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SARCOMAS CLINICAL
AND
RADIOGRAPHIC FEATURES
INDIAN DENTAL ACADEMY
Leader in continuing Dental Education
www.indiandentalacademy.com
HISTORY
• cancer is found among fossilized bone tumors,
human mummies in ancient Egypt, and ancient
manuscripts.
• Growths suggestive of the bone cancer called
osteosarcoma have been seen in mummies.
• Oldest description of cancer dates back to about
3000 BC in egyptian Papyrus. writing says about the
• disease, “There is no treatment.”
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• Origin of the word cancer is credited to the Greek
physician Hippocrates (460-370 BC), “Father of Medicine.”
• He used the terms carcinos - non-ulcer forming
• carcinoma - ulcer-forming tumors.( refers to crab in greek)
• Roman physician, Celsus (28-50 BC), later translated the
Greek term into cancer, the Latin word for crab
• Galen (130-200 AD), Roman physician, used the word
oncos (Greek for swelling) to describe tumors
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• Hyperplasias may produce tissue masses
referred to as tumors
• Neoplasm or tumor is “ a mass of tissue
formed as a result of abnormal, excessive,
uncoordinated, autonomous, purposeless
proliferation of cells”.
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Benign
Malignant
Carcinoma
SarcomaHamaratoma
Choriostoma
Teratoma
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BENIGN NEOPLASMS
• Dysmorphic proliferations of tissues
• They have the capacity for continuous autonomous
growth.
• They donot elaborate the enzymes and growth
factors necessary for metastasis
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Hamaratoma
• Dysmorphic proliferation of tissue that is native to
the area
• It does not have the capacity for continuous growth
but merely parallels that of the host
• They cease grow at some point in their course and
they do not infiltrate into surrounding tissues.
• E.g: hemangioma , pigmented nevi, odontoma,
ameloblastic fibro odontoma.
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Choriostoma
• Dysmorphic proliferations of tissue that are not
native to the site. They have a limited proliferation.
• Heterotopic GI cyst, may be found in the tongue or
floor of the mouth contains GI glandular structures;
• Bone or cartilage in the tongue
• Development of thyroid tissue in the posterior
tongue
• Ectopic sebaceous glands known as Fordyce
granules
• salivary gland tissue within lymph nodeswww.indiandentalacademy.com
TERATOMA
• Neoplasias that arise from multiple germ layers
• Produce tissues that are foreign to the part in which
they develop.
• They are distinct from other neoplasias that may also
show tissue diversity
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• Carcinoma: malignant tumors of epithelial origin
• Sarcoma: (sarcos- fleshy)
Malignant tumors of mesenchymal origin.
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Grading of sarcomas
• FNCLCC( FEDERATION NATIONAL DE CENTRES
DE LUTTE CONTRE LE CANCER)
• Based on tumor differentiation
• Mitosis count
• Tumor necrosis
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TUMOR DIFFERENTION
• Score 1 : sa closely resembling normal adult
mesenchymal tisssue ( well differentiated)
• Score 2 : sa for which hp typing is certain
• Score 3 : embryonal & undifferentiated
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MITOSIS COUNT
• Score 1 : 0-9/10(HPF)
• Score 2 : 10-19
• Score 3 : >/= 20
TUMOR NECROSIS
• Score 0 : no necrosis
• Score 1 : < 50% tumor necrosis
• Score 2 : > 50% tumor necrosis
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HISTOLOGIC GRADE
• Grade 1 : total score 2/3
• Grade 2 : total score 4/5
• Grade 3 : total score 6/7/8
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American Joint Committee on Cancer (AJCC) and
International Union Against Cancer (UICC) staging
system for soft tissue sarcomas
• Primary tumor
• Tx - Primary tumor cannot be assessed
• T0 - No evidence of primary tumor
• T1 - Tumor less than 5 cm in greatest dimension
(T1a, superficial; T1b, deep)
• T2 - Tumor greater than 5 cm in greatest
dimension (T2a, superficial; T2b, deep)
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• Regional lymph nodes
– Nx - Lymph nodes cannot be assessed
– N0 - No lymph node invovemnt
– N1 - Lymph nodes involvement present
• Distant metastases
– Mx - Distant metastases cannot be assessed
– M0 - No distant metastases
– M1 - Distant metastases present
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Histopathologic grade
• Gx - Grade cannot be assessed
• G1 - Well-differentiated
• G2 - Moderately differentiated
• G3 - Poorly differentiated
• G4 – Undifferentiated
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• IA (G1-2, T1a-b, N0, M0) - Low-grade, small, and
superficial or deep tumor
• IB (G1-2, T2a, N0, M0) - Low-grade, large, and
superficial tumor
• IIA (G1-2, T2b, N0, M0) - Low-grade, large, and
deep tumor
• IIB (G3-4, T1a-b, N0, M0) - High-grade, small, and
superficial or deep tumor
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• IIC (G3-4, T2a, N0, M0) - High-grade, large, and
superficial tumor
• III (G3-4, T2b, N0, M0) - High-grade, large, and
deep tumor
• IV (any G, any T, N1, M0) - Any metastasis
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CLASSIFICATION OF SOFT TISSUE
SARCOMAS
Sharon W Weiss, John R. Goldblum
Enzinger & Weiss Soft Tissue Tumors
5th Edition Elsevier 2008
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• FIBROBLASTIC/ MYOFIBROBLASTIC TUMOR
Malignant Fibrous Histiocytoma
Storiform
Myxoid
Giant Cell
Inflammatory Type
• LIPOMATOUS TUMORS
LIPOSARCOMA : LIPOMA LIKE, SCLEROSING,
SPINDLE
MYXOID/ROUND CELL LIPOSARCOMA
PLEOMORPHIC LIPOSARCOMA
DEDIFFERENTIATED LIPOSARCOMA
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• SMOOTH MUSCLE TUMORS
Leiomyosarcoma
• Skeletal Muscle Tumors
Embryonal Rhabdomyosarcoma
Usual , Botryoid, Spindle Cell
Alveolar Rhabdomyosarcoma
Pleomorphic Rhabdomyosarcoma
Sclerosing Rhabdomyosarcoma
Rhabdomyosarcoma With Ganglionic Cells
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• TUMOR OF BLOOD & LYMPH VESSELS
ANGIOSARCOMA , KAPOSI SARCOMA
HEMANGIOPERICYTOMA
• PERIVASCULAR TUMORS
Malignant Glomus Tumor
• SYNOVIAL TUMORS
Malignant Tenosynovial Giant Cell Tumor
• MESOTHELIAL TUMORS
Diffuse Mesothelioma
EPITHELIAL, SARCOMATOID, BIPHASIC
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• PRIMITIVE NEUROECTODERMAL TUMORS
• Ewings Sarcoma
• NERVE SHEATH TUMORS
• Neurofibrosarcoma/ schwannoma
• EXTRASKELETAL OSSEOUS & CARTILAGINOUS
TUMORS
• Osteosarcoma
• Chondrosarcoma
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MISCELLANEOUS:
• SYNOVIAL SARCOMA
• ALVEOLAR SOFT PART SARCOMA
• EPITHELOID SARCOMA
• DESMOPLASTIC SMALL ROUND CELL TUMOR
• MALIGNANT EXTRA RENAL RHABDOID TUMOR
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FIBROSARCOMA
• Malignant tumor of fibroblasts
• Most common soft tissue sarcoma
• Most common in extremities
• Only 10% occurs in h & n. m > f
• May arise from pre-exisisting lesions such as fibrous
dysplasia, chronic osteomyelitis, bone infarcts,
pagets disease.
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• Occur anywhere in h & n, & at any age
• Most common in children, young adults
• Can also occur in bone as primary or secondary forms
• Primary occurs centrally within medullary canal,
peripheral arise from periosteum.
• Produce variable amount of collagen
• Secondary arise from pre-exsisting lesion or after
radiotherapy to bone or soft tissue
• More aggressive tumor with poorer prognosis
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• Painless fibrous, fleshy masses that are destructive
of bone
• Peripheral lesions may invade local soft tissues
causing bulky clinically obvious lesion
• If involve course of nerve…neural abnormalities
• Involvement of tmj causes trismus
• Cause mobility of teeth if they are located in alveolar
bone
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• Hp: well differntiated fibrosarcomas consists of
spindle cels that classically form a “herring bone
pattern”
• Poorly differentiated tumors, cells are less organised
& may appear rounder/ovoid
• Produce less collagen when compared to well
differentiated.
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PARALLEL SHEETS OF CELLS ARRANGED IN
INTERTWINING WHORLS
Herring bone pattern
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• Radiology:
• Mostly in mandible
• Premolar-molar area is common
• Poorly demarcated, non corticated
• If soft tissue lesions occur adjacent to bone
they may cause saucer like depression similar
to scc
• Sclerosis may occur in adjacent normal bone
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• Internal structure: little
• Entirely radiolucent
• If lesion is not aggressive either residual jaw bone/
reactive osseous bone may be present
• Surrounding strctures:
• Alveolar process, inferior border, nv canals are lost
• Floor of sinus, wall of maxilla can be destroyed
• Loss of supporting bone around tooth.
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Dd :
• Fibrous dysplasia
• Osteosarcoma
• Fibrous histiocytoma
• Neurofibromas ,
• Malignant peripheral nerve sheath tumors.
“Each one must be distinguished by its unique
histopathologic features.”
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Fibrosarcoma causing
displacement of tooth,
tongue
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MALIGNANT FIBROUS HISTIOCYTOMA
• Sarcoma with both fibroblastic & histiocytic features
• Most common soft tissue sarcoma of late adult life.
• Many were previously diagnosed as fibrosarcomas,
malignant peripheral nerve sheath tumors, and
pleomorphic rhabdomyosarcomas.
