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83 Cards in this Set

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What are the 4 malignant BONE tumors we learned about?
1. Osteosarcoma (Osteogenic Sarcoma)
2. Chondrosarcoma
3. Ewing Sarcoma
4. Metastatic Tumors to the Jaws
What's another name for Osteosarcoma?
Osteogenic Sarcoma.
What's another name for Osteogenic Sarcoma?
Osteosarcoma
What is the MOST COMMON Primary Malignancy of bone?
Osteosarcoma.
Osteosarcomas of the jaw are rare. T/F?
True- Osteosarcoma is the MOST common primary malignancy of bone. Regardless, osteosarcomas of the Jaw are rare- accounting for only 6-8% of osteosarcomas.
List the 3 contributing factors for Osteosarcoma:
1. Paget Disease- 2 to 5% of Paget cases develop osteosarcoma.
2. Prior irradiation treatment for benign soft tissue or bone lesions.
3. Osteogenesis imperfecta
Osteogenesis Imperfecta is a contributing factor for Osteosarcoma. T/F?
True- along with
1. Paget Disease- 2 to 5% of Paget cases develop osteosarcoma.
2. Prior irradiation treatment for benign soft tissue or bone lesions.
Paget Disease is not a contributing factor for Osteosarcoma. T/F?
False- it is!
1. Paget Disease- 2 to 5% of Paget cases develop osteosarcoma.
2. Prior irradiation treatment for benign soft tissue or bone lesions.
3. Osteogenesis imperfecta
Smoking is a contributing factor for Osteosarcoma. T/F?
False. These 3 are:
1. Paget Disease- 2 to 5% of Paget cases develop osteosarcoma.
2. Prior irradiation treatment for benign soft tissue or bone lesions.
3. Osteogenesis imperfecta
Your patient has been treated for benign soft tissue lesions in the past with radiation as therapy. This form of treatment is a contributing factor for what type of Malignant Bone Tumor?
Osteosarcoma (Osteogenic Sarcoma)
What is the age predilection for Osteosarcoma?
3rd and 4th decades.
The 2nd & 3rd decades are the age predilection for Osteosarcoma. T/F?
False- 3rd and 4th decades.
The 4th & 5th decades are the age predilection for Osteosarcoma. T/F?
False- 3rd and 4th decades.
What is the average age for Osteosarcoma?
33 years old- this is 10-15 year older than for skeletal lesions.
Is the average age for Osteosarcoma older or younger than for skeletal lesions? What is this average age?
The average age for Osteosarcoma is 33 years old- this is 10-15 years older than for skeletal lesions! Most are 18-22 years old.
The maxilla is more commonly affected in Osteosarcoma. T/F?
False- mandible & maxilla are about equally affected.
The mandible is more commonly affected in Osteosarcoma. T/F?
False- mandible & maxilla are about equally affected.
What is the site of predilection for Osteosarcoma?
Mandible & maxilla are about equally affected.
What are the most common symptoms of Osteosarcoma?
Pain and swelling
Pain, Swelling and toothache are the most common symptoms of Osteosarcoma. T/F?
False- Pain and swelling are the most common symptoms of Osteosarcoma. Toothache is a symptom, but not one of the most common.
Pain and swelling are the most common symptoms of which Malignant Bone Tumor?
Osteosarcoma.
Your patient presents with pain, swelling, loose teeth, toothache, and paresthesia. Because you know your S/S of diseases like the back of your hand you automatically think that these 5 symptoms beautifully correlate with which Malignant Bone Tumor?
Osteosarcoma
Pain and swelling are the most common symptoms of Osteosarcoma. What are the other 3 symptoms/signs present?
1. Loose Teeth
2. Toothache
3. Paresthesia
How does Osteosarcoma present radiographically?
1. Bony expansion with opaque to mixed lucency/opacity pattern.
2. Sunburst pattern - 25% - from peripheral new bone formation
3. Widened PDL space- may be the earliest sign!
What may be the earliest sign of Osteosarcoma?
