Malignant Bone Neplasms

8/8/2019 Malignant Bone Neplasms

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Osteosarcoma is a malignancy of mesenchymal cells that have the ability to produce osteiod or immature bone .

Excluding hematopoietic neoplasms, osteosarcoma is the most

common malignancy to originate within the bone.

Two forms -Osteoblastic or sclerosing type and osteolytic type.

Majority having intramedullary origin but a small number may

be juxtacortical.



Occurs chiefly in young persons,

majority between the age 10 and

25 years.

Males affected more frequently than females.

Predominant site -long bones

chiefly the femur and tibia,

In older patients the axial skeleton and flat bones are

involved most frequently,.



R epresent 6% to 8% of all osteosarcomas. Occur most often in third and fourth

decades of life, with the mean age of 33 years.

Maxilla and mandible involved with about equal frequency.

Mandibular tumors arise more frequently in the posterior body and horizontal ramus.

Maxillary lesions -in the inferior portion i.e.alveolar ridge, sinus floor and palate.

Swelling and pain -most common symptoms.

Loosening of teeth, paraesthesia and nasal obstruction (in case of maxillary tumors)



Periapical, occlusal and panoramic

radiographs can be taken.

SCLE R OSING TYPE:

Excessive bone production.

In some cases, irregular spicules or

trabeculae of new bone may be seen radiating outwards on the

periphery of the lesion, producing

the so called " SUN- RA Y"

appearance .



OSTEOLYTIC TYPE:

Destructive one, producing an irregular radiolucency and

demonstrating both expansion of

cortical plates and destruction.

A n important early radiographic

change- symmetric widening of the

periodontal ligament space around a

tooth or several teeth due to result of

tumor infiltration along the

periodontal ligament space:



If the internal structure is minimal or absent- fibrosarcoma or

metastatic carcinoma If osseous structure is visible - chondrosarcoma

If speculated periosteal new bone is present - prostate and breast

metastases

Benign tumors such as ossifying fibroma and benign conditions

such as fibrous dysplasia may mimic osteosarcoma; however these

conditions are usually better demarcated and have a more

uniform internal structure



The treatment of osteosarcomas must be radical if there is to be

hope of curing the patient.

Treatment is by early mandibulectomy or maxillectomy together with wide excision of any soft-tissue extensions of the tumors.

May be combined with radiotherapy and/or chemotherapy .

The prognosis depends mainly on the extent of the tumor at

operation and The 5-year survival rate may range from 40% for

tumors less than 5cm in diameter to zero for tumors over 15cm.



Originates adjacent to the cortex of the bone, initially grow

outward from the surface not involveing the underlying

medullary cavity.

The P AR SOTE A L type is a lobulated nodule attached to the

cortex by a short stalk. There is no elevation of the periosteum

and no peripheral periosteal reaction. It is a low-grade

sarcoma having a small risk of recurrence and metastasis.

The PE R IOSTE A L type is a sessile lesion that arises within

the cortex and elevates the over lying periosteum often, the

leading edge of the tumor mass perforates the surface of the

periosteum and extends into surrounding soft tissue.



Characterized by the formation of cartilage , but not bone, by

the tumor cells.

Comprising about 10% of all primary tumors of skeleton but

rarely involving the jaws.

A bout half as common as osteosarcoma and about twice as common as Ewing's Sarcoma.



A rise ore frequently in the axilla.

A painless ass or s elling.

ay be associated ith separation or loosening of teeth.

axillary tu ors ay cause nasal obstruction, congestion,

epistaxis, photophobia, or visual loss.



Consists of a radiolucent process with poorly defined borders.

The radiolucent area often contains scattered and variable amounts of radiopaque foci, which are caused by calcification or ossification of the

cartilage matrix.

Penetration of the cortex can result in a sunburst pattern.

R oot resorption or symmetric widening of the periodontal ligament space of the teeth

They may grow in a lobular pattern with minimal or no foci of

calcification, appearing as a multilocular radiolucency.



Ill-defined radiolucent lesion of posterior mandible

containing radiopaque foci



Osteosarcoma -radiographically indistinguishable.

A lthough the typical calcifications of chondrosarcoma

may be absent from osteosarcoma.

Fibrous dysplasia - the radiopaque portion is abnormal

bone and not calcification.

Periphery of fibrous dysplasia is better defined and its

margin from adjacent teeth differs from that of

chondrosarcoma.



Grade II Chondrosarcoma

Moderately sized nuclei and increased cellularity, particularly

about the periphery of lobules. Low mitotic rate.

Grade III Chondrosarcoma

Highly cellular and may show a prominent spindle cell

proliferation.



