BENIGN NON ODONTOGENIC TUMORS

 TUMOR:
o The word tumor means swelling.

 ODONTOGENIC TUMOR:
o A group of neoplasms and tumor like malformations arising from cells of
odontogenic apparatus and their remnants.

 NON ODONTOGENIC TUMOR:

o Non-odontogenic jaw tumors, on the other hand, develop from the epithelium
and/or mesenchyme of a wide variety of tissues in the body, often originate in
non-tooth-bearing facial bones, and may develop in other sites outside of the
head and neck.

WHO CLASSIFICATION OF ODONTOGENIC AND NON ODONTOGENIC

TUMORS
CONTENTS OF TODAY’S SEMINAR WILL BE CONVERED IN FOLLOWING
HEADINGS:

 FIBROOSSEUOUS LESIONS:
 Fibrous dysplasia
 Ossifying fibroma
 Juvenile ossifying fibroma
 Cemento osseous dysplasia

 GIANT CELL LESIONS:

 Central giant cell granuloma
 Brown tumor of hyperparathyroidism
 Cherubism
 Aneurysmal Bone Cyst

 NEUROGENIC TUMORS
 Schawanomma
 Neurofibroma

 LANGERHANS CELL DISEASE

 Chronic localized
Chronic disseminated
Acute disseminated

 OSTEOID OSTEOMA AND OSTEOBLASTOMA

 OSTEOMA CHRONDROMA
 DESMOPLASTIC FIBROMA

EXAMINATION AND DIAGNOSTIC METHODS

HISTORY OF SPECIFIC LESION:

 Prolonged duration; may be congenital

 Long duration without pain; benign neoplasm
 Short duration, rapid growth; malignant growth
 Mode of onset and progress
  History of trauma may be obtained in many bone lesions like osteogenic sarcoma.
Spontaneous
 swelling and rapid growing lesion may be malignant, while very slowly growing lesion
may be benign growth.
 Exact site and shape
 Progress of the lesion - Whether the swelling has been growing slowly or it has
remained stationary for a long time (benign growth). Has it been growing again after a
stationary period of months/years (malignant transformation in a benign lesion) or has
it been continuously increasing in size (malignant growth)?
 Change in character of a lesion Whether there are ulcerations over the lesions?
Fluctuation, softening, etc. are noticed by the patient recently? Whether painless
swelling has become painful –secondary infection may have set in the lesion.
 Associated symptoms Pain, abnormal sensations, anaesthesia, paraesthesia over a
region, dysphasia, nasal obstruction – breathing difficulty, tenderness,
lymphadenopathy.
 Trismus
 Loss of body weight Malignant growth
 Recurrence
 Habits

CLINICAL EXAMINATION OF LESION:

 Number – whether single or multiple

 Size
 Site– palatal swellings may have salivary gland origin
 Shape and size of the lesion – whether ovoid, spherical, localized, diffuse, etc.
 Colour of the lesion – whether red or purple (haemangioma), blue (ranula)
 Surface – whether smooth, lobulated (Benign) or irregular, ulcerated, fungating growth
(malignancy)
 Whether it is pedunculated or sessile?
 Skin over the swelling – red, hot skin will suggest secondary infection

RADIOGRAPHIC CONSIDERATIONS:

 position
 size
 shape
 presence or absence of lesional calcifications
 estimation of soft tissue volume in relation to calcified tissue
 cyst formation
 impingement on or inclusion of vital anatomic structures,
 displacement of teeth or root resorption
  boundaries between lesion and bone

GENERAL RADIOGRAPHIC FEATURES:

 Benign lesions : Often encapsulate.