• Its overlap with fibrosarcomas is because both arise
from a similar precursor cell within a maturation
sequence beginning with the pluripotential mesenchymal
stem cell and ending with the mature fibrocyte
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• Men> women
• Occurs mostly in extremities
• Occur in soft tissue or bone
• Rare in maxillofacial region
• Common complaint is expanding mass that may/
maynot be painful
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• Hp :
• Most lesional cells are spindled fibroblasts like cells
• Arranged in storiform pattern
• Some areas contain histiocyte like cells
• Which have eosinophilic cytoplasm/ pale foamy
cytoplasm
• Sub classified into
• Myxoid, pleomorphic-storiform, angiomatoid(
aneurysmal), giant cell
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Spindle cells may be arranged randomly. But most commonly usually
present as streaming in interlacing fascicles from a central nidus and
intersecting with cells from adjacent aggregates….storiform/ criss
crossing patternwww.indiandentalacademy.com
Dd:
• Malignant fibrous histiocytomas must be distinguished
from the benign fibrous histiocytoma( rl expansion of
jaw)
• fibrosarcomas, malignant peripheral nerve sheath
tumors & rhabdomyosarcomas
• Clinical presentation of all these lesions is similar
• Distinction is mainly histopathologic, most true
fibrosarcomas and rhabdomyosarcomas occur at
younger ages.
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LIPOSARCOMA
• Liposarcomas are very rare tumors in the oral and
maxillofacial area( if occurs- neck)
• Thigh, retroperitoneum
• Present as a slow‐growing mass from a deep origin
• Liposarcomas originate from primitive mesenchymal
cells rather than from mature fat cells
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• liposarcomas are rare in subcutaneous areas;
they most commonly arise in intermuscular
fascial planes, which contain residual
mesenchymal stem cells.
• Liposarcomas do not develop from pre‐existing
benign lipomas. All are malignant from their
inception.
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RHABDOMYOSARCOMA
• Rhabdomyosarcomas are malignant tumors of
primitive mesenchymal cells that undergo partial
rhabdomyoblast differentiation
• Unlike other sts which occur in adults
• 44% occurs in omf regions
• the orbit is the most common location, followed by
the nasal cavity, mouth, sinuses, cheek, and neck.
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• Three basic histologic types: embryonal, alveolar,
and pleomorphic
• embryonal- best prognosis
• most common type found in omf
• Males > f (1.5:1).
• Tumors have a peak incidence at age 4 years and
another at age 17 years
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• The tumor presents as a rapidly growing, fleshy
mass, which readily invades and destroys bone.
• Rhabdomyosarcomas are not radiographically
distinctive but will show primarily the anticipated
soft tissue mass and bony destruction
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• Dd:
• A tumor with rapid growth and destructiveness in a child
or young adult should suggest a rhabdomyosarcoma.
• Other rapidly destructive lesions in this age group are
Ewing sarcoma, neuroblastoma & acute Langerhans cell
• histiocytosis, and less commonly, a malignant peripheral
T‐cell lymphoma. All of these are also known
to invade bone in a destructive manner.
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Leiomyosarcoma
• Leiomyosarcomas are a relatively uncommon type of sarcoma
• 7% of all soft tissue sarcomas.
• Most occur in the retroperitoneum and within the abdomen;
• others are associated with large blood vessels, such as the
inferior vena cava and pulmonary artery, and are often
referred to as leiomyosarcomas of vascular origin.
• Intraoral leiomyosarcomas are extremely rare because of the
paucity of smooth muscle in oral tissues
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ANGIOSARCOMA
• Angiosarcomas are malignant tumors that arise from either
vascular or lymphatic endothelium
• malignant hemangioendotheliomas/lymphangiosarcomas
• Rarest , accounting for 1% of h& n sts
• Can occur at any age, more common in elderly
• Males> females
• Skin of maxillofacial area, scalp
• Io lesions lips, palate, gingiva, tongue, centrally with in maxilla,
mandible.
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• 10% of angiosarcomas develop in chronic
lymphedematous tissues
• 10% are believed to be late effects of radiotherapy
for previous malignancies of other types.
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• Induration and ulceration of angiosarcomas lead to a
suspicion of malignancy.
• rarity makes them an unusual consideration on a
differential list.
• More common ulcerating malignant lesions that can
occur on facial and forehead skin as well as scalp
• primarily basal cell carcinoma, melanoma, skin squamous
cell carcinoma, and eccrine tumors of sweat gland origin.
• If the tumor is nodular and bulky, a so‐called turban
tumor/ cylindroma of the scalp, is another possibility.
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• Begin as a flat, ecchymotic‐looking area dark red or
bluish red with a firm, indurated edge.
• associated with facial edema and are presumably of
lymphatic endothelial origin.
• As the lesions mature, they become nodular and fleshy
and will ulcerate.
• Most are painless, but secondary infection of ulcerated
lesions may produce pain.
• They are not vascular lesions, per se, and therefore do
not pose a bleeding risk. Instead, they are of vascular
cellular origin
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• Hp: Angiosarcomas are infiltrating tumors that
usually form irregular vascular channels that often
intercommunicate to form a network.
• The endothelial cells lining the channels are plump
and hyperchromatic and may proliferate to form
papillary projections.
• Nuclear irregularities
• atypical mitoses are usually present
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Hp variants:
• Epithelial hemangioendothelioma
• Spindle cell hemangioendothelioma
• Kaposiform hemangioendothelioma
• Polymorphous hemangioendothelioma
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KAPOSI SARCOMA
• Angioreticuloendothelioma
• Multiple idiopathic hemorrhagic sarcoma of kaposi
• Multicentric proliferation of vascular & spindle
components.
• Etiology is unknown
• Co factor model : host factor- immunosuppression
• Infectious agent – human herpes virus 8/KSHV
• Environmental: overall geographic distribution
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• Classic:
• Occurs in late adult life. 70 to 90% occurs in males
• It forms violaceous macules, papules, and nodules often
symmetrically on the skin of the lower extremities.
• It is chronic and slowly progresses through an early patch
stage and into plaque and nodular stages
• The lesions increase slowly in size and number, spreading
proximally and emerging into plaques or vascular
nodules.
• Oral involvement is unusual. If occurs palatal
mucosa/gingiva.
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• Endemic/ lymphadenopathic/ african:
• Present as localised/ generalised enlargement of
lymphnodes
• Including cervical
• Mainly visceral involvement
• Less skin & mucous membrane involvement
• Salivary glands are affected
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• Transplantation associated:
• Seen in renal transplant patients
• Manifest 1 or 2yrs after transplantation
• Due to loss of cellular immunity
• Sarcomatous involvement of skin, internal organs
• Oral involvement is rare.
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Generalised involvement of alveolar mucosa in a
renal transplant patient
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• Aids related:
• 40% homosexual aids pts develop disease as early
sign
• Lesions occur in many cutaneous locations, along
lines of cleavage
• Tip of nose
• Oral lesions occur on any mucosal surface
• Commonly gingival & palatal mucosa
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• HP:
• PATCH STAGE: In the early patch stage, changes may
be extremely subtle and show only a proliferation of
both small and dilated vessels.
• A mild infiltrate of lymphocytes and plasma cells may
be seen at the periphery.
• PLAQUE: Vascular proliferation continues in the
plaque stage, and foci of spindle cells, typically
related to the vascular component, develop
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• NODULAR :
• nodular stage, the spindle cell component
dominates, encroaching on the previously obvious
vascular spaces
• picture resembles fibrosarcoma
• but Kaposi sarcoma will show the presence of slit‐like
spaces between the spindle cells,which contain
varying numbers of erythrocytes and some
hemosiderin
• hyaline globules are seen.www.indiandentalacademy.com
Branching blood
vessels
Spindle cells
KAPOSI SARCOMA
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• DD : oral & skin lesions resemble bruising
(ecchymosis) or a deeply located low‐grade
mucoepidermoid carcinoma.
• Papular and nodular lesions appear like
hemangiomas, lymphangiomas & hemangiomas.
• Even though it is uncommon, bacillary epithelioid
angiomatosis may appear ks
• On the alveolar ridge, they may resemble a pyogenic
granuloma, or a peripheral giant cell proliferation
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Hemangiopericytoma
• Oral hpc is typically rapidly enlarged
red/bluish mass which arises in all age groups.
• Soft, rubbery, painless, well demarcatedcfrom
surrounding mucosa
• Sessile/pedunculated
• Surface lobularity/ telengiectasis
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• Hp: well circumscribed, greyish white
• Less hemorrhagic than vascular tumors
• Numerous branching vascular channels
• Plump endothelial nuclei
• Tightly packed oval & spindle cells
• Branching vascular channels are in form of stag horn
pattern.
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STAG HORN PATTERN
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SYNOVIAL SARCOMA
• Un common form
• Represents 5 to 10% of all soft tissue
neoplasms
• Occurs near large joints/bursae
• Commonly involves extremities
• Rare in h&n
• Occurs in parapharyngeal/paravertebral areas
• Causing dyspnoea, dysphagia, hoarsness,
headache.
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• It so named because of its resemblance to
developing synovial tissue
• Arise from pleuripotent mesenchymal stem cells
near joint surface, tendon, tendon sheats, juxta
articular membranes, fascial aponeurosis
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• Radiograhic findings:
• Plain radiographs aid in diagnosis
• Produce spotty calcification ( snow
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WHO CLASSIFICATION OF
BONE SARCOMAS
Louis B Harrison, Roy B Sessions, Waun Ki Hong
Head & Neck Cancer- A Multidisciplinary
Approach
2nd Edition Lippincott Williams & Wilkins Usa
1998
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BONE FORMING TUMORS
OSTEOSARCOMA
• CONVENTIONAL CENTRAL OSTEOSARCOMA
Osteoblastic, chondroblastic, fibroblastic
• TELANGIECTATIC
• INTRAOSSEOUS WELL DIFF LOW GRADE
• ROUND CELL
• PAROSTEAL
• PERIOSTEAL
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CARTILAGE FORMING TUMORS
CHONDROSARCOMA
• JUXTAEPITHELIAL
• MESENCHYMAL
• DEDIFFERENTIATED
• CLEAR CELL
• MALIGNANT
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MARROW TUMORS
• EWINGS SARCOMA
• PNET OF BONE
VASCULAR TUMOR OF BONE
• ANGIOSARCOMA OF BONE
• MALIGNANT HEMANGIOPERICYTOMA
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Osteosarcoma
• Malignancy of mesenchymal cells that have ability to
produce osteoid or immature bone.
