatal course o Odontogenic tumors and cysts
o widespread organ, bone, and skin o Multiple Myeloma (older age group)
involvement by a proliferative process in
infants Treatment and Prognosis
• Chemotherapeutic agents
Etiology and Pathogenesis • may be fatal despite intensive treatment
• LCD tumor cells are similar to normal Langerhans cells • Pt with poor prognosis - allogenic bone marrow
that reside in epidermis and mucosa transplant
• development from normal to tumor Langerhans cells in • Individual lesions - surgical curettage, or low dose
UNKNOWN radiation therapy
• neoplastic process • Cytotoxic agents - vincristine S04, cyclophosphamide,
• exuberant reaction to an unknown antigenic challenge methotrexate with corticosteroids (widespread or
• deficiency of suppressor T cells + low levels of serum visceral involvement)
thymic factor = thymic abnormality • 10-15 year survival
• immunologic defects may affect normal regulatory
mechanisms, with reluctant Langerhans cell • Localized form of LCD - older children, adolescents and
proliferation young adults
• vigorous curettage, intralesional corticosteroid
Clinical Features injections and low-dose radiation therapy
• condtion of children and young adults • involved teeth are sacrificed due to lack of bony
• monostatic and polyostatic forms support
• skull, mandible, ribs, vertebrae and long bones • prognosis – good
• skin, mucosal or bone involvement in the head and • long term follow up to rule out recurrence of disease
neck region was noted 80% of children
• tenderness, pain and swelling are common patient
complaints
• loosening of teeth
• gingival tissues : inflammed, hyperplastic and ulcerated
• oral mucosal lesions : submucosal nodules, ulcers, and
leukoplakia
• solitary or multiple radiolucent
• lesions lesions often affect the alveolar bone - teeth
appear as if they were floating in space
• bone lesions with sharply circumscribed “punched-out”
appearance may occur in the central aspect of the
jaws

8
Tori and Exostoses • EXOSTOSES
o multiple (or single) bony excrescences
o symptomatic bony nodules that are present
along the Bu aspect of alveolar bone
o posterior portions of maxilla and mandible

• nodular protruberances of mature bone

• little clinical significance
• non-neoplastic, rare source of discomfort
• mucosa may be traumatically ulcerated, producing a
slow-healing, painful wound or less commonly,
osteomyelitis
• surgical removal for prosthetic rehab

Etiology and Pathogenesis

• inherited condition
• both genetic and environmental factors determine
development of mandibular tori
• Palatal Torus : Asians, Native Americans, Inuit (Eskimos) Histopathology
• Incidence in US : 20-25% • composed of hyperplastic bone
• Mandibular Tori : Blacks and Asians • mature cortical and trabecular bone
• US population : 6-12% • outer surface : smooth, rounded contour
• cause : UNKNOWN
• bony growths represents a reaction to increased or Treatment and Prognosis
abnormal occlusal stresses of teeth in involved areas • Tx is unnecessary
• Prosthodontic considerations
Clinical Features • cases of frequent trauma to overlying mucosa
• Surgical excision
• TORUS PALATINUS
o sessile, nodular mass of bone that appears
along the midline of the hard palate
o females > male 2:1
o 2nd to 3rd decade of life
o symmetric fashion
o asymptomatic
o nodular, spindled, lobular, or flat
o diffuse radiopaque mass on the radiographs

• TORUS MANDIBULARIS
o bony exophytic growths
o lingual aspect of the mandible, superior to the
mylohyoid ridge
o bilateral, premolar region
o asymptomatic
o slow growth
o 2nd to 3rd decade
▪ may arise as solitary nodules multiple
nodular masses that appear to
coalesce
▪ no gender predilection

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Coronoid Hyperplasia

Etiology and Pathogenesis

• cause : UNKNOWN
• (+) History of TRAUMA
• represents a Hyperplastic
process, though may be
neoplastic
• Unilateral Coronoid
Hyperplasia = Solitary
Osteochondroma
• Bilateral Coronoid Hyperplasia
• males = X-linked inherited origin
• increased activity of temporalis muscle with
unbalanced condylar support may be a causative
factor

Clinical Features
• often bilateral
• unilateral enlargement may be noted
• limited mandibular movement, which is progressive
over time
• painless
• not associated with facial swelling or asymmetry
• young male patients
• around puberty
• enlarged and elongated coronoid process are evident
radiographically
• Unilateral = misshapen or mushroomshaped on
radiographs
• TMJ radiographs are unremarkable

Histopathology
• consist of mature, hyper plastic bone
• bone may be partially covered by cartilaginous and
fibrous connective tissue
Differential Diagnosis
• Unilateral - Osseous and Chondroid neoplasms
Treatment and Prognosis
• surgical excision
• postoperative physiotherapy
• recurrence is rare

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