benign bone tumors and tumor-like conditions

Question 1

what is the origin of central ossifying fibroma

Answer 1

uncommon neoplasm that probably arises from periodontal ligament fibroblasts

Question 2

what has central ossifying fibroma been confused with?

Answer 2

focal cemento-osseous dysplasia

Question 3

where and who gets central ossifying fibroma

Answer 3

mandibular premolar/molar region, adult female, 3rd to 4th decade

Question 4

describe central ossifying fibroma

Answer 4

well-circumscribed radiolucency with variable amount of central opacity
asymptomatic swelling, root divergence, facial deformity with larger lesions
range from almost purely radiolucency to very radiodense with a well- defined lucent border

Question 5

characteristics of central ossifying fibroma

Answer 5

lesional tissue separates readily from host bone, often in one piece
cellular fibrous connective tissue with calcified trebeculae/spherules resembling cellular cementum or woven bone
similar histology to fibrous dysplasia/COD

Question 6

central ossifying fibroma treatment

Answer 6

enucleation

prognosis is very good, recurrence rate of 12 %

Question 7

what and where is osteoma?

Answer 7

benign osseous tumor, primarily affecting craniofacial bones (esp. paranasal sinuses)
painless slowly enlarging
in the jaws, predilection for mandibular body and condylar area

Question 8

what is the histology of osteoma?

Answer 8

normal bone +/- marrow tissue

Question 9

what is the genetics and prevalence of gardner syndrome?

Answer 9

AD- 1:8000-16000 live births

one of the the familial adenomatous polyposis syndromes; APC gene, chromosome 5

Question 10

how is gardner syndrome characterized?

Answer 10

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

Question 11

how does gardner syndrome look radiographically?

Answer 11

osteomas of gardner syndrome can resemble florid cemento osseous dysplasis or osteitis deformans
may see impacted sueprnumerary teeth or less commonly odontomas

Question 12

what significant event can happen with gardner syndrome?

Answer 12

the most significant aspect of the syndrome is the development of precancerous polyps of the colon
50% of patients develop adenocarcinoma of the colon by 30 years of age (approaches 100% by 5th decade)

Question 13

how is gardner syndrome managed?

Answer 13

prophylactic colectomy
removal of problematic cysts and osteomas
removal of impacted teeth/odontomas with prosthodontic work as needed
genetc counseling

Question 14

is central giant cell granuloma benign?

Answer 14

benign, somewhat controversial, lesion of the jaws
some pathologists feel this is a neoplasm while others consider it a reactive process
older designation: giant cell reparative granuloma

Question 15

what are the characteristics of central giant cell granuloma?

Answer 15

broad age range, 60% of pts.

Question 16

what does central giant cell granuloma look like histopathologically?

Answer 16

cellular granulation tissue with numerous benign multinucleated giant cells
RBC extravasation and hemosiderin deposits are common

Question 17

what is the treatment of central giant cell granuloma?

Answer 17

treatment generally consists of aggressive curettage, although alternative non-surgical therapies have been reported
recurrence rate of approximately 20%
prognosis is generally considered good

Question 18

what is hyperparathyroidism? and what is primary and secondary hyperthyroidism?

Answer 18

inappropriate secretion of parathormone
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 19

what does hyperthyroidism look like?

Answer 19

radiographically, loss of lamina dura and ground glass trebecular pattern
unilocular/multilocular radiolucencies may develop- “brown tumor”
diffuse jaw enlargment may develop with chronic renal failure: renal osteodystrophy

Question 20

what does hyperparathyroidism look like histopathologically?

Answer 20

brown tumors show vascular granulation tissue with numerous multinucleated giant cells and extravasated erythrocytes
microscopically identical to central giant cell granuloma

Question 21

what is renal osteodystrophy?

Answer 21

hyperplastic response of the bone in patients with poorly-controlled secondary hyperparathyroidism related to end-stage renal disease
prominent jaw enlargement may result

Question 22

what is the treatment of hyperparathyroidism?