 Gradual enlargement.
 Hence tumor borders are usually smooth and Radiographically well defined.
 Effect on adjacent tissues – benign tumor exerts pressure resulting in displacement of
teeth or bony cortices.
 Root resorption – benign tumors – resorption of teeth in a smooth fashion and any
along the adjacent edge of tumor.
 Malignant tumors – surround entire root if resorption occurs –sometimes no resorption.
 Odontogenic tumors may be:
o Radiolucent
o Mixed radiolucent and radiopaque
o Radiopaque

BIOPSY:

 Definitive diagnosis is established after incision, excision or intraoperative (frozen

section) biopsy
 Biopsy technique is selected after careful assessment of patient & of use of local,
sedation or GA
 Excisional biopsy is performed for completely calcified lesions
 Intraoperative frozen sections is used to study questionable soft tissue

MANAGEMENT:

 Goals of treatment
 Eradication of lesion with the least morbidity, preservation and restoration of function.
 Depends on
 Growth potential
 Size
 Anatomic location
 Association with vital structures
 Soft tissue involvement

SURGICAL TREATMENT:

 Curettage
 Cautery (electrocoagulation)
  Enbloc resection
 Resection with continuity defect
 Partial resection
 Total resection
 Reconstruction with bone grafting or appropriate free tissue transfer


FIBROUS DYSPLASIA

 Fibrous dysplasia is a condition in which normal medullary bone is gradually re-placed

by an abnormal fibrous connective tissue proliferation.
 The mesenchymal tissue contains variable amounts of an osseous matrix that
presumably arises through metaplasia and consists only of woven bone.
 Solitary or multifocal lesions in which there is arrest of bone development in the woven
bone stage with failure to mature to lamellar bone. The resultant fibro-osseous tissue is
poorly formed and structurally inadequate.
 Many authorities accept the premise that fibrous dysplasia rep-resents a non-neoplastic,
hamartomatous growth resulting from altered mesenchymal cell activity or a defect in
the control of bone cell activity.
 Recent investigations reveal presence of an activating mutation in the gene that codes
for the Gs alpha membrane-associated protein.
 Another hypothesis is that this condition represents focal bone expression of a
complicated endocrine disturbance.
 The finding of estrogen receptors in osteogenic cells of a patient with fibrous dysplasia
suggests defect in the regulation of these receptors and thus cellular activity.
 An inherited basis for the development of fibrous dysplasia has not been dis-covered.

MONOSTOTIC
70%-80% of fibrous dysplasia.
Occurs in rib, femur , tibia,
craniofacial bones and humerus.
Pain or pathologic fracture in 10-70yrs
Bone deformity less severe
Painless swelling of the jaw
Swelling involves labial or buccal
plate
Protuberant excrescence of inferior
border of mandible
POLYOSTOTIC
20%-30% of fibrous dysplasia.
Sites: Femur, tibia, pelvis, ribs, skull
and facial bones, upper
extrimites, lumbar spine, clavicle and
cervical spine .
Tends to occur in unilateral
distribution.
Involvement asymmetric and
generalized on bilateral lesions.
 Involves variable
number of bones,
accompanied by
pigmented lesions
of skin or café-au-
lait spots
Involves nearly all
bones in skeleton,
pigmented lesions
of skin or café-au-
lait spots and
endocrine
disturbances
Hyperthyroidsm,
hyperparathyroids,
cushing syndrome,
gonadotrophin-Mc-
Cune Albright
syndrome

JAFFE LICHENSTIEN MC-CUNE ALBRIGHT ENDOCRINE

SYNDROME SYNDROME DISTURBANCES ASSC.

CLINICAL FEATURES:

CAFE-AU-LAIT MAZABRAUDS MALIGNANCIES

SPOTS SYNDROME
Increased Fibrous Osteosarco Mc-
melanin in basal dysplasia and Cune albright
cells of intramuscular syndrome
epidermis myxoma, risk
of sarcomatous
malformation
Cutaneous Chonddrosarco
pigmentation Mc-Cune
seen ipsilateral albright
to side of bone syndrome
lesion
Occur at birth FibrosarcoMc-
precedes Cune albright
skeletal& syndrome
endocrine
diseases.
FibrosarcoMc-
Cune albright
syndrome
LAB FINDINGS:

No significant change
Elevated Alkaline Moderate increase in
in serum
phosphatase Basal Metabolic Rate
calcium/phosphorus

CRANIOFACIAL FIBROUS DYSPLASIA:

In 10-25% of pt. with Also in isolated Sites: frontal,sphenoid,

monostotic form. craniofacial form. maxillary,ethmoid
In 50% of pt. with No extracranial lesions bones.
polyostotic form present.

