bone neoplasms/tumors

Question 1

A __________ is an uncommon neoplasm that probably arises from periodontal ligament fibroblasts

Answer 1

Central Ossifying Fibroma

Question 2

Central Ossifying Fibromas can often be confused with what benign condition?

Answer 2

focal cemento-osseous dysplasia

Question 3

Clinical characteristics of Central Ossifying Fibromas:

Answer 3

1) Mandibular premolar/molar region
2) often in adult female, 3rd to 4th decade
3) Asymptomatic swelling, root divergence, facial deformity with larger lesions

Question 4

Radiographic features of Central Ossifying Fibromas:

Answer 4

A) Well-circumscribed radiolucency with variable amount of central opacity

B) Range from almost purely radiolucency to very radiodense with a well-defined lucent border

Question 5

T/F: when surgically removing a central ossifying fibroma, the lesional tissue will separate readily from the underlying bone

Answer 5

True

Question 6

Histological characteristics of Central Ossifying Fibromas:

Answer 6

A) Cellular fibrous connective tissue with calcified trabeculae/spherules resembling cellular cementum or woven bone

B) Similar histology to fibrous dysplasia/COD, radiographic correlation is often helpful

Question 7

what is the treatment/prognosis/reoccurrence for central ossifying fibromas?

Answer 7

1) Treatment consists of enucleation
2) Prognosis is very good
3) recurrence rate of 12%

Question 8

what is an Osteoma? what bones does it usually effect?

Answer 8

Benign osseous tumor

primarily affecting craniofacial bones (esp. paranasal sinuses)

Question 9

Characteristics of Osteomas:

Answer 9

1) Painless, slowly enlarging
2) In the jaws, predilection for mandibular body and condylar area
3) Histology: normal bone +/- marrow tissue

Question 10

T/F: Gardners syndrome has an Autosomal Recessive inheritance pattern

Answer 10

FALSE

Autosomal Dominant

Question 11

Inheritance characteristics of Gardners Syndrome:

Answer 11

A) 1:8,000-16,000 live births

B) One of the familial adenomatous polyposis syndromes

C) APC gene, chromosome 5

Question 12

what are the clinical characteristics of Gardners syndrome?

Answer 12

1) Characterized by multiple osteomas of the facial bones (skull, paranasal sinuses, mandible)
2) epidermoid cysts and desmoid tumors

Question 13

Radiographic features of Gardners Syndrome:

Answer 13

A) osteomas of Gardner syndrome can resemble florid cemento-osseous dysplasia or osteitis deformans

B) May see impacted supernumerary teeth or, less commonly, odontomas

Question 14

The most significant aspect of Gardners syndrome is the development of __________________

Answer 14

precancerous polyps of the colon

Question 15

50% of patients with gardeners syndrome develop _____________ of the colon by 30 years of age

Answer 15

adenocarcinoma

Question 16

how is gardeners syndrome managed?

Answer 16

A) Prophylactic colectomy

B) Removal of problematic cysts and osteomas

C) Removal of impacted teeth/odontomas with prosthodontic work as needed

D) Genetic counseling

Question 17

what neoplasm is considered “controversial” due to the fact it may be either a neoplasm or simply a reactive process?

Answer 17

Central Giant Cell Granuloma

Question 18

clinical characteristics of Central Giant Cell Granulomas:

Answer 18

1) Broad age range, 60% of pts are under 30 yrs old
2) 2:1 female predilection
3) location: Mandible favored (70%), often anterior, frequently crossing the midline

Question 19

Small central giant cell granulomas are often ________, while larger lesions become ________ and _________

Answer 19

Unilocular when small; larger lesions become multilocular and expansile

Question 20

what are the histopathological characteristics of giant cell granulomas?

Answer 20

A) cellular granulation tissue with numerous benign multinucleated giant cells

B) RBC extravasation and hemosiderin deposits are common

Question 21

what is the treatment/prognosis/recurrence rate for Giant Cell Granulomas?

Answer 21

A) Treatment generally consists of aggressive curettage, although alternative non-surgical therapies have been reported

B) Recurrence rate of approximately 20%

C) Prognosis is generally considered good

Question 22

____________ is caused by the Inappropriate secretion of parathormone

Answer 22

Hyperparathyroidism

Question 23

Define primary and secondary Hyperparathyroidism:

Answer 23

Primary - due to parathyroid hyperplasia, parathyroid adenoma, parathyroid carcinoma

Secondary - due to renal failure, which is responsible for poor calcium retention and altered vitamin D metabolism

Question 24

what are the radiographic characteristics of Hyperparathyroidism lesions?

Answer 24

1) Radiographically, loss of lamina dura and “ground-glass” trabecular pattern
2) Unilocular/multilocular radiolucencies may develop - “brown tumor”

Question 25

T/F: diffuse jaw enlargement can be caused by chronic renal failure

Answer 25

true secondary hyperparathyroidism (jaw enlargement) caused by renal failure

Question 26

what are the histopathological characteristics of lesions caused by Hyperparathyroidism?

Answer 26

1) Microscopically identical to central giant cell granuloma | 2) brown tumors show vascular granulation tissue with numerous multinucleated giant cells and extravasated erythrocytes

Question 27

what is the definition of Renal Osteodystrophy?

Answer 27

Hyperplastic response of the bone in patients with poorly-controlled secondary hyperparathyroidism related to end-stage renal disease

Question 28

what physical effects can result from Renal Osteodystrophy?