• Most common primary tumor to orginate in bone
after multiple myeloma
• arises from undifferentiated cells &
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Etiology
• UNKNOWN
• Risk factors are radiation exposure
• Genetic predisposition( 13q14) causing inactivation
of RB gene leding to dev of retinoblastoma, os
• Bone dysplasias fibrous dysplasia, pagets disease
increase risk
• Li-fraumeni syndrome ( germline t53 mutation)
predispose to os
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• Can occur in any bone
• Common in long bones
• Extragnathic os shows bimodal age distribution
• 10-20yrs( period of active growth) & >50yrs
• Site: distal femoral & proximal tibial metaphyses.
• Older pts axial skeleton & flat bones are involved
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Shoulder
Pelvic
Distal femur and
proximal tibia
Jaw
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• Os of jaws 6 to 8% of all os.
• Occurs mostly in 3rd & 4th decade of life
• Slight male predominance.
• Maxilla < mandible
• Mandible: posterior body, hori.ramus > ascending ramus
• Maxilla : inferior portion( alveolar ridge, sinus
floor,palate) > superior( zygoma, orbital rim)
• Os of jaws are better differentited , better prognosis, less
metastasis, than extragnathic os
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Clinical features:
• Pain, swelling of involved area – facial deformity
• Pt c/o sprain,arthritis or so called growing pain
• Loosening of teeth
• Paresthesia
• Nasal obstruction in max. tumors
• Epistaxis, hemorrhage
• h/o recent tooth extraction with a nodular/polypoid
some what reddish granuloma like growth growing from
tooth socket.
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• As tumor grows, eroding cortical plates expansion is
very firm because of dense fibrous tumor tissue
produced.
• Initially swelling is smoothly contoured, covered with
normal appearing mucosa
• When expansion becomes chronically traumatised
mucositis develops on surface
• Surface ulcerates and grayish white necrotic surface
results which can be removed with tongue blade.
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juxta cortical os
• Paraosteal: lobulated nodule
attached to cortex by a stalk
• No elevation of periosteum/its reaction
• Hp: mass contain spindle cell fibroblast like
proliferation
• Well dev trabeculae of bone
• They coalesce and form large solid mass of
bone
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• Periosteal :
• Sessile lesion
• Arise with in cortex
• Elevates overlying periosteum
• Provokes production peripheral periosteal bone
• Leading edge of tumor mass perforates periosteum
extends into soft tissue
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Histopathology
• Cells of tumor may producoe osteoid, chondriod, or
fibrous connective tissue
• Vary in shape uniform round/ spindle shaped to
highly pleomorphic
• Depending on relative amounts of osteoid,
cartilage/collagen os is div into osteoblastic( 50%),
chondroblastic, fibroblastic
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• Chondroblastic os are more common
• Composed entirely of malignant cartilage growing lobules
with only small foci of direct osteoid production by tumor
cells
• Other less common histologic variants are
• Malignant histiocytoma like
• Smallcell, epitheloid, telangiectatic, giant cell rich
• Low grade well differentiated os – minimal cellular atypia ,
abundant bone formation.
• May be misdiagnosed as fibrous dysplasia/ fibro osseous
lesion
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Telangiectatic
• Seen in adolescence & early adulthood
• rare variant of central highgrade
• Gross examination reveals a blood-filled cavity
• Hemorrhagic & necrotic areas are seen in tumor
• Microscopically dilated vascular channels lined with
multinucleated giant cells and an anaplastic
sarcomatous stroma with evident bone formation
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• Radiographically : large lytic defect,
• usually expansile and accompanied by an extensive
soft tissue component.
• Mri- fluid levels similar to those seen in an
aneurysmal bone cyst
• Occasionally, this lesion may be very difficult to
distinguish from an aneurysmal bone cyst.
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Radiology
Internal structure:
• Entirely rl/ osteolytic
• Mixed rl-ro
• Quite ro/ osteoblastic
• Osseous structure may be granular/sclerotic apparing bone
• In form of cotton balls, wisps, honey coomb internal structure
with adjacent destruction of pre-exisisting osseous
architecture.
• What ever may be presentation, normal trabecular pattern is
lost.
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Osteolytic:
• Unicentric, ill defined borders
• Moth eaten appearance
• Perforation & expansion of cortical margins into sub
periosteal bone
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Mixed
• Ragged, ill defined borders
• Ro due to excess bone formation intermingled with
resorption leading to rl areas
• In some sequestra appear , appear as well defined ro
• Rl destruction may be in form of strands…honey comb
appearance
• Sun burst appearance
• Cumulus cloud
• Codmans triangle are seen
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Periphery and shape
• ragged, Ill defined border
• When viewed against normal bone Lesion is usually rl
• No peripheral sclerosis / encapsulation
• If it involves periosteum:
• Typical sun ray spicules/hair on end trabeculae are
seen
• This occurs when periosteum is displaced, partially
destroyed & disorganised.
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Sun ray appearance
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Cortical bone expansion and
destruction….www.indiandentalacademy.com
Garringtons sign
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Effects on surrounding tissues
• Widening of pdl space occurs ( garrington sign)
• Band like widening along complete length of pdl
space unilaterally/bilaterally
• Also seen in other malignancies, osteoblastoma,
• Pts undergoing ortodontic treatment
• Unilateral bone resorption seen in periodontal
disease.
www.indiandentalacademy.com
• Antral/nasal wall cortices are lost in maxillary
lesions
• Mand lesions destroy cortex of neurovascular
canal, nv canal is symmetically widened &
enlarged
• Lamina dura of involved tooth is completely
destroyed.
www.indiandentalacademy.com
• Dd:
• If internal structure is minimal
fibrosarcoma/ osteoblastic metastatic ca
• Osseous structure visible
Chondrosarcoma (affects older age group, more often
involves maxilla)
• Spiculated periosteal reaction
Prostate/breast metastasis
• Benign tumors & conditions like ossifying fibroma, fo
lesioms mimic os radiographically.
www.indiandentalacademy.com
• Ossifying subperiosteal hematoma( a h/o recent
trauma to the bone)
• Peripheral fibroma with calcification( slow, benign
growth)
• Chronic osteomyelitis ( but infection is absent in os)
• An important clinical differential feature is
neurosensory loss.
• rare osteomyelitis or neural loss from a previous
biopsy or surgery, only malignancies can produce
objective paresthesias.
www.indiandentalacademy.com
• radiographs or CT scans at right angles to the cortex
should show extracortical bone and a destroyed cortex.
• Fibrous dysplasia and ossifying fibroma will not have
extracortical bone. The
• extracortical bone seen in osteomyelitis with proliferative
periostitis will be associated with an intact
• cortex.
• Even when other osteomyelitides produce extracortical
bone, it is parallel to the cortex rather
• than at right angles as is seen in osteosarcoma
www.indiandentalacademy.com
Chondrosarcoma
• Malignant tumor characterised by formation of cartilage
by tumor cells
• Comprise 10% of all tumors of skeleton
• Rare in jaws( 3% of all cs)
• Extragnathic it is primarily neoplasm of adulthood with
peak prevalance in 6th, 7th decades of life
• Tumors arising in younger age group is uncommon
• No sex or race predilection
• Site: ileum, femur, humerus
www.indiandentalacademy.com
• In h& n : cs common in maxilla ( anterior areas where
cartilage tissue is present.
• mandible: ccoronoid process, condylar h& n, symphyseal
region.less common in body, ramus, nasal septum, pns
• Chondrosarcomas most often develop in osseous
locations
• But 1/3rd originate in laryngeal cartilage/soft tissue
• Cs arise directly from cartilage or may occur within
benign cartilagenous tumors….secondary cs
www.indiandentalacademy.com
• A painless mass, swelling
• Associated with separation or loosening of teeth
• Pain is unusual
• Maxillary tumors may cause nasal obstruction,
congestion, epistaxis, photophobia, or visual loss.
• If cs occur near tmj trismus, abnormal joint function
occurs.
• When metastasis occurs lung is organ most
commonly involved.
www.indiandentalacademy.com
• Hp: composed of cartilage with varying degrees of
maturation & cellularity
• Lobular pattern with lobules separated by thin fibrous
connective tissue
• Central area demonstrates lobules with greatest degree
of maturity
• Peripheral area contains immature cartilage &
mesenchymal tissue consisting of round/spindle cells
• Calcification/ossification may occur in chondroid matrix
www.indiandentalacademy.com
• Some times at periphery of lobules of high grade cs,
a few fs like spindling tumor cells are present
• Osseous trabeculae when present are seen at
periphery of lobules & appears to be rimmed by
osteoblasts.
• When malignant cells produce osteoid lacework or
trabeculae even in small foci, tumor is graded as os
www.indiandentalacademy.com
• Four histological subtypes:
• Clear cell
• Dedifferentiated
• Myxoid
• Mesenchymal
• Occur centrally within bone or less commonly
in soft tissue.
www.indiandentalacademy.com
Variants :
• Clear cell cs:
cells with abundant clear cytoplasm
• Dedifferentiated cs:
high grade malignancy that show admixture of well
differentiated cs & malignnat mesenchymal tumor
resembling fibrosarcoma.
• Myxoid cs: soft tissue tumor. io variant is seen,
characterised by clear, vacuolated, eosinophilic
cytoplasm with a background of mucoid material.www.indiandentalacademy.com
• Mesenchymal cs: uncommon tumor of soft tissue &
bone
• Shows biphasic hp
• More common in soft tissue than bone
• Chondroid tissue is well differentiated, degree of
cellularity & atypia varies from benign chondroma to
well differentiated chindrosarcoma
• If cartilagenous foci is sparse, tumor may be
misdiagnosed as hemangiopericytoma
www.indiandentalacademy.com
• Radiographically….internal structure:
• radiolucent area with poorly defined borders
• Rl area contains scattered ro foci caused by
ossification of cartilage matrix
• Mixed rl- ro appearance
• Some times it appears as moth eaten bone
alternating with islands of residual bone unaffected
by tumor
www.indiandentalacademy.com
• Central ro structure appear as flocculent implying
snow like features
• Diffuse calcification may be superimposed on a bony
background that resembles granular/ground glass
appearing normal bone
• Examination of this flocculence may reveal central rl
nidus which is probably cartilage surrounded by
calcification.
www.indiandentalacademy.com
• Periphery :
• Generally roun/ovoid/lobulated
• Well defined corticated borders
• Occasionally peripheral periosteal reaction in form of
sunray, hair on end appearance is seen
• Uncommonly aggressive lesions ill defined, invasive ,
non corticated borders seen
www.indiandentalacademy.com
Effect on surrounding tissues:
• Relatively slow growing so expands cortex, rather
than rapidly destroying them
• Mand cases, ian expanded still maintaining corical
covering
• Maxillary lesions push wall of sinus or nasal fossa &
impinge on infratemporal fossa
• Lesions of condyle cause expansion, remodelling of
articular fossa & eminence.
www.indiandentalacademy.com
• If lesion is in articular disk region, widening of joint
space, remodelling of condylar neck occurs.