Hypertelorism, cranial
asymmetry, facial
Vestibular dysfunction,
deformity, visual
tinnitus, hearing loss.
impairment,
exophthalmos, blindness
HISTOLOGIC FEATURES:

Monostotic Polyostotic
Proliferating fibroblasts Lesions rich in spindle
in a compact stroma of shaped fibroblasts with a
interlacing collagen swirled appearance
fibres. within the marrow space
Irregular bony Islands of cartilaginous
trabeculae scattered tissue within lesions
throuout lesion .

No definite pattern. Affected bones may have

cystic lesions lined by
multinucleated giant cells
C-shaped or Chinese
character shaped .
Trabeculae usually
coarse woven bone

RADIOGRAPHICAL FEATURES:

• Network of fine bone trabeculae.

• Increased trabeculation – lesion more opaque & mottled appearance.
• Opaque with many delicate trabeculae: ’ground –glass’ or ‘peau d’ orange’
appearance.
• Cortical bone becomes thinned.
• Roots of teeth separated or moved out of normal position.
TREATMENT:

• Extent of skeletal involvement to be estimated.

1
• Radiographic surveys using plain films or technetium diphosphonate to determine
2 monostotic or polyostotic form of disease.

• Lesions exhibit a period of slowly progressive, persistent growth.

3

• Stabilization after the onset of puberty often follows.

4

• Small lesions may require only biopsy and periodic follow-up.

5
• Lesions resulting in functional or cosmetic disability may be treated by osseous
6 recontouring via a transoral approach
• Initiated following the active growth stage and during the period of stabilization of
7 the disease process
• En bloc resection for complete excision of fibrous dysplasia lesions is impractical and
8 unnecessary

• The disorder is non-neoplastic in nature

9

OSSIFYING FIBROMA

Replacement of normal bone by fibrous tissue and variable amount of newly formed bone and
cementum like structures.

CLINICAL FEATURES:

 Painless, slowly growing swelling

 Third and fourth decade of life
 Females are more commonly affected 5:1
 Mandible(premolar –molar ) most commonly affected
 Juvenile OF is a variant, occurring in children involving the craniofacial complex

NOTE: Peripheral ossifying fibroma= soft tissue counterpart of ossifying fibroma.

It represents a reactive hyperplasia of periodontal membrane fibres that also may induce some
bone formation

RADIOLOGICAL FEATURES:

 Well demarcated
 Unilocular with sclerotic border
 Can be radiolucent or radio-opaque
 Root resorption or divergence possible

HISTOLOGY:

 Collagenous stroma containing variable spindled or stellate cells

 Collagen fibers arranged in whorled/storiform pattern
 Basiphilic speheical calcifications and anastomosing trabeculae of cementum like
material.

TREATMENT:

 Intraoral approach for the surgical excision of tumor by enucleation is the preferred
method of management.
 Adjacent normal strcutures including teeth should be preserved whenever possible.
 Following large lesions with potential risk of post-operative fracture, intermaxillary
fixation is advised during initial healing stages.
 Root that are resorbed are removed
 The prognosis is good and the rate of recurrence is low.
 More extensive lesions whether primary or recurrent require definitive surgical
resection with bone grafting as definitive treatment.
GIANT CELL LESIONS

CENTRAL GIANT CELL GRANULOMA

 Initially described as central giant cell "reparative" granuloma, suggesting that the
disease process represents a reactive response to intrabone haemorrhage and
inflammation.
 The term central giant cell lesion has recently been proposed, as the microscopic
features are not those of a true granulomatous process."

CLINICAL FEATURES:

 Sixty percent to 75% of cases are diagnosed in patients younger than 30 years old
 Females > Males
 Almost exclusively in the maxilla and mandible, but isolated cases in small bones of the
hands and feet have been reported
 Mandible more frequently than in the maxilla
 Involve the anterior portion of the jaws, sometimes with ex-tension across the midline
 Produces expansion or swelling of the affected jaw
 Two categories: nonaggressive and aggressive
o Nonaggressive--- more common, asymptomatic, slow growing and fail to
demonstrate cortical perforation or root resorption.
o Aggressive----- rapidly growing, and producing cortical perforation, root
resorption, and, rarely, paraesthesia

RADIOGRAPHICAL FEATURES:

 Multilocular > unilocular radiolucency of bone

 Well demarcated margins , often presenting a scalloped border.
 Expansion and thinning of cortices are often noted
 Perforation and extension into soft tissues evident in some of the aggressive variants.