Prognosis related to the size, location and grade of the lesion.

The most effective treatment -radical surgical excision.

R adiation and chemotherapy -less effective and primarily used

for unrespectable high-grade chondrosarcoma.

Local recurrence leads to death by direct extension of the tumor into vital structures of head and neck.



A lso called as 'Endothelial Myeloma' or ' R ound cell Sarcoma'.

Composed of small undifferentiated round cells of uncertain

histogensis.

6% to 8% of all primary malignant bone tumors

Third most common osseous neoplasm after osteosarcoma and chondrosarcoma.



Second decade of life.

Slight male predominance

The long bones, pelvis and ribs are

affected most frequently.

Pain, often associated with

swelling -most common symptom.



Intermittent pain varying from dull to severe.

Fever, leukocytosis and an elevated ES R .

Facial neuralgia and lip paraesthesia

Soft tissue mass overlying the affected area of bone.

More common in the mandible than the maxilla.

Paraesthesia and loosening of teeth.



Irregular lytic bone destruction with ill-defined margins.

Cortical destruction or expansion may or may not be present.

´ onion-skin" appearance the film.

Thickened cortex infiltrated by the tumor.

Osteophyte formation



O steo yelitis ay share so e of the radiographic features of

ing·s sarco a; ho ever osteo yelitis is likely to have

de onstrable sequestration present ithin confines of the lesion,

hereas ing·s sarco a does not.

In case of eosinophilic granulo a of the ja , la inar periosteal

reaction is present in this condition, not in ing·s sarco a.

he other central pri ary alignancies of bone such as

osteosarco a, chondrosarco a and fibrosarco a



Composed of solid sheets or mass of small round cells with very little

stroma.

Small and round cells with little

cytoplasm and relatively large round or ovoid nuclei..

Broad sheets of small round cells

with well-defined nuclear outlines

and ill-defined cytoplasmic borders. Tiny vascular channels

A bsence of multinucleated giant

cells.



Combined surgery, radiotherapy and multi drug chemotherapy.

Because of the risk of post radiation sarcomas, sometimes

radiation is not recommended.

It frequently metastasizes to the lungs, liver, lymph nodes and other bones.

The anatomical location of the tumor is a critical factor in

prognosis.

Pelvic lesions are associated with poorest diagnosis. Distal lesions have a better prognosis than those in a proximal location.



Most common form of cancer involving the bone.

Carcinoma of lung, breast, prostate, thyroid and kidney give rise

to majority of gnathic metastasis.

Metastatic spread of a carcinoma to jaws usually occurs by

haematogenous route.

Sarcomas arising in soft tissues and other bones may metastasize

to the jaws, but this is very rare.



Most patients are older,

The vertebrae, ribs, pelvis and skull -most frequent sites for

metastasis.

The jaws - uncommon sites for metastasis.

Metastasis to maxilla uncommon

Pain, swelling, loosening of teeth, a mass or paraesthesia.

Metastasis to the mandible with involvement of the inferior alveolar nerve producing NUMB-CHIN SYND R OME having

unexplained loss of sensation in the lower lip .



In some instances, the patient may be completely

asymptomatic and the diagnosis occurs only after

radiographic examination.

A n osseous metastasis may occur in a non-healing

extraction site from which the tooth was recently removed

because of complaints of local pain or significant mobility.



A ppear as radiolucent defects.

May be well-circumscribed resembling a cyst, but more often is

ill defined with a " MOTH-E A TEN" appearance.

Widening of periodontal ligament.

Some carcinomas, particularly from the prostate and breast,

may stimulate new bone formation in the metastatic site,

resulting in radiopaque or mixed radiolucent and radiopaque

lesion



Panoramic radiograph showing destruction of alveolar bone surrounding the roots of Mandibular 2 nd

molar.



Multiple myeloma- border is usually better defined than in

metastatic disease.

Periapical inflammatory lesion -periodontal ligament space widening from inflammatory lesion is centered about the

apex of root. In contrast the malignant tumor causes

irregular widening which may extend up the side of the root.

Squamous cell carcinoma - differentiated by clinical

examination.



V arying microscopic appearance

In some instances, the

metastasis tumor is well

differentiated and closely resembles a colcannon of a

specific site, such as kidney,

colon or thyroid.

However, metastatic

carcinomas are poorly

differentiated



Uncommon malignancy of plasma cell origin that often

appears to have a multicentric origin within bone.

Neoplasm of bone that originates from cells of the bone

marrow which bear a remarkable to plasma cells.



Disease of adults, with men being affected slightly more often than women.

Median age at diagnosis -between 60 and 80years

Twice as frequently in blacks as whites.