A widened PDL space
Your patient presents with pain, swelling, loose teeth, toothache, paresthesia and now a widened PDL space. What do you suspect?
Osteosarcoma
A sunburst pattern is seen in osteosarcoma which is from Periphernal new bone formation. What % is this?
25%
24% of Osteosarcomas show the Sunburst Pattern. Where does this come from?
From peripheral new bone formation
There is bony expansion seen in Osteosarcoma. T/F?
True- with opaque to mixed lucency/opacity pattern.
Histologically, what does Osteosarcoma look like?
1. Atypical, bizzarre Osteoblasts
2. Osteoblasts in disorderly array around cellular bony trabeculae
3. May also have atypical chondroid elements
What is the treatment of choice for osteosarcoma is?
Radical Surgical Excision
Chemotherapy is used for Osteosarcoma. T/F?
True- it may be used to control metastases. Pulmonary metastases is seen in 50% of patients!
Radiation therapy is very effective for Osteosarcoma & chemotherapy as well. T/F?
False- Radiation therapy is NOT very effective for osteosarcoma. However, chemotherapy is used to control metastases.
Prognosis is : good, bad, poor
for Osteosarcoma?
Very POOR for both skeletal and jaw lesions, however with newer tx moalities 5 yr survival rates for skeletal lesions have improved to 60%. Jaw lesions currently is not as good as for skeletal lesions.
Survival rates for jaw lesions seen in osteosarcoma vary from ___% to ___%, depending on study. Up to ___% survival has been reported with initial radical surgery.
Survival rates for jaw lesions seen in osteosarcoma vary from 30 % to 70 %, depending on study. Up to 80% survival has been reported with initial radical surgery.
A malignancy of mesenchymal cells having the ability to produce osteoid or immature bone (aka malignancy of osteoblasts) that most often occurs in 20-40 year olds is seen in your patient. Upon examining her xray you see triangular elevation of the periosteum (Codman's Triangle) as well as opaque spiking at crestal bone ("spiking" resorption = resorption of roots making roots looking very tapered and narrow) as well as a symmetrical widening of the PDL space. What does your patient have and how will you tx her?
Osteosarcoma.
Tx = RADICAL SURGICAL EXCISION & CHEMOTHERAPY to control metastases.
A systemic Dz defined as an absolute decrease in the amount of bone below the levels required for adeuqate mechanical support is known as?
Osteoporosis
Metabolic bone resorption causes Osteoporosis. It is seen in the elderly and particularly affects postmenopausal females. It can be severe enough to produce radiographically detectable changes in bone. What diagnostic radiographic jaw changes do we see?
We do NOT see any diagnostic radiographic changes in osteoporosis!
What is a generic term that refers to increased amounts of calcified bone?
Osteosclerosis (NOS- not otherwise specified)
Osteosclerosis may be seen in patients with:
1. Metastatic tumors- prostrate & breast
2. Lead poisoning
3. Hypothyroidism(helps preserve bone mass- a good consequence).
T/F?
True
Idiopathic Osteosclerosis of the Jaws is a distinct clinicopathologic disorder. T/F?
True
What are 2 other names for Hyperparathyroidism of the bone?
1. Osteitis Fibrosa Cystica
2. Von Recklinghausen's Dz of Bone
Osteitis Fibrosa Cystica is another name for what?
Hyperparathyroidism of the Bone (and von Recklinghausen's Dz of Bone)
What is characterized by generalized bone loss (diffuse bony changes seen in all 4 quadrants!) and replacement by fibrous tissue, has predilection for MIDDLE AGED females and ground glass appearance with loss of lamina dura?
Hyperparathyroidism of the Bone
Primary Hyperparathyroidism of the Bone is caused by what?
Primary Hyperparathyroidism of the Bone is caused by a FUNCTIONAL BENIGN PARATHYROID ADENOMA.
In Primary Hyperparathyroidsm of the Bone we see an increase in PTH secretion which leads to elevated serum calcium. This is associated with Bone RESORPTION and bone lesions as Calcium is liberated from the skeleton. What 3 other findings are important? (They all rhyme)
1. Stones = urolithiasis, nephrolithiasis & cholelithiasis
2. Moans = abdominal pain from Peptic Ulcer Dz
3. Groans = Psychiatric changes
Peptic Ulcer Dz can cause what feature of Primary Hyperparathyroidism of Bone?