HISTOPATHOLOGICAL FEATURES:

Proliferation of spindled fibroblasts in a variably collagenous stroma

 Numerous small vascular channels are evident

 Multinucleated giant cells are present throughout the connective tissue stroma
 Giant cells are frequently aggregated in clusters around vascular channels
  Clinically aggressive giant cell granulomas demonstrate a denser distribution of
mononuclear and giant cells with less fibrovascular tissue
 Inflammatory cells are not typically seen unless they are secondary in nature

TREATMENT:

 Most widely accepted method of surgical treatment is aggressive curettage.

 Curettage of the tumor mass followed by removal of the peripheral bony margins
results in a low recurrence rate and good prognosis.
 Thorough and meticulous curettage of the lesion around the roots of involved teeth
should be accomplished.
 Central giant cell granulomas displaying aggressive clinical features have a greater
tendency to recur following conservative treatment. Definitive eradication of these
lesions may require more extensive surgery, including resection.
 Intralesional injections of corticosteroids have been proposed as a nonsurgical method
of management for central giant cell granuloma (56 Original reports indicated that this
approach resulted in a very high cure rate)
 Use of exogenous calcitonin may have some merit in the treatment of aggressive
lesions.

DIFFERENTIAL DIAGNOSIS:

Brown tumor of hyperparathyroidism---similar histologic features.

A diagnosis of central giant cell granuloma should prompt investigation of serum calcium,
phosphate, and parathyroid hormone levels.

BROWN TUMOR OF HYPERPARATHYROIDISM

• The term brown tumour comes from the colour of lesion, which results from the
vascularity, haemorrhage and deposits of hemosiderin.
• Therefore, the brown tumour is actually a kind of giant cell lesion and often appears as a
multiple and expansive osteolytic lesion of the bone.
• Because it is difficult to distinguish histopathologically brown tumour from other giant
cell lesions, a clinical diagnosis is made based on the association with HPT.
• Histologically, all giant cell lesions have two main components: mononu- clear stromal
cells and multinucleated giant cells.

HYPERPARATHYROIDISM:

• Overproduction of parathyroid hormone.

 • Primary hyperparathyroidism= uncontrolled production due to an adenoma,
hyperplasia
• Secondary hyperparathyroidism= in response to hypocalcemia due to chronic renal
failure

CLINICAL FEATURES:

• The lesions may be solitary or multifocal.

• It represents the terminal stage of the bone remodelling process during secondary HPT.
• They commonly affect the mandible, clavicle, ribs, and pelvis.
• In the craniofacial skeleton involvement of the maxilla, temporal bone, nasal cavity, and
paranasal sinuses may be seen.
• Involvement of the orbital bones has also been reported

RADIOGRAPHICAL FEATURES:

• Oral radiographic manifestations of HPT include a generalized loss of lamina dura

surrounding the roots of the teeth, loss of cortication around the inferior alveolar canal
and maxillary sinus, and of the trabecular pattern of the jaws.
• Peripheral manifestation of brown tumour on the oral cavity is rare

HISTOPATHOLOGY:

Histologically, all giant cell lesions have two main components:

• Mononuclear stromal cells
• Multinucleated giant cells.

DIAGNOSIS:

• Blood biochemistry investigations:

1. Serum calcium (normal range, 8.5–10.5).
2. Alkaline phosphatase (normal range up to 100).
3. Parathormone (normal range, 15–65).
4. Phosphorus (normal range, 2.4–4.1).
• Sestamibi scan of the parathyroid glands: Tc-99m, a mild and safe radioactive agent, is
injected intravenously and absorbed by the hyperactive parathyroid gland and detected
by scanning.

HOW TO APPROACH SUCH PATIENTS:

1. Pay attention to clinical signs/ symptoms.

2. Groans, stones and bones.
3. Serum calcium and phosphate, PTH, alkaline phosphatase are done.
 4. Raised PTH, raised serum Ca and raised ALP= primary hyperparathyroidism.
5. Raised PTH, Low serum Ca, Normal ALP, Renal functions abnormal= secondary
hyperparathyroidism.