Bone pain is the most characteristic presenting symptom.

Fever may be present as a result of neutropenia with increased

susceptibility to infection.

Metastatic calcification may involve the soft tissues.R enal failure



Mandible is more frequently involved than the maxilla. The ramous angle and molar region of mandible -most frequent

sites of the lesion.

Sites classically affected include the oral mucosa, particularly

tongue. The tongue may show diffuse enlargement and firmness or may

have more of a nodular appearance.

Pain, swelling, and expansion of the jaw, numbness and mobility of tooth.

In addition, extra osseous lesions occur which may resemble gingival enlargements

Extension of the disease to other sites, outside the skeleton such as to lymph nodes, skin and viscera also occurs.



Numerous sharply punched out areas.

May vary in size from a few

millimeters to a centimeter or more in diameter, but there is usually no

peripheral bone reaction.

Diffuse distribution lesions of bone

may also occur.



Metastatic carcinoma ² knowledge of prior malignancy may help to differentiate from multiple myeloma.

Osteomyelitis ² a visible cause for it usually exists. A dditionally,

osteomyelitis causes sclerosis in adjacent bone, where multiple

myeloma does not. Simple bone cysts ²usually corticated in part and characteristically

interdigitate between the roots of the teeth in a much younger

population.

Metabolic diseases such as thalassemia, gaucher,s disease ² ruled out on the basis of medical history.



Composed of sheets of closely packed cells resembling plasma cells.

These are round or ovoid cells with

eccentrically placed nuclei exhibiting

chromatin dumping in a "cart wheel"

or "Checker board" pattern".

A perinuclear halo may be present.

R ussell bodies are common as in chronic inflammatory lesions with

numerous typical plasma cells.



Chemotherapy -treatment of choice.

R adiation therapy also used, often in combination with

chemotherapy.

Unfortunately, the prognosis is poor, with the median

survival time being two to three years.



A lso called Histiocytosis X, Eosinophilic granuloma, Langerhans

cell Granuloma.

It includes a spectrum of clinicopathologic disorders characterized

by proliferation of histiocyte-like cells that are accompanied by varying numbers of eosinophils, lymphocytes, plasma cells and

multinucleated giant cells.

The distinctive histiocyte-like cells present in this lesion are called

Langerhans cells and the condition as Langerhans cells Histiocytosis.

Langerhans cells are dendritic mononuclear cells normally found in

the epidermis, lymph nodes and bone marrow.



Primarily occurs in older children and young adults.

Male to female ratio is 2:1

The clinicopathologic spectrum includes the following.

Monostotic or polyostotic eosinophilic granuloma of bone- Solitary or multiple bone lesions without visceral

involvement.

Chronic Disseminated Histiocytosis- A disease involving bone, skin and viscera (Hand-

Schuller Christian disease). A cute Disseminated Histiocytosis:- A disease with prominent cutaneous, visceral and

bone marrow involvement occurring mainly in infants. (Letterer-Siwe disease).

l ll h l h l



Clinically, the lesion may present no physical signs or

symptoms and may be found only upon on incidental

radiographic examination of bones of the head or other areas.

On the other hand, there may be local pain, swelling and tenderness.

The lesion may occur in the jaw and overlying soft tissues of

mouth.

A lthough the skull and mandible are common sites of involvement the femur, humerus, ribs and other bones may

also be affected.

General malaise and fever.



The lesions are destructive and are well demarcated, roughly

round or oval in shape.

The area destroyed is replaced by soft tissue, the composition of

which varies, depending upon the stage at which the lesion is

examined.

The tissue of early lesion is soft and brown and, since there is

no necrosis, is not friable.

Later the lesion becomes fibrous and the grayish.



Periodontal disease ² the epicenter of bone destruction in LCH is approximately in the midroot region. In contrast the bone

destruction in periodontal disease starts at the alveolar crest

and extends apically down the root surface.

Squamous cell carcinoma ² the borders of LCH lesion are typically well defined.

Simple bone cysts ² the alveolar crest is maintained in simple

bone cysts and a partial cortex may be present.

Solitary intraosseous lesions ² differentiated by well defined borders and the periosteal reaction seen in Histiocytosis.



Diffuse infiltration of large, pale- staining mono-nuclear cells that resemble histiocyte.

Indistinct cytoplasmic borders

and rounded or indented vesicular nuclei.

V arying numbers of eosinophils are typically interspersed among the histiocyte-like cells.

Plasma cells, lymphocytes and multinucleated giant cells are often seen, and areas of necrosis and hemorrhage may be present.



A ccessible bone lesions, such as those in the maxilla and

mandible -treated by curettage.