Answer 28

Prominent jaw enlargement may result

Question 29

what are the treatments for hyperparathyroidism?

Answer 29

If primary, remove the source of excess hormone secretion If secondary, better control of serum calcium levels. Parathyroidectomy may be needed if medical therapy fails. Renal transplant is another alternative

Question 30

A ___________ is a malignancy showing production of osteoid by tumor cells

Answer 30

Osteosarcoma

Question 31

T/F: Osteosarcomas are the most common primary bone malignancy

Answer 31

true

Question 32

how many cases of osteosarcoma are found each year? what population groups are at the highest risk?

Answer 32

A) About 1,000 new cases in the US annually; only 6-8% of these affect the jaws B) Bi-modal age distribution, major peak: 2nd decade, lesser peak: after 6th decade

Question 33

T/F: Osteosarcoma of the jaws is different epidemiologically compared to osteosarcoma of long bones

Answer 33

True

Question 34

what age groups are likely to acquire osteosarcoma of long bones? how about lesions of the jaws/maxilla?

Answer 34

1) Osteosarcoma of long bones; knee area or pelvis; mean age: 18 years 2) Osteosarcoma of the jaws; maxilla = mandible; mean age: 33-39 years

Question 35

clinical characteristics of Osteosarcomas:

Answer 35

Initial complaint is often pain, followed by swelling, loose teeth or paresthesia

Question 36

radiographic signs of osteosarcoma:

Answer 36

1) Mixed radiopaque/radiolucent lesion with ill-defined borders 2) Symmetrically widened PDL of teeth in the area may be seen 3) “Sun-burst” pattern is uncommon in jaws

Question 37

what are the histological characteristics of Osteosarcomas?

Answer 37

A) Infiltrating sheets of malignant spindle-shaped or angular lesional cells B) Direct production of osteoid or bone C) Osteoblastic, chondroblastic and fibroblastic differentiation may be seen

Question 38

what is the treatment/prognosis/survival for Osteosarcomas?

Answer 38

1) Treatment is radical surgery, together with chemotherapy is some cases 2) Overall 5 yr survival: 60-70% 3) Death is more often due to uncontrolled local disease than metastasis

Question 39

A _____________ is a rare malignancy of cartilaginous differentiation

Answer 39

Chondrosarcoma

Question 40

T/F: chondrosarcomas are only half as common as osteosarcomas

Answer 40

true

Question 41

what groups are at the highest risk for developing chondrosarcomas? what sites are most likely to be effected?

Answer 41

A) Most are seen in adult males (4th to 6th decade) B) affect femur, pelvis or ribs

Question 42

Chondrosarcomas account for only ____% of all head and neck malignancies

Answer 42

0.1%

Question 43

where in the head/neck region are chondrosarcomas most likely to be found? what are the symptoms associated with them?

Answer 43

A) found in Anterior maxilla, Posterior mandible B) May present with pain, swelling or loose teeth

Question 44

what are the radiographic finding for chondrosarcomas?

Answer 44

1) Poorly defined radiolucency with variable amounts of radiopacity 2) Larger lesions may appear multilocular 3) May see widened PDL of the teeth in the area of the tumor, similar to osteosarcoma

Question 45

list the histopathological characteristics of Chondrosarcomas:

Answer 45

invasive lobules of atypical cells showing cartilaginous differentiation

Question 46

T/F: most chondrosarcomas of the jaw are high grade (grade 3)

Answer 46

FALSE most are low grade (1 or 2)

Question 47

Treatment/prognosis/recurrence of chondrosarcomas: what is the 5 & 10 year survival rate for chondrosarcomas?

Answer 47

A) Treatment is radical surgery B) Prognosis depends on the location and histopathologic grade of the tumor C) 5 and 10 yr survival: 87% and 71% respectively

Question 48

T/F: Death due to chondrosarcomas are usually due to direct extension of tumor, involving vital structures

Answer 48

true

Question 49

T/F: death due to osteosarcomas are due to uncontrolled disease, while death due to chondrosarcoma is due to direct effects of the tumor

Answer 49

true

Question 50

__________ is the most common form of cancer involving bone

Answer 50

Metastatic Disease

Question 51

Metastatic deposits from malignancies below the neck may affect the jaws through ____________________ veins which lack valves

Answer 51

Batson’s paravertebral plexus of veins

Question 52

what groups are at the highest risk for metastatic disease? what areas does it usually effect in the head/neck?

Answer 52

Mean age: 43 - 52 years No gender predilection Marked predilection for the posterior mandible

Question 53

clinical characteristics of metastatic disease in the head/neck region:

Answer 53

A) Paresthesia, tooth mobility, swelling, hemorrhage, pathologic fracture, trismus B) Failure of tooth socket to heal: consider metastatic disease or lymphoma

Question 54

what does metastatic disease look like radiographically

Answer 54

Poorly defined radiolucency less commonly, mixed radioluceny/radiopacity

Question 55

histopath features of metastatic disease:

Answer 55

A) lesions mimic the primary malignancy from which they arose B) Scattered clusters of lesional cells give a “seeded” effect C) Breast, lung, colon, thyroid, prostate, kidney, melanoma are among the more common primary malignancies

Question 56

___% of jaw metastases represent the initial manifestation of Metastatic disease

Answer 56

22%

Question 57

what is the treatment/prognosis for metastatic disease?

Answer 57

Treatment: palliation, usually with radiation therapy, anti-resorptives Prognosis: very poor; most patients die within one year of the diagnosis