• Erosion of articular fossa occurs
• If lesion occurs near tooth, root resorption, widening
of pdl space, tooth displacement may occur.
www.indiandentalacademy.com
• Dd;
• Os : typical calcifications of cs are absent
• Fibrous dysplasia: periphery is well defined. ro
portion of fibrous dysplasia is abnormal bone & not
the calcifications
• Because of their slow growth and especially their
intact overlying mucosa, most cases will initially
resemble a benign odontogenic tumor or a benign
tumor of bone
www.indiandentalacademy.com
• Some punctate radiopacities are identifiable, the lesion
will resemble a
• calcifying epithelial odontogenic tumor
• an ossifying fibroma
• an immature osteoblastoma
• a cavernous hemangioma of bone.
• The more obviously aggressive presentations with
irregular radiolucencies and perhaps neurosensory loss
would be consistent with an intraosseous carcinoma, an
osteosarcoma, and a malignant fibrous histiocytoma
www.indiandentalacademy.com
Ewings sarcoma
• Notoriously aggressive and destructive malignancy
of bone arising from marrow mesenchymal stem cells
• Genetically and histologically distinctive small round
cell sarcoma of bone
• 85% to 90% cases, tumor cells show reciprocal
translocation b/n chromosomes 11 & 22. (q24;q12)
• Third most common osseous neoplasm than os, cs
• It was first described by James Ewing in 1920 as a
"diffuse endothelioma of bone.“www.indiandentalacademy.com
• In both the jaws and long bones,peak age of
occurrence is in the teenage years (50%). Young men
slightly > young women
• mandible's posterior body, the angle and ramus
regions are common. Cause bony expansion , mobile
teeth, extensive destruction of bone and
necrosis…fever occurs
• present a picture similar to that of an osteomyelitis.
• rapid growth rate. Metastasis is common.www.indiandentalacademy.com
• Hp : Ewing sarcomas are composed of densely packed,
rather uniform cells with little intercellular stroma
• The nuclei are rounded to oval with defined nuclear
borders and a
• finely granular chromatin pattern
• The cytoplasm is indistinct and may be vacuolated.
• The cells are two to three times the size of a lymphocyte.
• Mitoses are infrequent.
• Rapid growth, undergo considerable necrosis, sometimes
resulting in a perivascular pattern of viable tumor cells
www.indiandentalacademy.com
Tumor cells are arranged in broad sheets…filigree pattern in
which infiltratin strands of tumor cells are separated by thin
fibrovascular septae
Well defined nuclei
Indistinct
cytoplasm
www.indiandentalacademy.com
• Hp dd: includes other small round cell tumors,
including
• neuroblastoma,lymphoma, small cell
osteosarcoma& embryonal rhabdomyosarcoma.
• Ewing sarcoma will usually have intracytoplasmic
glycogen granules demonstrated by periodic
acid‐Schiff (PAS) and diastase staining.
• neuroblastoma and embryonal rhabdomyosarcomas
may also yield positive staining.( for glycogen)
www.indiandentalacademy.com
Rf:
• Internal structure:
• Es is a destructive process with little unduction of bone.
• It commences on internal aspect of bone & involves
endosteal & periosteal surfaces later , usually entirely rl.
• Periphery: rl poorly demarcated & never corticated.
• Advancing end destroys bone in an uneven fashion.
• May cause pathological fractures.
• Extends into adjacent soft tissue.
www.indiandentalacademy.com
Effect on surrounding tissues:
• adjacent normal structures ian canal, lower border
of mandible, alveolar cortical plates are destroyed.
• It doesnot characteristically cause root resorption, it
destroys supporting bone adjacent to tooth.
www.indiandentalacademy.com
• Panoramic radiographs and a CT scan
• an ill‐defined, irregular resorption of bone with focal
areas of residual bone resembling sequestra.
• Pathologic fractures are common, attesting to the degree
of bone destruction.
• Multilayered periosteal reaction that has been described
as an "onion skin" appearance,
• (Similar to proliferative periostitis in osteomyelitis)
• such a radiographic appearance is almost never seen
when Ewing sarcoma arises in the jaws.
www.indiandentalacademy.com
• Ewing sarcoma in the jaws will produce a destructive
radiolucency with resorbed tooth roots and
displaced teeth.
• On rare occasions, a Ewing sarcoma may produce a
periosteal new bone formation perpendicular to the
cortex and thereby create the "sun‐ray" appearance
more frequently seen in osteosarcomas
www.indiandentalacademy.com
• Dd :
• Pain , fever, leukocytosis suggest a suppurative
osteomyelitis.
• Reinforced by radiographs showing destructive
bone pattern , bone foci resembling a sequestrum,
layered periosteal "onion skin“
appearance…resembling proliferative periostitis.
www.indiandentalacademy.com
• Other aggressive malignancies that occur in this
young age group include
• Rhabdomyosarcoma ,
• Osteosarcoma, fibrosarcoma, and neuroblastoma
• Eosinophilic granuloma of jaw is also a destructive
process, but is associated with laminar periosteal
reaction, where as in jaws ewings sarcoma doesnot
produce.
www.indiandentalacademy.com
Periosteal reactions
www.indiandentalacademy.com
• periosteum is a membrane several cell layers thick that
covers almost all of every bone.
• only parts not covered by this membrane are the parts
covered by cartilage.
• Besides covering the bone and sharing some of its blood
supply with the bone,
• it also produces bone when it is stimulated
appropriately.
• Practically anything that breaks, tears, stretches,
inflames, or even touches the periosteum.
www.indiandentalacademy.com
•With slow-growing processes, the
periosteum has plenty of time to
respond to the process.
•It can produce new bone just as fast
as the lesion is growing.
• solid, uninterrupted periosteal new
bone along the margin of the affected
bone.
www.indiandentalacademy.com
•In case of rapid growing tumors,
new bone is not formed at the rate
of tumor.
•An interrupted pattern is formed
•This may result in a pattern of one
or more concentric shells of new
bone over the lesion. This pattern is
sometimes called lamellated or
"onion-skin" periosteal reaction.
www.indiandentalacademy.com
•If the lesion grows rapidly but steadily, the
periosteum will not have enough time to
lay down even a thin shell of bone,
•In such cases, the tiny fibers that connect
the periosteum to the bone (Sharpey's
fibers) become stretched out
perpendicular to the bone.
•When these fibers ossify, they produce a
pattern sometimes called "sunburst" or
"hair-on-end" periosteal reaction,
depending of how much of the bone is
involved by the proceswww.indiandentalacademy.com
• Codmans triangle :
• When a process is growing too fast, it
penetrate through the cortex causing
separation of the periosteum and formation of
lamellated new bone. If the periosteum
elevates to a significant degree, it can break
forming an acute angle
www.indiandentalacademy.com
• When this little bit of ossification is seen tangentially
on a radiograph, it forms a small angle with the
surface of the bone, but not a complete triangle..
www.indiandentalacademy.com
• References
1. Robert E Marx, Diane Stern Oral And Maxillofacial
Pathology: A Rationale For diagnosis & Treatment.
1st edition, Quintessence.2003.