Low doses of radiation -for less accessible bone lesion

Intralesional injection with corticosteroids in case of

localized bone lesions.

The Prognosis for bone lesions in the absence of

significant visceral involvement is generally good.



A lso called as Epidermoid carcinoma .

Most common oral malignancy which may be defined as a

malignant tumor arising from surface epithelium.

Characterized initially by invasion of malignant epithelial cells

into the underlying connective tissue with subsequent spread into

deeper soft tissues, adjacent bone, local regional lymph nodes and ultimately to distant sites such as the lung, liver and skeleton.



Occurs in persons older than 50 years. Males are more commonly affected than females.

It commonly involves the lateral border of the tongue, therefore a common site to observe bone invasion is the posterior mandible.

Lesions of lip and floor of mouth may invade the

anterior mandible. Squamous cell carcinoma appears initially as

white or red irregular patchy lesions of affected epithelium..

With time, these lesions exhibit central ulceration; a rolled or indurated border, which represents peripheral invasion of malignant cells; and palpable infiltration into adjacent muscle or bone.

Pain may be variable, regional lymphadenopathy characterized by rubbery hard lymph nodes.



Other clinical features include a soft tissue mass,

paraesthesia, anesthesia, dysesthesia, pain, foul smell,

trismus, grossly loosened teeth or hemorrhage.

Large lesions can obstruct the airway, Eustachian tube or

the nasopharynx.

Patients often report a significant weight loss and feel unwell



Squamous cell carcinoma may erode into underlying bone from any direction, producing a radiolucency that is polymorphous and

irregular in outline.

If bone involvement is extensive the periphery appears to have finger like extension preceding a zone of osseous destruction.

Sclerosis in underlying osseous structures may be seen in

association with erosions from surface carcinomas.



Evidence of invasion of bone around teeth may first

appear as widening of periodontal ligament space with loss of adjacent lamina dura.

Teeth may appear to float in a mass of radiolucent soft

tissue.

Destruction of adjacent normal cortical boundaries such

as the floor of the nose, maxillary sinus or Buccal or

lingual Mandibular plate may occur.



It is characterized by invasive islands and cords of malignant squamous epithelial cells.

Invasion is represented by irregular extension of lesional epithelium through the basement

membrane and into subepithelial connective tissue.

Individual squamous cells and sheets or islands of cells appear as individual entities within connective tissue. They may extend deeply into underlying adipose tissue, muscle

or bone. The lesional cells generally show abundant

eosinophilic cytoplasm with large, often darkly staining nuclei and increased nuclear- to-cytoplasmic ratio.



Combination of surgery and radiation therapy. The choice of treatment depends upon the protocol of the treating

centre and the location and severity of the tumor.

Generally, if an adequate margin of normal tissue can be obtained,surgery is the usual treatment, followed by radiation treatment.

A n alternative is to use radiation as the primary treatment followed by surgical salvage.

Currently, chemotherapy is used as an adjunct to either radiation or surgical treatment.

The prognosis for survival depends upon tumor stage. The 5-year disease free survival rate for intraoral carcinoma is 76% if metastasis has not occurred at the time of diagnosis (stage1 and II), 41% when cervical nodes are involved (stageIII) and 9% when metastasis below clavicle is present.



A lso called Primary intraosseous carcinoma, intra alveolar

carcinoma or central mandibular carcinoma.

Primary intraosseous carcinoma is a squamous cell carcinoma arising within the jaw that has no original

connection with the surface epithelium of oral mucosa.

It is presumed to arise from intraosseous remnants of the odontogenic epithelium.



These neoplasms are rare and may remain silent until they have reached a fairly large state.

It mainly occurs in fourth to eighth decades of life and more

common in males.

The mandible is far more commonly involved than maxilla.Posterior region are involved more frequently than anterior

region.

A s the lesion is associated with remnants of dental lamina, it

originates only in tooth bearing parts of jaw. The surface epithelium is invariably normal in appearance.

Pain, pathologic fracture, and sensory nerve abnormalities such

as lip paraesthesia and lymphadenopathy



The periphery of majority of lesions is ill- defined, although some may be well defined.

They are most often rounded or irregular in shape and have a border that demonstrates osseous destruction and varying degrees of extension

at periphery. The degree of raggedness of the border may reflect the aggressiveness of

lesion.

The internal structure is wholly radiolucent with no evidence of bone production and very little residual bone left within the centre of the lesion.

These lesions are capable of causing destruction of antral or nasal floors, loss of cortical outline of the mandibular canal and effacement of the lamina dura. R oot resorption is unusual.



If the lesions are not aggressive and have a smooth border

and radiolucent area they may be mistaken for Periapical

cysts and granuloma.

