3 - bone neoplasms Flashcards
3 benign neoplasms of bone
central ossifying fibroma (hyperparathyroidism-jaw tumor syndrome)
Osteoma (gardner syndrome)
central giant cell granuloma (hyperparathyroidism, renal osteodystrophy)
3 malignant neoplasms of bone
chondrosarcoma
osteosarcoma
metastatic disease
benign
confused with focal cemento-osseous dysplasia in past
orging PDL? Odontogenic? ~controversy
these tumors also seen outside the jaws
most consider it to be an osteogenic neoplasm
central ossifying fibroma
central ossifying fibroma
common/uncommon young or adult male or female symptomatic/asympto expansion or none
where in mouth
uncommon - rare
adult 3/4 decade
female predilection
asymptomatic esp when small
may see jaw expansion - facial asymm to significaint deformity
primarily mandibular molar/PM region - but also maxilla
radiographic features
usually uniocular (occasionally multilocular
well circumscribed with varying amount of central opacity - range from almost completely radiolucent to radiodense with a defined lucent border
resembles focal cemento-osseous dysplasia
central ossifying fibroma (benign)
can cause expansion and root divergence
histopathic features
fibrous tissue with variable mixture of bony trabeculae and cementum like material
mineralized product in cellular fibrous CT
microscopically similar to fibrous dysplasia: need radiograph for correlation
central ossifying fibroma
usually shells out in one piece
Tx for central ossifying fibroma
Px and recurrence?
enucleation - lesion tends to shell out as one mass
excellent Px and recurrence uncommon
multiple jaw lesions that histopathologically are consistent with central ossifying fibroma
hyperparathyoid jaw tumor syndrome
hyperparathuroidism - hypercalcemia
increased risk for development of parathyroid carcinoma
benign
palatal tori, mandibular tori, buccal exostoses are NOT considered to be _, although they are histopathologically identical
composed of compact or cancellous bone
osteomas
osteomas are confirmed by continuous growth
NOT osteomas
Osteoma - benign
young/adult
sympto/asymptp
fast/slow growing
adult
usually painless, but pain/swelling possible
slow growing
may arise on the bone surface (exostosis) or within bone (enostosis)
benign bone neoplasm
paranasal sinus involvement (frontal, ethomoid and maxillary) more common than gnathic lesions
if gnathic lesions - body of mandible and condyle most often, (inferior border of angle. coronoid process and ramus also)
osteoma
osteoma clinical locations
paranasal sinus - most often
body of mandible often lingual PM/molar region
condylar involvement may limit opening - maloclussion- midline deviation and chin to UNAFFECTED SIDE
radiographic features
circumscribed radiopaque mass
periosteal may be uniformly sclerotic or have central trabeculations
endosteal may appear similar/idential to idiopathic osteosclerosis or condensing osteitis
osteoma
radiographically endosteal within bone osteoma looks like _
idiopathic osteosclerosis or condensing osteitis
histopathological features
dense bone, minimal marrow or trabeculae and fibrofatty marrow
osteoma
osteoma tx Px
tx - non if small and sympto - observe
conservative excision
condylar osteomas usually symptomatic - local resection or condylectomy
Px - good
rare recurrence
if multiple osteomas - need to rule out _, uncommon autosomal domintant, highly penetrant (most people with the mutation will develop the disease)
~30% new mutations
gardner syndrome
most common cancers seen in bone
metastatic cancer
multiple myeloma
osteosarcoma
condylata sarcoma
one of several polyposis syndromes
most significant aspect is development of precancerous polyps of the colon
osteoma - Gardner syndrome
generally refers to cases in which the extra intestinal manifestations are especially prominent
multiple osteoma - Gardner syndrome
intestinal polyps; one of the multiple intestinal polyposis syndromes
colorectial polps develop by 2nd decade, ultimately transform to _
adenocarcinoma
multiple osteomas, develop around puberty
epidermoid cysts
desmoid tumors (locally aggressive fibrous neoplasms of soft tisssue)
Osteoma - Gardner syndrome
osteoma and Gardner Syndrome abnormalities of teeth
impacts
supernumerary teeth ~35%
odontomas ~10% cases
Osteoma - Gardner syndorme
can resemble _ cemento-osseous dysplasia or _
radiopacities ranging from a few mm to several cm in diameter
florid COD
or fibrous dysplasia
histopatholgoical features
keratinized stratified squamous epi with keratin debris in the lumen of epidermoid cyst
consistent with normaly findings in osteoma and epidermoid cysts
osteoma - gardner syndrome
tx is prophylatic colectomy
removal of cosmetically problematic cysts and osteomas
genetic counseling
Px - guarded - follow up
50% of patients develop adenocarcinoma of the colon by 30 years old
gardner syndrome
Central giant cell granuloma
benign/malignant
etiology?