Moans
Secondary Hyperparathyroidism of Bone is caused by Poor renal function as seen in Chronic Renal Failure. This results in a lack of what Vitamin?
Vitamin D3 which causes a decreased Calcium ABSORPTION. Calcium may be also lost in the urine.
Calcium is lost in the urine in which type of Hyperparathyroidism?
Secondary
In Secondary Hyperparathyroidsim there is retention of PO4 occurs as well as parathyroid hyperplasia with increased output of PTH occuring to compensate for what?
Compensate for the low levels of serum calcium. As calcium is mobilized from the bones, bone lesions develop from EXCESSIVE resorption.
Pathologic bone fractures occur in Hyperparathyroidism of the Bone and are Common in the Jaws. There also may be a shifting of teeth. T/F?
FALSE- pathologic fractures DO occur but are NOT common in the Jaws.

TRUE- shifting of teeth may occur.
Radiographically we see:
1. Ground glass appearance
2. Loss of Lamina Dura
3. Multilocular Radiolucencies
It occurs in Middle-aged females and has a generalized bone loss and replacement of Fibrous Tissue. What disease is this?
Hyperparathyroidism of Bone
Histologically we see:
1. Cellular fibrous stroma, much like granulation tissue
2. Very vascular and thus associated with FOCI OF HEMORRHAGE, giving lesion a BROWN COLOR GROSSLY. Hence lesions are referred to as what?
3. Multinucleated Giant Cells
4. Scattered Bone Trabeculae (residual)
This is what Dz?
Hyperparathyroidism of the Bone.

Lesions are referred to as BROWN TUMORS of HYPERPARATHYROIDSM
Tx for primary & secondary Hyperparathyroidism of bone is?
primary = remove parathyroid adenoma. Brown Tumor can complete resolve!
Secondary = attempt to control contributing factors- chronic renal Dz
Can brown tumors completely resolve in primary hyperparathyroidism?
Yes!
Hyperparathyroidsm:
1. All 4 quads involved
2. Older Adults
3. Ground glass
Compare these 3 features to Fibrous Dysplasia
Fibrous Dysplasia
1. One quad
2. Young person
3. Ground glass
What are the 3 metabolic Bone Diseases we learned?
1. Osteoporosis
2. Osteosclerosis
3. Hyperparathyroidism- primary & secondary
What are the inherited Dz of Bone that we learned?
1. Osteopetrosis
2. Cherubism
3. Tori & Exostoses
Osteopetrosis is a rare genetic Dz. What are the 3 other names for it?
1. Marble Bone Dz
2. Albers-Schonberg Dz
3. Osteosclerosis Fragilis Generalista
Osteopetrosis is also known as Marble Bone Dz because it is dense and hard. Basic Features include:
1. Normal Bone Apposition
2. Abnormal to NO bone RESORPTION = increase density of bone throughout the skeleton- affects the WHOLE body!
3. Blue Sclera
4. Bone marrow gets narrow & vascularity is diminished
What Laboratory Finding will be increased in the Benign form?
Benign form shows Increased Serum Acid Phosphatase
Benign Osteopetrosis has a middle age to older onset vs the Malignant form which has Neonatal onset. Which one is Dominant and which is recessive?
Benign = Hereditary- Dominant
Malignant = Recessive- death by 20 yrs, no known survivers
Your patient is a 30 yr male who claims to have Malignant Osteopetrosis. He acquired it as a neonate. Common manifestations he had were:
1. Optic atropy
2. Hepatosplenomegaly
3. Loss of Hearing
4. Pathologic Fractures
5. Secondary Infection.
What do you tell him?
Those manifestations are true
(1. Optic Atrophy
2. Hepatosplenomegaly
3. Loss of hearing
4. Pathologic fractures
5. Secondary Infection)
however it doesn't make sense that he is still alive bc death is supposed to be by 20 years and here are NO known survivors.