2. Stuart C White , Michael J Pharoah , Textbook Of
Oral Radiology Principles And Interpretation
3. Norman k wood paul w goaz. Dd of oral and
maxilllofacial lesions. 5th edition elsevier 2007
www.indiandentalacademy.com
• R Rajendran B Sivapathasundharam Shafer’s
Textbook Of Oral Pathology. 5th Edition, Elsevier,
2008
• Brad W Neville Douglas D Damm Carl M Allen Jerry
E Bouquot. Oral And Maxillofacial Pathology. 3rd
Edition Elsevier, 2009
www.indiandentalacademy.com
www.indiandentalacademy.com

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  • 1. SARCOMAS CLINICAL AND RADIOGRAPHIC FEATURES INDIAN DENTAL ACADEMY Leader in continuing Dental Education www.indiandentalacademy.com
  • 2. HISTORY • cancer is found among fossilized bone tumors, human mummies in ancient Egypt, and ancient manuscripts. • Growths suggestive of the bone cancer called osteosarcoma have been seen in mummies. • Oldest description of cancer dates back to about 3000 BC in egyptian Papyrus. writing says about the • disease, “There is no treatment.” www.indiandentalacademy.com
  • 3. • Origin of the word cancer is credited to the Greek physician Hippocrates (460-370 BC), “Father of Medicine.” • He used the terms carcinos - non-ulcer forming • carcinoma - ulcer-forming tumors.( refers to crab in greek) • Roman physician, Celsus (28-50 BC), later translated the Greek term into cancer, the Latin word for crab • Galen (130-200 AD), Roman physician, used the word oncos (Greek for swelling) to describe tumors www.indiandentalacademy.com
  • 4. • Hyperplasias may produce tissue masses referred to as tumors • Neoplasm or tumor is “ a mass of tissue formed as a result of abnormal, excessive, uncoordinated, autonomous, purposeless proliferation of cells”. www.indiandentalacademy.com
  • 6. BENIGN NEOPLASMS • Dysmorphic proliferations of tissues • They have the capacity for continuous autonomous growth. • They donot elaborate the enzymes and growth factors necessary for metastasis www.indiandentalacademy.com
  • 7. Hamaratoma • Dysmorphic proliferation of tissue that is native to the area • It does not have the capacity for continuous growth but merely parallels that of the host • They cease grow at some point in their course and they do not infiltrate into surrounding tissues. • E.g: hemangioma , pigmented nevi, odontoma, ameloblastic fibro odontoma. www.indiandentalacademy.com
  • 8. Choriostoma • Dysmorphic proliferations of tissue that are not native to the site. They have a limited proliferation. • Heterotopic GI cyst, may be found in the tongue or floor of the mouth contains GI glandular structures; • Bone or cartilage in the tongue • Development of thyroid tissue in the posterior tongue • Ectopic sebaceous glands known as Fordyce granules • salivary gland tissue within lymph nodeswww.indiandentalacademy.com
  • 9. TERATOMA • Neoplasias that arise from multiple germ layers • Produce tissues that are foreign to the part in which they develop. • They are distinct from other neoplasias that may also show tissue diversity www.indiandentalacademy.com
  • 10. • Carcinoma: malignant tumors of epithelial origin • Sarcoma: (sarcos- fleshy) Malignant tumors of mesenchymal origin. www.indiandentalacademy.com
  • 11. Grading of sarcomas • FNCLCC( FEDERATION NATIONAL DE CENTRES DE LUTTE CONTRE LE CANCER) • Based on tumor differentiation • Mitosis count • Tumor necrosis www.indiandentalacademy.com
  • 12. TUMOR DIFFERENTION • Score 1 : sa closely resembling normal adult mesenchymal tisssue ( well differentiated) • Score 2 : sa for which hp typing is certain • Score 3 : embryonal & undifferentiated www.indiandentalacademy.com
  • 13. MITOSIS COUNT • Score 1 : 0-9/10(HPF) • Score 2 : 10-19 • Score 3 : >/= 20 TUMOR NECROSIS • Score 0 : no necrosis • Score 1 : < 50% tumor necrosis • Score 2 : > 50% tumor necrosis www.indiandentalacademy.com
  • 14. HISTOLOGIC GRADE • Grade 1 : total score 2/3 • Grade 2 : total score 4/5 • Grade 3 : total score 6/7/8 www.indiandentalacademy.com
  • 15. American Joint Committee on Cancer (AJCC) and International Union Against Cancer (UICC) staging system for soft tissue sarcomas • Primary tumor • Tx - Primary tumor cannot be assessed • T0 - No evidence of primary tumor • T1 - Tumor less than 5 cm in greatest dimension (T1a, superficial; T1b, deep) • T2 - Tumor greater than 5 cm in greatest dimension (T2a, superficial; T2b, deep) www.indiandentalacademy.com
  • 16. • Regional lymph nodes – Nx - Lymph nodes cannot be assessed – N0 - No lymph node invovemnt – N1 - Lymph nodes involvement present • Distant metastases – Mx - Distant metastases cannot be assessed – M0 - No distant metastases – M1 - Distant metastases present www.indiandentalacademy.com
  • 17. Histopathologic grade • Gx - Grade cannot be assessed • G1 - Well-differentiated • G2 - Moderately differentiated • G3 - Poorly differentiated • G4 – Undifferentiated www.indiandentalacademy.com
  • 18. • IA (G1-2, T1a-b, N0, M0) - Low-grade, small, and superficial or deep tumor • IB (G1-2, T2a, N0, M0) - Low-grade, large, and superficial tumor • IIA (G1-2, T2b, N0, M0) - Low-grade, large, and deep tumor • IIB (G3-4, T1a-b, N0, M0) - High-grade, small, and superficial or deep tumor www.indiandentalacademy.com
  • 19. • IIC (G3-4, T2a, N0, M0) - High-grade, large, and superficial tumor • III (G3-4, T2b, N0, M0) - High-grade, large, and deep tumor • IV (any G, any T, N1, M0) - Any metastasis www.indiandentalacademy.com
  • 21. CLASSIFICATION OF SOFT TISSUE SARCOMAS Sharon W Weiss, John R. Goldblum Enzinger & Weiss Soft Tissue Tumors 5th Edition Elsevier 2008 www.indiandentalacademy.com
  • 22. • FIBROBLASTIC/ MYOFIBROBLASTIC TUMOR Malignant Fibrous Histiocytoma Storiform Myxoid Giant Cell Inflammatory Type • LIPOMATOUS TUMORS LIPOSARCOMA : LIPOMA LIKE, SCLEROSING, SPINDLE MYXOID/ROUND CELL LIPOSARCOMA PLEOMORPHIC LIPOSARCOMA DEDIFFERENTIATED LIPOSARCOMA www.indiandentalacademy.com
  • 23. • SMOOTH MUSCLE TUMORS Leiomyosarcoma • Skeletal Muscle Tumors Embryonal Rhabdomyosarcoma Usual , Botryoid, Spindle Cell Alveolar Rhabdomyosarcoma Pleomorphic Rhabdomyosarcoma Sclerosing Rhabdomyosarcoma Rhabdomyosarcoma With Ganglionic Cells www.indiandentalacademy.com
  • 24. • TUMOR OF BLOOD & LYMPH VESSELS ANGIOSARCOMA , KAPOSI SARCOMA HEMANGIOPERICYTOMA • PERIVASCULAR TUMORS Malignant Glomus Tumor • SYNOVIAL TUMORS Malignant Tenosynovial Giant Cell Tumor • MESOTHELIAL TUMORS Diffuse Mesothelioma EPITHELIAL, SARCOMATOID, BIPHASIC www.indiandentalacademy.com
  • 25. • PRIMITIVE NEUROECTODERMAL TUMORS • Ewings Sarcoma • NERVE SHEATH TUMORS • Neurofibrosarcoma/ schwannoma • EXTRASKELETAL OSSEOUS & CARTILAGINOUS TUMORS • Osteosarcoma • Chondrosarcoma www.indiandentalacademy.com
  • 26. MISCELLANEOUS: • SYNOVIAL SARCOMA • ALVEOLAR SOFT PART SARCOMA • EPITHELOID SARCOMA • DESMOPLASTIC SMALL ROUND CELL TUMOR • MALIGNANT EXTRA RENAL RHABDOID TUMOR www.indiandentalacademy.com
  • 27. FIBROSARCOMA • Malignant tumor of fibroblasts • Most common soft tissue sarcoma • Most common in extremities • Only 10% occurs in h & n. m > f • May arise from pre-exisisting lesions such as fibrous dysplasia, chronic osteomyelitis, bone infarcts, pagets disease. www.indiandentalacademy.com
  • 28. • Occur anywhere in h & n, & at any age • Most common in children, young adults • Can also occur in bone as primary or secondary forms • Primary occurs centrally within medullary canal, peripheral arise from periosteum. • Produce variable amount of collagen • Secondary arise from pre-exsisting lesion or after radiotherapy to bone or soft tissue • More aggressive tumor with poorer prognosis www.indiandentalacademy.com
  • 29. • Painless fibrous, fleshy masses that are destructive of bone • Peripheral lesions may invade local soft tissues causing bulky clinically obvious lesion • If involve course of nerve…neural abnormalities • Involvement of tmj causes trismus • Cause mobility of teeth if they are located in alveolar bone www.indiandentalacademy.com
  • 30. • Hp: well differntiated fibrosarcomas consists of spindle cels that classically form a “herring bone pattern” • Poorly differentiated tumors, cells are less organised & may appear rounder/ovoid • Produce less collagen when compared to well differentiated. www.indiandentalacademy.com
  • 31. PARALLEL SHEETS OF CELLS ARRANGED IN INTERTWINING WHORLS Herring bone pattern www.indiandentalacademy.com
  • 32. • Radiology: • Mostly in mandible • Premolar-molar area is common • Poorly demarcated, non corticated • If soft tissue lesions occur adjacent to bone they may cause saucer like depression similar to scc • Sclerosis may occur in adjacent normal bone www.indiandentalacademy.com
  • 33. • Internal structure: little • Entirely radiolucent • If lesion is not aggressive either residual jaw bone/ reactive osseous bone may be present • Surrounding strctures: • Alveolar process, inferior border, nv canals are lost • Floor of sinus, wall of maxilla can be destroyed • Loss of supporting bone around tooth. www.indiandentalacademy.com
  • 34. Dd : • Fibrous dysplasia • Osteosarcoma • Fibrous histiocytoma • Neurofibromas , • Malignant peripheral nerve sheath tumors. “Each one must be distinguished by its unique histopathologic features.” www.indiandentalacademy.com
  • 35. Fibrosarcoma causing displacement of tooth, tongue www.indiandentalacademy.com
  • 36. MALIGNANT FIBROUS HISTIOCYTOMA • Sarcoma with both fibroblastic & histiocytic features • Most common soft tissue sarcoma of late adult life. • Many were previously diagnosed as fibrosarcomas, malignant peripheral nerve sheath tumors, and pleomorphic rhabdomyosarcomas. • Its overlap with fibrosarcomas is because both arise from a similar precursor cell within a maturation sequence beginning with the pluripotential mesenchymal stem cell and ending with the mature fibrocyte www.indiandentalacademy.com
  • 37. • Men> women • Occurs mostly in extremities • Occur in soft tissue or bone • Rare in maxillofacial region • Common complaint is expanding mass that may/ maynot be painful www.indiandentalacademy.com
  • 38. • Hp : • Most lesional cells are spindled fibroblasts like cells • Arranged in storiform pattern • Some areas contain histiocyte like cells • Which have eosinophilic cytoplasm/ pale foamy cytoplasm • Sub classified into • Myxoid, pleomorphic-storiform, angiomatoid( aneurysmal), giant cell www.indiandentalacademy.com
  • 39. Spindle cells may be arranged randomly. But most commonly usually present as streaming in interlacing fascicles from a central nidus and intersecting with cells from adjacent aggregates….storiform/ criss crossing patternwww.indiandentalacademy.com