CGCG vs giant cell tumor of bone
young or old
male or female
mandi or max
anterior or posterior
sym/asymp
expand or no
benign
unknown etiology
tumor of bone - arises especially in knee distal femur
broad age - most before 30
2:1 female
mandible usually often crosses midline
more common anterior
asymptomatic
can expand - locally aggressive behavir (root resoprtion)
can break thru bone and mvoe into soft tissue
radiographic features
unilocular/multioclular radiolucency
range in size from a few mm to several cm
well-delineated, generally not corticated
root resorption suggests aggressive behavior, but root divergence suggests benign process
central giant cell granuloma
histopathological features
same as brown tumor of hyperparathyroidism, peripheral giant cell granuloma and giant cell tumor of bone
need radiographic correlation and blood test for parathromone PTH
central giant cell granuloma
tx for central giant cell granuloma
Px
recurrence
tx - aggressive curettage/ peripherl osteotomy - rule-out hyperparathyroidism
Px - good
15-20% recurrence
2 types of primary or secondary hyperparathyroidism
parathroid hyperplasia, parathyroid adenoma or carcinoma - inappropriate secretion of PTH causing increased Ca++ in blood
primary
2 types of primary or secondary hyperparathyroidism
renal failure, altered vitamin D metabolism - poor calcium retention
secondary
inadequate production of Vitamin D - which is needed to absorb Ca from the gut – increased serum Ca
central giant cell granuloma
PTH
_ activation causing increased _ uptake by kidneys
osteoclastic activation
increased Ca uptake by kidneys
increase vitamin D synthesis (kidneys) which promotes Ca++ from the gut
“bones, stones, groans, moans” thrones
radiographic features
loss of lamina dura
“ground glass” trabecular pattern
unilocular/multilocular radioluceny
hyperparathyroidism
brown tumor of hyperthyroidism renal osteodystrophy (enlargement of jaws due to long -standing renal failure)
cystic change of _ asspear as giant cell tumor of bone
hyperparathyroidism
activates osteoclastic
histopathologic features
brown tumors show vascular granulation tissue with extravasated erythrocytes and numerous benign multinucleated giant cells
microscoppically the same as Peripheral giant cell granuloma, CGCG and giant cell tumor of bone
hyperparathyroidism
hyperparathyroidism feature
unusual hyprplastic response of bone in patients with poorly controlled secondary hyperparathyroidism
often seen in patients on long-term renal dialysis
may manifest as prominent jaw enlargement
renal osteodystrophy
hyperparathyrodism tx
Px
remove source of hormone secretion if primary (remove parathyrodi
better control of cerum Ca if secondary - parathyroidectomy may be needed - renal transplant
px - fair - associated with etiology
malignancy of cartilaginous differentiation
chondrosarcoma
chondrosarcoma
common/rare
male/female
young or adult
bones affected
sympto/asympt
rare - but 2nd most common PRIMARY bone malignancy
male - 4th-6th adult
mainly femur, pelvis, or ribs - 10% head and neck
- may present with pain or swelling(may miic dental infection
2nd most common primary bone malignancy 10% head and neck
may mimic dental infection pain swelling loose teeth
chondrosarcoma
primary - meaning from bone tissue
radiographic features
poorly defined radioluceny with variable amounts of radiopacity
larger lesions may appear multilocular
may see widened PDL in area of tumor
loss of lamina dura - teeth test vital
chondrosarcoma
histopathological features
invasive lobules of atypical cells showing cartilaginous differentiation
lobules of cartilage with hyperchromatic cells
eggshell lick chamber with vacuolar space holding the chondrocyte
chondrosarcoma
chondrosarcoma tx
px - metastasis especially to _
radical surgerh “one chance for cure”
px - depend on location and histo grade of tumor but generally poor
death usually by direct extension of tumor involving vital structures
metastasis especiallly to lung
Osteosarcoma
rare or common
bones affected
young/adult
rare ~900 cases in US annually - most common primary bone malgnacny;
8% head and neck
10% shoulder
15-pelvis
60 - knee femur- long bones usually around knee
younger ~18
jaws ~28
pain often initial compliant long bones and jaws
most common primary bone malignancy
8% affect jaws
osteosarcoma
twice as common as chondrosarcoma
swelling, loose teeth, test teeth vital
paresthesia
radiographic features
“sunburst” appearance pattern uncommon in jaws
mixed radiolucent/radiopaque with ill-defined borders
symmetrically widened PDL of teeth in the area
calcification filling sinus - widened PDL - spiked appearance of roots
osteosarcoma
histopathological features
infiltrating sheets of malignant spindle cells
production of osteoid by tumor cells
mitotic activity variable
osteoblastic, chondroblastic, and fibroblastic differentiation can be seen
osteosarcoma
osteosarcoma tx
Px, metastasis especially to _
radical surgery go for cure
more recently induction chemotherapy, then surgery to remove the tumor
px FAIR - 5 year survival 30-50%, better Px for jaws
one study using radical surgery had 80% 5 year survival
esp. to lung - death usually due to uncontrolled disease
metastatic disease
overall metastases typically go to _
jaws occasionally affected ~_% of all oral malignancies
bone
1%
oral soft tissues can be affected as well
must be aware of this diagnosis due to importance
the most common form of cancer involving bone
metastatic disease
then multiple myeloma
metastasis from _ may affect jaws via Batson’s paravertebral plexus of veins
from below the neck
batsons plexus - a valveless vertebral venous plexus - may allow retrograde spread of tumor cells, bypassing filtration thru the lungs
connects deep pelvic veins and thoracic cenis in the internal vertebral venous plexuses
metastatic disease most common cancer in bone
young or old
bones?
over 1/2 of affected patients are greater than 50
mandible 61%
maxilla 24%
soft tissue 15% (gingiva 54%, tongue 22.5%)
paresthesia, tooth mobility, swelling, hemorrhage, pathologic fracture, trismus - lack of a healing tooth socket consider _ _ _
granulation tissue
lymphoma
metastatic disease
radiographic features
poorly defined radioluceny less commonly radiopacity
moth eaten
histo - same pattern as primary malignancy
“seeded effect” from scattered clusters of cells
most common _______
metastatic disease
breast, lung, colon, thyroid, prostate, kidney, melanoma
metastatic disease tx and px
_%of jaw metasases are initial manifestation
tx - palliation - radiation therapy
px = very poor - most patients die within 1 year of the dx
22% of jaw metastases are initial manifestation