TREATMENT:

• In Primary hyperparathyroidism, hyperplastic mass or functional tumor must be

removed surgically to reduce PTH levels to normal.
• In Secondary hyperparathyroidism, restriction of dietary phosphate, use of phosphate
binding agents and pharmacological treatment with an Vitamin D metabolite. Patients
who do not respond to medical treatment could undergo parathyroidectomy. Renal
transplantation may restore normal physiologic processes.
• The treatment for brown tumor in the jaws includes enucleation and curettage, radical
resection and reconstruction, radiation therapy, and chemotherapy. Surgical excision of
the brown tumors is indicated for large and disfiguring lesions and in case that the
affected bone is weakened. However, some authors initially treat this lesion with
systemic corticosteroids and, when it reduces its size, they make its surgical excision.

CHERUBISM

 Cherubism is a rare inherited condition affecting the jaws.

 Characterized by replacement of normal bone by a proliferation of fibrovascular tissue
containing multinucleated giant cells.
 Patients exhibit bilateral, symmetric expansion of the affected bones, resulting in a
characteristic facial fullness.
 Round, full face and exposure of the lower sclera with the resulting gaze of "eyes up-
turned toward heaven"
 Autosomal dominant disorder.

CLINICAL FEATURES:

 Identified be-tween the ages of 2 and 7 years

 Grade 1 disease reflects involvement of bilateral mandibular ascending rami
 grade 2 disease consists of bilateral maxillary tuberosity involvement in addition to
lesions of mandibular rami
 More extensive, generalized involvement of posterior and anterior regions of the
mandible and maxilla is grade 3 disease.
 Grade 4 when anatomic sites involved includes coronoid processes and condyles (rarely)
 Pain-less, typically slow expansion
 The bony expansion is firm and nontender to intraoral palpation
  Pre-mature exfoliation of primary teeth may occur. The developing permanent teeth in
areas of involvement are usually displaced, malformed, or absent
 Regional lymphadenopathy has been reported as a common finding in patients with
cherubism.

RADIOGRAPHIC FEATURES:

 Well-defined, multilocular radiolucencies

 Lesions characteristically present in a bilaterally symmetric fashion (Fig. 13-33). small,
bilateral, multilocular radiolucencies of the posterior mandible grade 1 cherubism
 As lesions progress, it produces expansion and thinning of cortical bone.
 Perforation noted occasionally.
 Displaced and malformed evident within the multilocular radiolucent lesions

HISTOPATHOLOGY:

 Proliferation of loose fibrovascular tissue with aggregates of multinucleated giant cells.

 Lesional tissue consists of uniform spindled to ovoid fibroblasts within a loosely
collagenous, well-vascularized stroma.
 A distinctive feature of cherubism is the presence of an acellular, eosinophilic deposit
that appears to ring small vascular channels.
 Not a sensitive marker, as the eosinophilic cuff is absent in many cases.

NOTE: These features are similar to those of other giant cell—containing lesions of jawbones,
including giant cell granuloma. For this reason, familial, clinical, and radiographic correlation is
required for a definitive diagnosis in most cases

TREATMENT AND PROGNOSIS:

 The degree and rate of clinical progression in a given patient usually determine the
approach to management of cherubism.
 Surgical intervention consisting of conservative curettage or debulking of lesions with
surgical recontouring is the treatment of choice.
 Multiple procedures may be necessary to provide the desired functional and aesthetic
results.
 The decision regarding treatment for children with extensive, rapidly growing lesions is
difficult.
 Radiation therapy is not indicated as a treatment for cherubism.

NOTE: An association between cherubism and a Noonan-like syndrome has been documented."
exhibiting the clinical, radiographic, and histopathologic features of cherubism. Individuals
show variable expression of a variety of clinical abnormalities, including short stature, cardiac
defects, mental retardation, and a characteristic facies. The approach to management of the
jaw lesions is similar to that for classic cherubism.

ANEURYSMAL BONE CYST

 May develop as a primary disease process or as a secondary lesion in a pre-existing bone

lesion.
 Associated bone lesions a with the secondary development of ABC include fibrous
dysplasia, ossifying fibroma, central giant cell granuloma, osteoblastoma, and
osteosarcoma.
 Secondary aneurysmal bone cysts have been reported to represent between 30% and
50% of cases.