  • 40. Dd: • Malignant fibrous histiocytomas must be distinguished from the benign fibrous histiocytoma( rl expansion of jaw) • fibrosarcomas, malignant peripheral nerve sheath tumors & rhabdomyosarcomas • Clinical presentation of all these lesions is similar • Distinction is mainly histopathologic, most true fibrosarcomas and rhabdomyosarcomas occur at younger ages. www.indiandentalacademy.com
  • 41. LIPOSARCOMA • Liposarcomas are very rare tumors in the oral and maxillofacial area( if occurs- neck) • Thigh, retroperitoneum • Present as a slow‐growing mass from a deep origin • Liposarcomas originate from primitive mesenchymal cells rather than from mature fat cells www.indiandentalacademy.com
  • 42. • liposarcomas are rare in subcutaneous areas; they most commonly arise in intermuscular fascial planes, which contain residual mesenchymal stem cells. • Liposarcomas do not develop from pre‐existing benign lipomas. All are malignant from their inception. www.indiandentalacademy.com
  • 43. RHABDOMYOSARCOMA • Rhabdomyosarcomas are malignant tumors of primitive mesenchymal cells that undergo partial rhabdomyoblast differentiation • Unlike other sts which occur in adults • 44% occurs in omf regions • the orbit is the most common location, followed by the nasal cavity, mouth, sinuses, cheek, and neck. www.indiandentalacademy.com
  • 44. • Three basic histologic types: embryonal, alveolar, and pleomorphic • embryonal- best prognosis • most common type found in omf • Males > f (1.5:1). • Tumors have a peak incidence at age 4 years and another at age 17 years www.indiandentalacademy.com
  • 45. • The tumor presents as a rapidly growing, fleshy mass, which readily invades and destroys bone. • Rhabdomyosarcomas are not radiographically distinctive but will show primarily the anticipated soft tissue mass and bony destruction www.indiandentalacademy.com
  • 46. • Dd: • A tumor with rapid growth and destructiveness in a child or young adult should suggest a rhabdomyosarcoma. • Other rapidly destructive lesions in this age group are Ewing sarcoma, neuroblastoma & acute Langerhans cell • histiocytosis, and less commonly, a malignant peripheral T‐cell lymphoma. All of these are also known to invade bone in a destructive manner. www.indiandentalacademy.com
  • 47. Leiomyosarcoma • Leiomyosarcomas are a relatively uncommon type of sarcoma • 7% of all soft tissue sarcomas. • Most occur in the retroperitoneum and within the abdomen; • others are associated with large blood vessels, such as the inferior vena cava and pulmonary artery, and are often referred to as leiomyosarcomas of vascular origin. • Intraoral leiomyosarcomas are extremely rare because of the paucity of smooth muscle in oral tissues www.indiandentalacademy.com
  • 48. ANGIOSARCOMA • Angiosarcomas are malignant tumors that arise from either vascular or lymphatic endothelium • malignant hemangioendotheliomas/lymphangiosarcomas • Rarest , accounting for 1% of h& n sts • Can occur at any age, more common in elderly • Males> females • Skin of maxillofacial area, scalp • Io lesions lips, palate, gingiva, tongue, centrally with in maxilla, mandible. www.indiandentalacademy.com
  • 49. • 10% of angiosarcomas develop in chronic lymphedematous tissues • 10% are believed to be late effects of radiotherapy for previous malignancies of other types. www.indiandentalacademy.com
  • 50. • Induration and ulceration of angiosarcomas lead to a suspicion of malignancy. • rarity makes them an unusual consideration on a differential list. • More common ulcerating malignant lesions that can occur on facial and forehead skin as well as scalp • primarily basal cell carcinoma, melanoma, skin squamous cell carcinoma, and eccrine tumors of sweat gland origin. • If the tumor is nodular and bulky, a so‐called turban tumor/ cylindroma of the scalp, is another possibility. www.indiandentalacademy.com
  • 51. • Begin as a flat, ecchymotic‐looking area dark red or bluish red with a firm, indurated edge. • associated with facial edema and are presumably of lymphatic endothelial origin. • As the lesions mature, they become nodular and fleshy and will ulcerate. • Most are painless, but secondary infection of ulcerated lesions may produce pain. • They are not vascular lesions, per se, and therefore do not pose a bleeding risk. Instead, they are of vascular cellular origin www.indiandentalacademy.com
  • 52. • Hp: Angiosarcomas are infiltrating tumors that usually form irregular vascular channels that often intercommunicate to form a network. • The endothelial cells lining the channels are plump and hyperchromatic and may proliferate to form papillary projections. • Nuclear irregularities • atypical mitoses are usually present www.indiandentalacademy.com
  • 53. Hp variants: • Epithelial hemangioendothelioma • Spindle cell hemangioendothelioma • Kaposiform hemangioendothelioma • Polymorphous hemangioendothelioma www.indiandentalacademy.com
  • 54. KAPOSI SARCOMA • Angioreticuloendothelioma • Multiple idiopathic hemorrhagic sarcoma of kaposi • Multicentric proliferation of vascular & spindle components. • Etiology is unknown • Co factor model : host factor- immunosuppression • Infectious agent – human herpes virus 8/KSHV • Environmental: overall geographic distribution www.indiandentalacademy.com
  • 55. • Classic: • Occurs in late adult life. 70 to 90% occurs in males • It forms violaceous macules, papules, and nodules often symmetrically on the skin of the lower extremities. • It is chronic and slowly progresses through an early patch stage and into plaque and nodular stages • The lesions increase slowly in size and number, spreading proximally and emerging into plaques or vascular nodules. • Oral involvement is unusual. If occurs palatal mucosa/gingiva. www.indiandentalacademy.com
  • 56. • Endemic/ lymphadenopathic/ african: • Present as localised/ generalised enlargement of lymphnodes • Including cervical • Mainly visceral involvement • Less skin & mucous membrane involvement • Salivary glands are affected www.indiandentalacademy.com
  • 57. • Transplantation associated: • Seen in renal transplant patients • Manifest 1 or 2yrs after transplantation • Due to loss of cellular immunity • Sarcomatous involvement of skin, internal organs • Oral involvement is rare. www.indiandentalacademy.com
  • 58. Generalised involvement of alveolar mucosa in a renal transplant patient www.indiandentalacademy.com
  • 59. • Aids related: • 40% homosexual aids pts develop disease as early sign • Lesions occur in many cutaneous locations, along lines of cleavage • Tip of nose • Oral lesions occur on any mucosal surface • Commonly gingival & palatal mucosa www.indiandentalacademy.com
  • 60. • HP: • PATCH STAGE: In the early patch stage, changes may be extremely subtle and show only a proliferation of both small and dilated vessels. • A mild infiltrate of lymphocytes and plasma cells may be seen at the periphery. • PLAQUE: Vascular proliferation continues in the plaque stage, and foci of spindle cells, typically related to the vascular component, develop www.indiandentalacademy.com
  • 61. • NODULAR : • nodular stage, the spindle cell component dominates, encroaching on the previously obvious vascular spaces • picture resembles fibrosarcoma • but Kaposi sarcoma will show the presence of slit‐like spaces between the spindle cells,which contain varying numbers of erythrocytes and some hemosiderin • hyaline globules are seen.www.indiandentalacademy.com
  • 62. Branching blood vessels Spindle cells KAPOSI SARCOMA www.indiandentalacademy.com
  • 63. • DD : oral & skin lesions resemble bruising (ecchymosis) or a deeply located low‐grade mucoepidermoid carcinoma. • Papular and nodular lesions appear like hemangiomas, lymphangiomas & hemangiomas. • Even though it is uncommon, bacillary epithelioid angiomatosis may appear ks • On the alveolar ridge, they may resemble a pyogenic granuloma, or a peripheral giant cell proliferation www.indiandentalacademy.com
  • 64. Hemangiopericytoma • Oral hpc is typically rapidly enlarged red/bluish mass which arises in all age groups. • Soft, rubbery, painless, well demarcatedcfrom surrounding mucosa • Sessile/pedunculated • Surface lobularity/ telengiectasis www.indiandentalacademy.com
  • 65. • Hp: well circumscribed, greyish white • Less hemorrhagic than vascular tumors • Numerous branching vascular channels • Plump endothelial nuclei • Tightly packed oval & spindle cells • Branching vascular channels are in form of stag horn pattern. www.indiandentalacademy.com
  • 67. SYNOVIAL SARCOMA • Un common form • Represents 5 to 10% of all soft tissue neoplasms • Occurs near large joints/bursae • Commonly involves extremities • Rare in h&n • Occurs in parapharyngeal/paravertebral areas • Causing dyspnoea, dysphagia, hoarsness, headache. www.indiandentalacademy.com
  • 68. • It so named because of its resemblance to developing synovial tissue • Arise from pleuripotent mesenchymal stem cells near joint surface, tendon, tendon sheats, juxta articular membranes, fascial aponeurosis www.indiandentalacademy.com
  • 69. • Radiograhic findings: • Plain radiographs aid in diagnosis • Produce spotty calcification ( snow www.indiandentalacademy.com
  • 70. WHO CLASSIFICATION OF BONE SARCOMAS Louis B Harrison, Roy B Sessions, Waun Ki Hong Head & Neck Cancer- A Multidisciplinary Approach 2nd Edition Lippincott Williams & Wilkins Usa 1998 www.indiandentalacademy.com
  • 71. BONE FORMING TUMORS OSTEOSARCOMA • CONVENTIONAL CENTRAL OSTEOSARCOMA Osteoblastic, chondroblastic, fibroblastic • TELANGIECTATIC • INTRAOSSEOUS WELL DIFF LOW GRADE • ROUND CELL • PAROSTEAL • PERIOSTEAL www.indiandentalacademy.com
  • 72. CARTILAGE FORMING TUMORS CHONDROSARCOMA • JUXTAEPITHELIAL • MESENCHYMAL • DEDIFFERENTIATED • CLEAR CELL • MALIGNANT www.indiandentalacademy.com
  • 73. MARROW TUMORS • EWINGS SARCOMA • PNET OF BONE VASCULAR TUMOR OF BONE • ANGIOSARCOMA OF BONE • MALIGNANT HEMANGIOPERICYTOMA www.indiandentalacademy.com
  • 74. Osteosarcoma • Malignancy of mesenchymal cells that have ability to produce osteoid or immature bone. • Most common primary tumor to orginate in bone after multiple myeloma • arises from undifferentiated cells & www.indiandentalacademy.com
  • 75. Etiology • UNKNOWN • Risk factors are radiation exposure • Genetic predisposition( 13q14) causing inactivation of RB gene leding to dev of retinoblastoma, os • Bone dysplasias fibrous dysplasia, pagets disease increase risk • Li-fraumeni syndrome ( germline t53 mutation) predispose to os www.indiandentalacademy.com
  • 76. • Can occur in any bone • Common in long bones • Extragnathic os shows bimodal age distribution • 10-20yrs( period of active growth) & >50yrs • Site: distal femoral & proximal tibial metaphyses. • Older pts axial skeleton & flat bones are involved www.indiandentalacademy.com
  • 77. Shoulder Pelvic Distal femur and proximal tibia Jaw www.indiandentalacademy.com