Answer 22

if primary, remove the source of excess hormon secretion
if secondary, better control of serum calcium levels. prathyroidectomy may be needed if medical therapy fails. renal transplant is another alternative

Question 23

what are the characteristics of osteosarcoma

Answer 23

malignancy showing production of osteoid by tumor cells
most common primary bone malignancy
about 1000 new cases in the US annually, only 6-8 % of these affect the jaws
bimodal age distribution major peak: 2nd decade, lesser peak: after 6th decade

Question 24

how is osteosarcoma of long bones different from the jaw?

Answer 24

osteosarcoma of long bones; knee area or pevis; mean age:18 years
osteosarcoma of the jaws, maxilla=mandible, mean age= 33-39 years

Question 25

what does osteosarcoma look like?

Answer 25

initial complaint is often pain, followed by swelling, loose teeth or parethesia
mixed radiopaque/radiolucent lesion with ill defined borders
symmetrically widened PDL of teeth in the area may be seen
sun burst patter is uncommon in jaws

Question 26

what does osteosarcoma look like histopatholically?

Answer 26

infiltrating sheets of malignant spindle-shaped or angular lesional cells
direct production of osteoid or bone
mitotic activity can vary from field to field
osteoblastic, chondroblastic, and fibroblastic differentiation may be seen

Question 27

what is the treatment of osteosarcoma?

Answer 27

radical surgery, together with chemotherapy is some cases
overall survival- 60-70%
death is more often due to uncontrolled local disease than metastasis (which is lower overall in gnathic lesions compared to extragnathic)

Question 28

what are the characteristics of chondrosarcoma?

Answer 28

rare malignancy of cartilaginous differentiation (1/2 as common as osteosarcoma)
most are seen in adult males (4th to 6th decade) and affect femur, pelvis or ribs
only 0.1% of all head and neck malignancies
anterior maxilla, posterior mandible
may be present with pain, swelling or loose teeth

Question 29

how does chondrosarcoma look radiographically?

Answer 29

poorly defined radiolucency with variable amounts of radiopacity
larger lesions may appear multilocular
may see widened pdl of the teeth in the area of the tumor, similar to osteosarcoma

Question 30

how does chondrosarcoma look histopathologically?

Answer 30

characterized by invasive lobules of atypical cells showing cartilaginous differentiation
grades 1-3 recognized, most jaw lesions are lower grade (1 or 2)

Question 31

what is the treatment of chondrosarcoma?

Answer 31

radical surgery
prognosis depends on the location and histopathologic grade of the tumor
5 and 10 yr survival: 98% and 71% respectively
death is usually due to direct extension of tumor, involving vital structures

Question 32

what is metastic disease?

Answer 32

most common form of cancer to involve bone
jaws are occasionally affected
metastatic deposits from malignancies below the neck may affect the jaws through batson’s paravertebral plexus of veins which lack valves

Question 33

what are the characteristics of metastatic disease?

Answer 33

mean age: 43-52 years
no gender predilection
marked predilection for the posterior mandible
paresthesia, tooth mobility, swelling, hemorrhage, pathologic fracture, trismus
failure of tooth socket to heal; consider metastatic disease or lymphoma

Question 34

what does metastatic disease look like radiographically?

Answer 34

poorly defined radiolucency; less commonly, mixed radiolucency/radiopacity

Question 35

what does metastatic disease look like histopathologically?

Answer 35

lesions mimic the primary malignancy from which they arose
scattered clusters of lesional cells give a “seeded” effect
breast, lung, colon, thyroid, prostate, kidney, melanoma are among the more common primary malignancies
22% of jaw metastases represent the initial manifestations of the malignant process

Question 36

what is the treatment and prognosis of metastatic disease?

Answer 36

palliation, usually with radiation therapy, anti-resorptives

prognosis- very poor, most patients die within one year of the diagnosis