ETIOPATHOGENESIS:

Secondary lesions represent an injury to the capillary network of the primary lesion.

The resulting "blowout" reaction caused by capillary pressure from the extravascular blood
produces the expansile, destructive aneurysmal bone cyst.

CLINICAL FEATURES:

 Younger than 30 years of age, with a peak incidence in the second decade of life.
 Mandible > Maxilla
 Posteriorly > Anteriorly
 Jaw lesions: Females > Males
 Other parts of the skeleton: No gender predilection
 Typically, nonpulsatile and without bruits despite the predominance of a vascular
component

RADIOLOGICAL FEATURES:

 unilocular or multilocular radiolucency

 A characteristic radiographic feature has been described as a "ballooning" distention of
periosteum with a thin outline of reactive, subperiosteal bone."
 When developing in the tooth-bearing regions of the jaws, tooth displacement or
external root resorption is evident.
HISTOPATHOLOGY:

 The grossly like soft tissue mass filled with considerable dark, venous blood.
 Numerous cavernous, sinusoidal spaces filled with blood are surrounded by loose,
fibrous connective tissue.
 The connective tissue septae contain small capillaries, multinucleated giant cells,
inflammatory cells, extravasated erythrocytes, and hemosiderin.
 multinucleated, osteoclast-like giant cells
 One immunohistochemical study demonstrated the focal presence of endothelium.

TREATMENT:

 Complete surgical removal using curettage is the most commonly employed treatment
for aneurysmal bone cysts."
 Recurrence rates for all skeletal sites range from 20% to 70%, although recurrences in
the jaw may not be as frequent as in other bones.
 cauterization or cryotherapy of the bony cavity following removal of the lesion is
generally advocated.
 Life-threatening haemorrhage does not typically occur.
 Significant blood loss may develop in large lesions until the tissue is completely
removed.
 En bloc excision may be required for treatment of recurrent lesions or for large,
relatively inaccessible aneurysmal bone cysts.

LANGERHANS CELL HISTIOCYTOSIS

 Langerhans Cell histiocytosis formerly known as histiocytosis X affects children and

young adults.
 The disease is caused by a proliferation of antigen presenting cells that are monoclonal
CD1A positive cells of unknown resistance.
 Pain and swelling are first common presenting complaints.
 When alveolar bones are involved teeth may become loose and surrounding soft tiisue
become inflamed and hyperplastic
 Radiographically, well circumscribed radiolucent lesions.
 Radiograph appearance of floating teeth is common
 In 1953, Lichtenstein classified these diseases as histiocytosis X and grouped them into
three major categories:
 o Eosinophilic granuloma (chronic focal): involves lytic bone lesions without
visceral involvement. When present in maxillofacial region, patient complains of
pain, swelling, non healing gingival lesions and loose teeth.
o Hand-schuller- Christian syndrome (chronic disseminated): involves bone, skin
and viscera. Classic clinical triad includes multiple lytic bone lesions,
exophthalmos, diabetes insipidus.
o Letterer-siwe syndrome (acute disseminated): prominent in infants with
cutaneous, visceral and bone marrow involvement. Pancytopenia,
lymphadenopathy, liver and splenic involvements.

HISTOPATHOLOGICAL FEATURES:

 Proliferation of large cell with abundant cytoplasm, indistinct cell borders and oval to
reniform nuclei
 Admixed with plenty of eosinophils and other inflammatory cells.
 Electron microscopically, proliferated cells are significant because of the presence of rod
shaped cytoplasmic structure called Birkbeck granules.
 Immunohistochemically, expression of CD1A antigen, S-100 protein, human leukocyte
antigen DR can provide useful diagnostic information.