  • 78. • Os of jaws 6 to 8% of all os. • Occurs mostly in 3rd & 4th decade of life • Slight male predominance. • Maxilla < mandible • Mandible: posterior body, hori.ramus > ascending ramus • Maxilla : inferior portion( alveolar ridge, sinus floor,palate) > superior( zygoma, orbital rim) • Os of jaws are better differentited , better prognosis, less metastasis, than extragnathic os www.indiandentalacademy.com
  • 79. Clinical features: • Pain, swelling of involved area – facial deformity • Pt c/o sprain,arthritis or so called growing pain • Loosening of teeth • Paresthesia • Nasal obstruction in max. tumors • Epistaxis, hemorrhage • h/o recent tooth extraction with a nodular/polypoid some what reddish granuloma like growth growing from tooth socket. www.indiandentalacademy.com
  • 80. • As tumor grows, eroding cortical plates expansion is very firm because of dense fibrous tumor tissue produced. • Initially swelling is smoothly contoured, covered with normal appearing mucosa • When expansion becomes chronically traumatised mucositis develops on surface • Surface ulcerates and grayish white necrotic surface results which can be removed with tongue blade. www.indiandentalacademy.com
  • 81. juxta cortical os • Paraosteal: lobulated nodule attached to cortex by a stalk • No elevation of periosteum/its reaction • Hp: mass contain spindle cell fibroblast like proliferation • Well dev trabeculae of bone • They coalesce and form large solid mass of bone www.indiandentalacademy.com
  • 82. • Periosteal : • Sessile lesion • Arise with in cortex • Elevates overlying periosteum • Provokes production peripheral periosteal bone • Leading edge of tumor mass perforates periosteum extends into soft tissue www.indiandentalacademy.com
  • 83. Histopathology • Cells of tumor may producoe osteoid, chondriod, or fibrous connective tissue • Vary in shape uniform round/ spindle shaped to highly pleomorphic • Depending on relative amounts of osteoid, cartilage/collagen os is div into osteoblastic( 50%), chondroblastic, fibroblastic www.indiandentalacademy.com
  • 84. • Chondroblastic os are more common • Composed entirely of malignant cartilage growing lobules with only small foci of direct osteoid production by tumor cells • Other less common histologic variants are • Malignant histiocytoma like • Smallcell, epitheloid, telangiectatic, giant cell rich • Low grade well differentiated os – minimal cellular atypia , abundant bone formation. • May be misdiagnosed as fibrous dysplasia/ fibro osseous lesion www.indiandentalacademy.com
  • 86. Telangiectatic • Seen in adolescence & early adulthood • rare variant of central highgrade • Gross examination reveals a blood-filled cavity • Hemorrhagic & necrotic areas are seen in tumor • Microscopically dilated vascular channels lined with multinucleated giant cells and an anaplastic sarcomatous stroma with evident bone formation www.indiandentalacademy.com
  • 87. • Radiographically : large lytic defect, • usually expansile and accompanied by an extensive soft tissue component. • Mri- fluid levels similar to those seen in an aneurysmal bone cyst • Occasionally, this lesion may be very difficult to distinguish from an aneurysmal bone cyst. www.indiandentalacademy.com
  • 88. Radiology Internal structure: • Entirely rl/ osteolytic • Mixed rl-ro • Quite ro/ osteoblastic • Osseous structure may be granular/sclerotic apparing bone • In form of cotton balls, wisps, honey coomb internal structure with adjacent destruction of pre-exisisting osseous architecture. • What ever may be presentation, normal trabecular pattern is lost. www.indiandentalacademy.com
  • 89. Osteolytic: • Unicentric, ill defined borders • Moth eaten appearance • Perforation & expansion of cortical margins into sub periosteal bone www.indiandentalacademy.com
  • 90. Mixed • Ragged, ill defined borders • Ro due to excess bone formation intermingled with resorption leading to rl areas • In some sequestra appear , appear as well defined ro • Rl destruction may be in form of strands…honey comb appearance • Sun burst appearance • Cumulus cloud • Codmans triangle are seen www.indiandentalacademy.com
  • 91. Periphery and shape • ragged, Ill defined border • When viewed against normal bone Lesion is usually rl • No peripheral sclerosis / encapsulation • If it involves periosteum: • Typical sun ray spicules/hair on end trabeculae are seen • This occurs when periosteum is displaced, partially destroyed & disorganised. www.indiandentalacademy.com
  • 93. Cortical bone expansion and destruction….www.indiandentalacademy.com
  • 96. Effects on surrounding tissues • Widening of pdl space occurs ( garrington sign) • Band like widening along complete length of pdl space unilaterally/bilaterally • Also seen in other malignancies, osteoblastoma, • Pts undergoing ortodontic treatment • Unilateral bone resorption seen in periodontal disease. www.indiandentalacademy.com
  • 97. • Antral/nasal wall cortices are lost in maxillary lesions • Mand lesions destroy cortex of neurovascular canal, nv canal is symmetically widened & enlarged • Lamina dura of involved tooth is completely destroyed. www.indiandentalacademy.com
  • 98. • Dd: • If internal structure is minimal fibrosarcoma/ osteoblastic metastatic ca • Osseous structure visible Chondrosarcoma (affects older age group, more often involves maxilla) • Spiculated periosteal reaction Prostate/breast metastasis • Benign tumors & conditions like ossifying fibroma, fo lesioms mimic os radiographically. www.indiandentalacademy.com
  • 99. • Ossifying subperiosteal hematoma( a h/o recent trauma to the bone) • Peripheral fibroma with calcification( slow, benign growth) • Chronic osteomyelitis ( but infection is absent in os) • An important clinical differential feature is neurosensory loss. • rare osteomyelitis or neural loss from a previous biopsy or surgery, only malignancies can produce objective paresthesias. www.indiandentalacademy.com
  • 100. • radiographs or CT scans at right angles to the cortex should show extracortical bone and a destroyed cortex. • Fibrous dysplasia and ossifying fibroma will not have extracortical bone. The • extracortical bone seen in osteomyelitis with proliferative periostitis will be associated with an intact • cortex. • Even when other osteomyelitides produce extracortical bone, it is parallel to the cortex rather • than at right angles as is seen in osteosarcoma www.indiandentalacademy.com
  • 101. Chondrosarcoma • Malignant tumor characterised by formation of cartilage by tumor cells • Comprise 10% of all tumors of skeleton • Rare in jaws( 3% of all cs) • Extragnathic it is primarily neoplasm of adulthood with peak prevalance in 6th, 7th decades of life • Tumors arising in younger age group is uncommon • No sex or race predilection • Site: ileum, femur, humerus www.indiandentalacademy.com
  • 102. • In h& n : cs common in maxilla ( anterior areas where cartilage tissue is present. • mandible: ccoronoid process, condylar h& n, symphyseal region.less common in body, ramus, nasal septum, pns • Chondrosarcomas most often develop in osseous locations • But 1/3rd originate in laryngeal cartilage/soft tissue • Cs arise directly from cartilage or may occur within benign cartilagenous tumors….secondary cs www.indiandentalacademy.com
  • 103. • A painless mass, swelling • Associated with separation or loosening of teeth • Pain is unusual • Maxillary tumors may cause nasal obstruction, congestion, epistaxis, photophobia, or visual loss. • If cs occur near tmj trismus, abnormal joint function occurs. • When metastasis occurs lung is organ most commonly involved. www.indiandentalacademy.com
  • 104. • Hp: composed of cartilage with varying degrees of maturation & cellularity • Lobular pattern with lobules separated by thin fibrous connective tissue • Central area demonstrates lobules with greatest degree of maturity • Peripheral area contains immature cartilage & mesenchymal tissue consisting of round/spindle cells • Calcification/ossification may occur in chondroid matrix www.indiandentalacademy.com
  • 105. • Some times at periphery of lobules of high grade cs, a few fs like spindling tumor cells are present • Osseous trabeculae when present are seen at periphery of lobules & appears to be rimmed by osteoblasts. • When malignant cells produce osteoid lacework or trabeculae even in small foci, tumor is graded as os www.indiandentalacademy.com
  • 106. • Four histological subtypes: • Clear cell • Dedifferentiated • Myxoid • Mesenchymal • Occur centrally within bone or less commonly in soft tissue. www.indiandentalacademy.com
  • 107. Variants : • Clear cell cs: cells with abundant clear cytoplasm • Dedifferentiated cs: high grade malignancy that show admixture of well differentiated cs & malignnat mesenchymal tumor resembling fibrosarcoma. • Myxoid cs: soft tissue tumor. io variant is seen, characterised by clear, vacuolated, eosinophilic cytoplasm with a background of mucoid material.www.indiandentalacademy.com
  • 108. • Mesenchymal cs: uncommon tumor of soft tissue & bone • Shows biphasic hp • More common in soft tissue than bone • Chondroid tissue is well differentiated, degree of cellularity & atypia varies from benign chondroma to well differentiated chindrosarcoma • If cartilagenous foci is sparse, tumor may be misdiagnosed as hemangiopericytoma www.indiandentalacademy.com
  • 109. • Radiographically….internal structure: • radiolucent area with poorly defined borders • Rl area contains scattered ro foci caused by ossification of cartilage matrix • Mixed rl- ro appearance • Some times it appears as moth eaten bone alternating with islands of residual bone unaffected by tumor www.indiandentalacademy.com
  • 110. • Central ro structure appear as flocculent implying snow like features • Diffuse calcification may be superimposed on a bony background that resembles granular/ground glass appearing normal bone • Examination of this flocculence may reveal central rl nidus which is probably cartilage surrounded by calcification. www.indiandentalacademy.com
  • 111. • Periphery : • Generally roun/ovoid/lobulated • Well defined corticated borders • Occasionally peripheral periosteal reaction in form of sunray, hair on end appearance is seen • Uncommonly aggressive lesions ill defined, invasive , non corticated borders seen www.indiandentalacademy.com
  • 112. Effect on surrounding tissues: • Relatively slow growing so expands cortex, rather than rapidly destroying them • Mand cases, ian expanded still maintaining corical covering • Maxillary lesions push wall of sinus or nasal fossa & impinge on infratemporal fossa • Lesions of condyle cause expansion, remodelling of articular fossa & eminence. www.indiandentalacademy.com