If the lesions are not centered about the apex of a tooth,

then it is difficult to differentiate from odontogenic cysts

and tumors.



It is characterized by invasive islands and cords of

malignant squamous epithelial cells.

The lesional cells generally show abundant eosinophilic cytoplasm with large, often darkly staining nuclei and

increased nuclear-to-cytoplasmic ratio.

V arying degree of cellular and nuclear pleomorphism is seen. Keratin pearls may be produced into lesional cells.



Generally, these tumors are excised with their surrounding

osseous structure in an en-bloc resection.

R adiation and chemotherapy can be used as adjunctive

therapies.



A lso called as Epidermoid cell carcinoma or ex odontogenic

cyst.

This condition may arise from inflammatory, Periapical,

residual, dentigerous and odontogenic keratocysts.

Histologically, the lining squamous epithelium of the cyst gives rise to the malignant neoplasm.



This tumor may occur anywhere an odontogenic cyst is found,namely, tooth-bearing portions of the jaws.

Most cases occur in the mandible, with a few cases reported in

the anterior maxilla.

The most common presenting sign or symptom associated with condition is pain. The pain may be characterized as dull and of

several months duration.

Swelling is occasionally reported.

Pathologic fracture may occur, as may fistula formation and regional lymphadenopathy.

If the upper jaw is involved, sinus pain or swelling may be

present



A s the lesion arises from a cyst, the shape is often round or ovoid.

If it is a small lesion in the cyst wall, the periphery may be mostly

well defined and even corticated. A s the malignant tissue

progressively replaces cyst lining, the smooth border is lost or becomes ill-defined.

The advanced lesion has an ill-defined, infiltrative periphery that

lacks any cortication.

It is capable of thinning and destroying the lamina dura of adjacent teeth or adjacent cortical boundaries such as inferior border of jaw or

floor of the nose.

It may result in complete destruction of the alveolar process.



If a dental cyst is infected, it may loose its corticated

boundary and appear ragged and identical to a malignant

lesion arising in a preexisting cyst. However inflamed

cysts usually show a reactive Periapical sclerosis, this is

not normally present in a cyst which has undergone

malignant transformation..



It is characterized by invasive islands and cords of

malignant squamous epithelial cells.

The lesional cells generally show abundant eosinophilic

cytoplasm with large, often darkly staining nuclei and

increased nuclear-to-cytoplasmic ratio.

V arying degree of cellular and nuclear pleomorphism is

seen. Keratin pearls may be produced into lesional cells.



Generally, these tumors are excised with their surrounding

osseous structure in an en-bloc resection.

R adiation and chemotherapy may be used as adjunctive

therapies.



Melanoma is a malignant lesion of melanocytic origin that

arises from a benign melanocytic lesion or de novo.

The mucosal melanoma tends to appear at higher stage and is more aggressive.

Melanoma usually affects parotid gland, usually as a

metastatic deposit from scalp, conjuctival or paranasal tumor.



Sixth or seventh decade of life.Males are commonly affected than females.

Four of every five oral melanomas are found on the hard palate

or maxillary alveolus.

A n oral lesion typically begins as a brown to black macule with irregular borders.

The macule extends laterally, and a lobulated, exophytic mass

develops once the vertical growth is initiated.

Ulceration may develop early, but many lesions are dark,

lobulated, exophytic masses without ulceration at the time of

diagnosis.

Pain is not a common feature except in ulcerated lesion



The tumor consists of radiolucency with ill-defined

margins.

There is lytic bone destruction.

The borders are irregular giving it a characteristic

´MOTH-E A TEN µ appearance.



A malgam tattoo

Oral melanoticmacule

Melanocytic hyperplasia Focal hemosiderin deposit

Local patch of melanoplakia



Melanomas consist of neoplastic

melanocytes often surrounded by clear

halo, both within the epithelium and

invading deeper tissues.

These cells are round to spindle shaped

and typically speckled or intensely

pigmented with melanin.

In the early stages of the neoplasm,

atypical melanocytes are seen scattered

singly among basal epithelial cells or

nests within basal cell layer.

In later stages malignant melanocytes

are seen invading the connective tissue.



Surgical excision is the only curative treatment.

For small early tumors, surgical margins of 1 cm are necessary to achieve control. However, for larger and deeply invasive tumors, wide surgical excision is

recommended. It may metastasize to regional lymph nodes, so surgical

excision of regional lymph nodes is recommended.

R adiation therapy has no significant impact; however

chemotherapy and immunotherapy can be used as an adjunct.

The prognosis is extremely poor. Young patients have a better survival than older ones.

Malignant Bone Neplasms

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