TREATMENT:

 A definitive diagnostic of LCH is made through incisional or excisional biopsy, cytological

examination, electron microscopy and immunohistochemical markers for CD1A positive
antigen.
 Clinical extent of the disease determines the treatment.
 If multisystemic disease is absent, local disease can be treated with excisional biopsy,
low-dose radiotherapy or mild chemotherapy.
 Several option to treat this disease includes: Surgery (simple biopsy or curettage),
radiation (200-1200 cGy), chemotherapy, intralesional corticosteroids and no treatment.
 With involvement of multiple bone site, treatment follows and aggressive approach.
o Egeler and D’ Angio divided the disease …..restrictive and extensive
o Restrictive: chronic focal and chronic disseminated. Initial treatment
unnecessary because many monostotic lesios regress spontaneously. Biopsy
stimulates healing with or without curettage. Further treatment dictated by
clinical symptoms. Polyostotic LCH without visceral involvement can be treated
with curettage or intralesional corticosteroids. Foow up recommended due to
progression/ recurrence of the disease.
o Extensive: chronic disseminated and acute disseminated. Systemic
chemotherapy. Monotherapy or combination regimens of agents including
 chlorambucil, cyclophosphamide, daunomycin, mercatopurince, methotrexate,
vinblastine and vincristine-with or without steroids.

OSTEOMA

Benign tumors consist primarily of mature, compact, cancellous bone.

some lesions likely represent true neo-plasms of bone, other lesions may be alter-ations of
bone as a response to trauma or infection.

CLINICAL FEATURES:

 Most commonly affect the craniofacial and jaw bones.

 Less commonly, surface of the long bones and clavicles.
 Most commonly identified in young adults, with the peak incidence between the 2 nd and
5th decades.
 conflicting gender predilection (some reports indicate an increased frequency in males,
although a marked female predominance was noted in one study of osteomas of the
jaws.)
 Lesions may arise in the maxilla or mandible
 Lesions that develop within the paranasal sunuses may produce symptoms such as
recurrent sinusitis, headaches, or ophthalmologic complaints, depending on the exact
site of the osteoma.
 Endosteal osteomas
o small and asymptomatic
o Some demonstrate progressive growth, producing gradual expansion of the
affected area.
 Periosteal Osteomas
o Osteomas that arise on the surface of the bone are referred to as periosteal
osteomas
o Periosteal osteomas…..slow-growing, asymptomatic, bony, hard masses.
o Asymmetry of the affected bone
o Affecting the angle of the mandible.
o Osteomas that develop centrally within medullary bone are called endosteal
osteomas or enostoses.

The presence of multiple osteomas of the jaws or sino-orbital region should prompt further
investigation into the possibility that the patient has Gardner's syndrome

Gardner’s Syndorme:
o Autosomal dominant disorder.
 o The defective gene, the adenomatous polyposis coli gene, is present in a small region on
the long arm of chromo-some 5 (5q21).
o Most common manifestations adenomatous polyps of the gastrointestinal tract multiple
osteomas, dermal and mesenteric fibrous tumors, and epidermal and trichilemmal cysts
of skin.
o Hyperplastic alterations of retinal pigmented epithelium which may precede the colonic
and osseous lesions.
o Dental abnormalities such as impacted permanent and supernumerary teeth and
odontomas.
o The gastrointestinal polyps typically arise in the colon a rectum but can also affect the
stomach a duodenum. The adenomatous polyps have a virtually 100% chance of
transforming to adenocarcinoma unless prophylactic colectomy is performed.

RADIOGRAPHIC FEATURES:

• Well-circumscribed, densely sclerotic, radiopaque masses.

• Some lesions may demonstrate a circumscribed, sclerotic periphery with a mixed,
trabeculated radiographic pattern centrally.

HISTOPATHOLOGY:

Two different microscopic patterns of osteoma of the jaws have been described.

1. One type is composed of relatively dense, compact bone with sparse marrow tissue.
The bone is mature and lamellar, with osteons and haversian canals.
2. Second type consists of lamellar trabeculae of cancellous bone with abundant fibrofatty
marrow between bony trabeculae.

Some sporadic osteomas of the paranasal sinuses and facial bones are composed of dense,
immature bone that may exhibit areas of osteoblastic and osteoclastic activity, which suggests
osteoblastoma-like features.

TREATMENT AND PROGNOSIS:

• Periodic clinical and radiographic examination -> Small, non-progressing, asymptomatic.