  • 113. • If lesion is in articular disk region, widening of joint space, remodelling of condylar neck occurs. • Erosion of articular fossa occurs • If lesion occurs near tooth, root resorption, widening of pdl space, tooth displacement may occur. www.indiandentalacademy.com
  • 114. • Dd; • Os : typical calcifications of cs are absent • Fibrous dysplasia: periphery is well defined. ro portion of fibrous dysplasia is abnormal bone & not the calcifications • Because of their slow growth and especially their intact overlying mucosa, most cases will initially resemble a benign odontogenic tumor or a benign tumor of bone www.indiandentalacademy.com
  • 115. • Some punctate radiopacities are identifiable, the lesion will resemble a • calcifying epithelial odontogenic tumor • an ossifying fibroma • an immature osteoblastoma • a cavernous hemangioma of bone. • The more obviously aggressive presentations with irregular radiolucencies and perhaps neurosensory loss would be consistent with an intraosseous carcinoma, an osteosarcoma, and a malignant fibrous histiocytoma www.indiandentalacademy.com
  • 116. Ewings sarcoma • Notoriously aggressive and destructive malignancy of bone arising from marrow mesenchymal stem cells • Genetically and histologically distinctive small round cell sarcoma of bone • 85% to 90% cases, tumor cells show reciprocal translocation b/n chromosomes 11 & 22. (q24;q12) • Third most common osseous neoplasm than os, cs • It was first described by James Ewing in 1920 as a "diffuse endothelioma of bone.“www.indiandentalacademy.com
  • 117. • In both the jaws and long bones,peak age of occurrence is in the teenage years (50%). Young men slightly > young women • mandible's posterior body, the angle and ramus regions are common. Cause bony expansion , mobile teeth, extensive destruction of bone and necrosis…fever occurs • present a picture similar to that of an osteomyelitis. • rapid growth rate. Metastasis is common.www.indiandentalacademy.com
  • 118. • Hp : Ewing sarcomas are composed of densely packed, rather uniform cells with little intercellular stroma • The nuclei are rounded to oval with defined nuclear borders and a • finely granular chromatin pattern • The cytoplasm is indistinct and may be vacuolated. • The cells are two to three times the size of a lymphocyte. • Mitoses are infrequent. • Rapid growth, undergo considerable necrosis, sometimes resulting in a perivascular pattern of viable tumor cells www.indiandentalacademy.com
  • 119. Tumor cells are arranged in broad sheets…filigree pattern in which infiltratin strands of tumor cells are separated by thin fibrovascular septae Well defined nuclei Indistinct cytoplasm www.indiandentalacademy.com
  • 120. • Hp dd: includes other small round cell tumors, including • neuroblastoma,lymphoma, small cell osteosarcoma& embryonal rhabdomyosarcoma. • Ewing sarcoma will usually have intracytoplasmic glycogen granules demonstrated by periodic acid‐Schiff (PAS) and diastase staining. • neuroblastoma and embryonal rhabdomyosarcomas may also yield positive staining.( for glycogen) www.indiandentalacademy.com
  • 121. Rf: • Internal structure: • Es is a destructive process with little unduction of bone. • It commences on internal aspect of bone & involves endosteal & periosteal surfaces later , usually entirely rl. • Periphery: rl poorly demarcated & never corticated. • Advancing end destroys bone in an uneven fashion. • May cause pathological fractures. • Extends into adjacent soft tissue. www.indiandentalacademy.com
  • 122. Effect on surrounding tissues: • adjacent normal structures ian canal, lower border of mandible, alveolar cortical plates are destroyed. • It doesnot characteristically cause root resorption, it destroys supporting bone adjacent to tooth. www.indiandentalacademy.com
  • 123. • Panoramic radiographs and a CT scan • an ill‐defined, irregular resorption of bone with focal areas of residual bone resembling sequestra. • Pathologic fractures are common, attesting to the degree of bone destruction. • Multilayered periosteal reaction that has been described as an "onion skin" appearance, • (Similar to proliferative periostitis in osteomyelitis) • such a radiographic appearance is almost never seen when Ewing sarcoma arises in the jaws. www.indiandentalacademy.com
  • 124. • Ewing sarcoma in the jaws will produce a destructive radiolucency with resorbed tooth roots and displaced teeth. • On rare occasions, a Ewing sarcoma may produce a periosteal new bone formation perpendicular to the cortex and thereby create the "sun‐ray" appearance more frequently seen in osteosarcomas www.indiandentalacademy.com
  • 125. • Dd : • Pain , fever, leukocytosis suggest a suppurative osteomyelitis. • Reinforced by radiographs showing destructive bone pattern , bone foci resembling a sequestrum, layered periosteal "onion skin“ appearance…resembling proliferative periostitis. www.indiandentalacademy.com
  • 126. • Other aggressive malignancies that occur in this young age group include • Rhabdomyosarcoma , • Osteosarcoma, fibrosarcoma, and neuroblastoma • Eosinophilic granuloma of jaw is also a destructive process, but is associated with laminar periosteal reaction, where as in jaws ewings sarcoma doesnot produce. www.indiandentalacademy.com
  • 128. • periosteum is a membrane several cell layers thick that covers almost all of every bone. • only parts not covered by this membrane are the parts covered by cartilage. • Besides covering the bone and sharing some of its blood supply with the bone, • it also produces bone when it is stimulated appropriately. • Practically anything that breaks, tears, stretches, inflames, or even touches the periosteum. www.indiandentalacademy.com
  • 129. •With slow-growing processes, the periosteum has plenty of time to respond to the process. •It can produce new bone just as fast as the lesion is growing. • solid, uninterrupted periosteal new bone along the margin of the affected bone. www.indiandentalacademy.com
  • 130. •In case of rapid growing tumors, new bone is not formed at the rate of tumor. •An interrupted pattern is formed •This may result in a pattern of one or more concentric shells of new bone over the lesion. This pattern is sometimes called lamellated or "onion-skin" periosteal reaction. www.indiandentalacademy.com
  • 131. •If the lesion grows rapidly but steadily, the periosteum will not have enough time to lay down even a thin shell of bone, •In such cases, the tiny fibers that connect the periosteum to the bone (Sharpey's fibers) become stretched out perpendicular to the bone. •When these fibers ossify, they produce a pattern sometimes called "sunburst" or "hair-on-end" periosteal reaction, depending of how much of the bone is involved by the proceswww.indiandentalacademy.com
  • 132. • Codmans triangle : • When a process is growing too fast, it penetrate through the cortex causing separation of the periosteum and formation of lamellated new bone. If the periosteum elevates to a significant degree, it can break forming an acute angle www.indiandentalacademy.com
  • 133. • When this little bit of ossification is seen tangentially on a radiograph, it forms a small angle with the surface of the bone, but not a complete triangle.. www.indiandentalacademy.com
  • 134. • References 1. Robert E Marx, Diane Stern Oral And Maxillofacial Pathology: A Rationale For diagnosis & Treatment. 1st edition, Quintessence.2003. 2. Stuart C White , Michael J Pharoah , Textbook Of Oral Radiology Principles And Interpretation 3. Norman k wood paul w goaz. Dd of oral and maxilllofacial lesions. 5th edition elsevier 2007 www.indiandentalacademy.com
  • 135. • R Rajendran B Sivapathasundharam Shafer’s Textbook Of Oral Pathology. 5th Edition, Elsevier, 2008 • Brad W Neville Douglas D Damm Carl M Allen Jerry E Bouquot. Oral And Maxillofacial Pathology. 3rd Edition Elsevier, 2009 www.indiandentalacademy.com

Notas do Editor

  1. (although the word cancer was not used)
  2. finger-like spreading projections from a cancer is similar to shape of a crab.
  3. similar to hamartomas
  4. Another was given by us national cancer institute
  5. more immature cell in this sequence and therefore have a more aggressive behavior
  6. second most common sarcoma in adults (after the malignant fibrous histiocytoma) when all areas are considered
  7. Per se…by itself……..
  8. Moritz Kaposi first described this form in five individuals in 1872
  9. Characteristically, individuals live with these lesions and die of unrelated causes years later
  10. Benign tumor….but has definite malignant counterpart…telengiectasia/angioectasias (also known as spider veins) are small dilated blood vessels[1] near the surface of the skin or mucous membranes, measuring between 0.5 and 1 millimeter in diameter.[2]
  11. Spindle cells are of mesenchymal origin, and form the body's connective tissue, fat, muscle, bone, cartilage and blood vessels
  12. Serum alk phosphatase level is increased……. Pathological # uncommon( expect telangiectatic)
  13. Pathologic #
  14. Metastasis hematogenously…..primarily to lungs…
  15. Osteolytic type is least differentiated and carries poor prognosis….
  16. Sun ray not seen….
  17. If periosteum is elevated, maintains its osteogenic potential, but is breached in centre, codmans triangle is formed
  18. Due to infiltration of tumor mass into pdl space……….
  19. Ossifying fibroma is better demarcated and it has uniform internal architecture.
  20. translocation of chromosomes 11 and 22 at their respective q24 and q12 loci…..tumor of long bones…relatively rare in jaws…
  21. It is rare in the mandible in general and even more rare in the maxilla
  22. Filigree… ornamental work of fine (typically gold or silver) wire formed into delicate tracery.
  23. seen occasionally when Ewing sarcoma arises within the diaphysis of long bones.
  24. This interrupted pattern can manifest itself in several ways, depending on just how steadily the lesion grows. If the lesion grows unevenly in fits and starts, then the periosteum may have time to lay down a thin shell of calcified new bone before the lesion takes off again on its next growth spurt.
  25. pattern may appear quite different.
  26. Codmans triangle : another pattern in rapidly growing tumors a bit of a misnomer ( not complete triangle) When a process is growing too fast for the periosteum to respond with even thin shells of new bone, sometimes only the edges of the raised periosteum will ossify. When this little bit of ossification is seen tangentially on a radiograph, it forms a small angle with the surface of the bone, but not a complete triangle.. a bit of a misnomer ( not complete triangle)
  27. Robert E. Marx (Author), Diane Stern (Author)