Benign Osteopetrosis' common manifestations include:
1. Multiple Patholgic fractures (40% cases)
2. Pain- bc pressure on sensory nerves
3. Cranial Nerve Palsy- common
4. Osteomyelitis.
What is the age of onset?
Middle aged to older onset
Your patient is a middle aged woman who presents with:
1. Multiple pathologic fractures
2. Pain
3. Cranial Nerve Palsy
4. Osteomyelitis.
She acquired this Dz through Mendelian Dominant fashion and her lab results show an increased serum acid Phosphatase.
She said her previous doc said she had some bone Dz. Based on these findings what does she have?
Benign Osteopetrosis
Your 5 year old pt presents with a bone dz involving most of her skeleton. She presents with:
1. Pathologic Fractures
2. Secondary infections
3. Loss of hearing
4. Hepatosplenomegaly
5. Optic Atrophy
What Dz does she have?
Malignant Osteopetrosis
Upon doing a Comp Oral Eval you see:
1. Bone sclerosis due to delayed eruption
2. Premature exfoliation due to defect in the PDL
3. Enamel hyPOplasia
4. Predisposition to Osteomyelitis
5. Pathologic Jaw Fracture.
What Dz does your pt have?
Osteopetrosis
Osteopetrosis predisposes to Osteomyelitis. T/F?
True
You will see Enamel Hyperplasia in Osteopetrosis. T/F?
FALSE- you will see enamel HYPOplasia in Osteopetrosis
Radiographically you see:
1. Dense Homongenous Milky Opaque changes
2. Increase Cortical Thickening
3. Tooth Roots may be obscured by Dense Bone
Your pt has blue sclera.
What Dz does she have?
Osteopetrosis
Histologic Features of Osteopetrosis include:
1. Decreased Osteoclasts- younger pts = Malignant
2. Osteoclasts may be deficient in what?
3. Adults = abnormal collagen matrix.
RNA
Tx for Ostepetrosis is?
There is NO effective Tx!
Increased PTH has caused some resorption
There is no effective TX for Osteopetrosis. What can you give your pt to cause some resoprtion?
Increase PTH
Penetrance for males having Cherubsim is 50-75%. T/F?
FALSE- Males have 100% penetrance.

Females have 50-75% penetrance
Cherubism is a genetic abnormality that is inheretied as an Autosomal Dominant mendelian Disorder. 100% penetrance in males (male dominance) and 50-75% prenetrancein females. Mutations have been mapped to which chromosome?
Chromosome 4p16
Mutation in Cherubism involves which gene?
SH3BP2- it codes for a protein involved insignal transduction to upregulate osteoblastic and clastic activity. Mutation may lead to pathologic acitivation of OSTEOCLASTs and disruption of morphogenesis in jaws
Clinical features of Cherubism include:
1. Bilateral symmetrical swelling of mandible
2. Maxilla-displacemnt of orbits looking up
3. Pigmented skin lesions
What age is the onset?
Childhood- ages 3 to 4 yrs
Dental finding in Cherubism include:
1. Premature Loss of Deciduous Teeth
2. Defective Permanent teeth which include---
1. Missing
2. Displacesd
3. Lack of eruption
Radiogrpahic features noted:
1. Soap bubble pattern of multilocular lucency-classic
2. Lesions bilateral and symmetric, involving mandible and the max
3. Teeth may be dsiplaced or "floating in the air"
what Dz?
Cherubsim
Histologic Features of Cherubism include:
1. Fibrovascular stroma with scattered ginant cells
2. Perivascular eosinophilic cuffing
3. Very similar to what type of granuloma?
Central Giant Cell Granuloma
Cherubism can bed tx with Radiation. T/F?
FALSE- do NOT tx with radiation!
Pathologica fractrues is expected in Cherubism. T/F?
False- is NOT expected
Surgical recontrouing in Cherubism is postmponed until when?
Skeletal growth is complete
There may be some regression at puberty in Cherubism. T/F?
true