• Surgical intervention for large and deforming, progressively growing lesions, or are
associated with symptoms such as pain, sinusitis, headaches, and ophthalmologic
abnormalities.
• Conservative surgical excision is appropriate treatment.
• Endosteal osteomas of the jaws may be treated with enucleation using an intraoral
approach.
• Periosteal osteomas are more likely to require surgical treatment, as they tend to
produce facial swelling and asymmetry. Conservative surgical excision is again the
treatment of choice.
• Osteotomes may be used to separate the osteoma from the attachment to the
underlying cortical bone.
• Periosteal osteomas of the posterior mandible may require an extraoral surgical
approach. Recurrence following surgical excision is not expected.
• Patients with multiple osteomas of the jaws and craniofacial bones require a thorough
work-up for evidence of Gardner's syndrome.
• The clinical progression and treatment of the adenomatous polyps or adenocarcinoma
of the gastrointestinal tract are of primary importance in determining the prognosis.

OSTEOBLASTOMA

• Osteoblastoma is an uncommon lesion of bone considered by most authorities to

represent a benign neoplastic process.
• The designation osteoblastoma…..lesions that are greater than 2 cm in diameter.
• Support for the apparent neoplastic nature of osteoblastoma came from a report of
chromosomal translocation in one case of osteoblastoma.

CLINICAL FEATURES:

• Most commonly arises in vertebrae and long bones such as the proximal femur and
humerus.
• Axial skeleton more commonly peripheral skeleton.
• craniofacial bones are affected in 11% to 15% of cases.
• Posterior mandible is the most frequent site in the head and neck: condyles and
coronoid processes are only rarely affected.
• 90% of reported cases presenting before the age of 30 years.
• Distinct male predominance of 2:1.
• Pain, often quite severe, is the most common presenting symptom.
• The lesion may or may not produce cortical expansion and swelling. When swelling is
evident, the area may be tender to palpation, although mucosal ulceration is
typically absent.
• As lesions enlarge and replace normal bone, the associated teeth may become
mobile.
NOTE: In contrast to osteoid osteoma, the pain associated with osteoblastoma is not
effectively relieved with aspirin and nonsteroidal anti-inflammatory agents."
The timing of the pain may also be more variable in nature, without the characteristic
nocturnal pain pattern of osteoid osteoma.
 RADIOLOGICAL FEATURES:

• Well defined, round to ovoid, mixed radiolucent-radiopaque lesions

• The degree of calcification of the central tumor mass may be minimal, in which case
the lesion is primarily radiolucent.
• When the amount of calcification is more extensive, the central opaque lesion is
usually surrounded by a thin radioucent rim.
• Vast majority in intramedullary locations, a few cases have been reported in
periosteal sites.
• A "sun-ray" pattern of new bone formation, as can be seen in various malignant
bone tumors, may be evident in these lesions.

NOTE: Sclerosis of perilesional bone, a relatively constant feature of osteoid osteoma, is

generally absent in osteoblastoma.

DESMOPLASTIC FIBROMA

 Benign, locally aggressive, solitary lesion of bone.

 The tumor is rare and affects the long bones and pelvis although recent reports indicate
that the mandible may be the most common site of skeletal involvement

CLINICAL FEATURES:

 Younger than 30 years of age, with the peak incidence in the second decade of life.
 No apparent gender predilection. Mandible more frequently than maxilla.
 The body, angle, and ramus regions of the mandible are the most common sites of
involvement.
 Slowly progressive, asymptomatic enlargement of the affected portion of the bone.

RADIOLOGICAL FEATURES:

 Large unilocular or multilocular radiolucency.

 Poorly defined margins with cortical thinning and perforation.
 Soft tissue extension may occur and result in the presence of a palpable tumor mass.
 Extension into muscles of mastication causing trismus been noted.
 Root resorption is common.
HISTOPATHOLOGICAL FEATURES:

 Spindled, elongated fibroblasts in an abundant, densely collagenous matrix.

 Irregular, poorly defined, infiltrative margins.
 The production of bone is not a feature of this tumor.
 Ultrastructural and immunohistochemical studies indicate proliferating cells in
desmoplastic fibroma displaying myofibroblastic differentiation.

TREATMENT:

 Exhibits locally aggressive biologic behavior, although it does not metastasize.

 Most reported cases were treated initially with curettage, which has been associated
with a recurrence rate of 30% to 40%.
 Despite this potential for recurrence, some advocate thorough curettage as adequate
treatment for lesions that have not perforated cortical bone in patients available for
close, frequent follow-up.
 Others suggest that initial treatment of desmoplastic fibroma should consist of surgical
resection.