3 - bone neoplasms Flashcards by Luke Lloyd

3 benign neoplasms of bone

central ossifying fibroma (hyperparathyroidism-jaw tumor syndrome)

Osteoma (gardner syndrome)

central giant cell granuloma (hyperparathyroidism, renal osteodystrophy)

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3 malignant neoplasms of bone

chondrosarcoma

osteosarcoma

metastatic disease

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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

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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

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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

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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

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Tx for central ossifying fibroma

Px and recurrence?

enucleation - lesion tends to shell out as one mass

excellent Px and recurrence uncommon

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multiple jaw lesions that histopathologically are consistent with central ossifying fibroma

hyperparathyoid jaw tumor syndrome

hyperparathuroidism - hypercalcemia

increased risk for development of parathyroid carcinoma

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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

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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)

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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

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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

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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

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radiographically endosteal within bone osteoma looks like _

idiopathic osteosclerosis or condensing osteitis

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histopathological features

dense bone, minimal marrow or trabeculae and fibrofatty marrow

osteoma

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osteoma tx Px

tx - non if small and sympto - observe

conservative excision
condylar osteomas usually symptomatic - local resection or condylectomy

Px - good

rare recurrence

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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

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most common cancers seen in bone

metastatic cancer
multiple myeloma
osteosarcoma
condylata sarcoma

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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

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multiple osteoma - Gardner syndrome

intestinal polyps; one of the multiple intestinal polyposis syndromes

colorectial polps develop by 2nd decade, ultimately transform to _

adenocarcinoma

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multiple osteomas, develop around puberty
epidermoid cysts
desmoid tumors (locally aggressive fibrous neoplasms of soft tisssue)

Osteoma - Gardner syndrome

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osteoma and Gardner Syndrome abnormalities of teeth

impacts
supernumerary teeth ~35%
odontomas ~10% cases

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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