Bone Neoplasms Flashcards

1
Q

an uncommon neoplasm that probably arises from periodontal ligament fibroblasts.

A

central ossifying fibroma

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2
Q

could be confused with focal cemento-osseous dysplasia in the past (also osteoporotic bone marrow defect, fibrous dysplasia)

A

central ossifying fibroma

*note: cemento-osseous dysplasia is more apical

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3
Q

What region of the mouth is most affected by central ossifying fibroma? Age and gender?

A
  • mandibular premolar/molar

- adult female 3rd-4th decade

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4
Q

What are the signs and symptoms of central ossifying fibroma?

A
  • well-circumscribed radiolucency with variable central opacity
  • asymptomatic swelling, root divergence, facial deformity with larger lesions
  • can range in lucency/opacity
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5
Q

Describe the histology of central ossifying fibroma

A
  • lesional tissue separates readily from host bone, often in one piece
  • cellular fibrous CT with calcified trabeculae/spherules (resembles cellular cementum or woven bone)
  • similar histology to fibrous dysplasia or cemento-osseous dysplasia so radiographic correlation is helpful
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6
Q

What is the treatment of central ossifying fibroma? Prognosis?

A
  • treatment: enucleation

- prognosis: very good; recurrence is 12%

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7
Q

What is a benign osseous tumor, primarily affecting the craniofacial bones (especially paranasal sinuses)?

A

osteoma

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8
Q

Tori probably represent the majority of ___.

A

osteomas

*note: tori are usually static and develop but don’t enlarge afterward whereas osteomas keep enlarging

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9
Q

What are the signs and symptoms of osteoma? Where is it most frequently located?

A
  • painless slow enlarging bone mass

- most often in mandibular body and condylar area

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10
Q

Describe the histology of an osteoma.

A

normal bone +/- marrow tissue

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11
Q

What other condition is associated with osteoma?

A

Gardner Syndrome

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12
Q

Describe how Gardner syndrome is acquired. How often? What family of disease?

A
  • autosomal dominant; APC gene, chromosome 5
  • 1 in 8,000-16,000
  • familial adenomatous polyposis syndromes
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13
Q

What are the characteristics of Gardner Syndrome?

A
  • mulitple osteomas of the facial bones (skull, paranasal sinuses, mandible)
  • epidermoid cysts
  • desmoid tumors
    ALSO:
  • impacted supernumerary teeth, or less commonly, odontomas
  • development of precancerous polyps of the colon

*note: presence of osteomas helps to distinguish Gardner from cleidocranial dysplasia

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14
Q

What can Gardner’s syndrome radiographically resemble?

A

florid cemento-osseous dysplasia or osteitis deformans

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15
Q

__% of Gardner syndrome patients develop adenocarcinoma of the colon by age 30; __% by the 5th decade

A

50%

100%

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16
Q

Describe the management of Gardner syndrome.

A
  • prophylactic colectomy
  • removal of problematic cysts and osteomas
  • removal of impacted teeth/odontomas with prosthodontic work as needed
  • genetic counseling
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17
Q

What condition does some think is a neoplasm while others think it is a reactive lesion?

A

central giant cell granuloma

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18
Q

What age is most susceptible to a central giant cell granuloma? Gender? Area of oral cavity?

A
  • broad age range (60% of patients
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19
Q

True or false: The teeth in a central giant cell granuloma are non-vital.

A

FALSE. Teeth are vital

*note: these are soetimes treated endodontically mistakenly

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20
Q

How do central giant cell granulomas appear radiographically? Histologically?

A
  • appear unilocular when small –> multilocular and expansile when larger
  • cellular granulation tissue with numerous benign multi-nucleated giant cells; RBC extravasation and hemosiderin deposits
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21
Q

What does a brown tumor of hyperparathyroidism resemble histologically?

A

central giant cell granuloma

22
Q

What is the treatment of central giant cell granuloma? Prognosis?

A
  • aggressive curettage, or alternate non-surgical therapies

- prognosis good, recur 20%

23
Q

What is the difference between primary and secondary hyperparathyroidism?

A
  • 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
24
Q

What are the signs and symptoms of hyperparathyroidism?

A
  • radiographic loss of lamina dura and “ground glass” trabeculae pattern
  • “brown tumor”: unilocular/multilocular radiolucencies may develop
  • renal osteodystrompy (diffuse jaw enlargement with chronic renal failure)
25
Q

Describe the histology of hyperparathyroidism.

A
  • brown tumors show vascular granulation tissue with numerous multinucleated giant calls and extravasated erythrocytes
  • IDENTICAL TO CENTRAL GIANT CELL GRANULOMA
26
Q

What is renal osteodystrophy and what condition is it related to?

A
  • hyperplastic response of the bone in patients with poorly-controlled secondary hyperparathyroidism related to end-stage renal disease; prominent jaw enlargement may result
  • note: distinguish from Paget’s disease with alkaline phosphatase test (high AP in Paget disease)
27
Q

What is the treatment for hyperparathyroidism?

A
  • PRIMARY: remove source of excess hormone

- SECONDARY: better control of serum calcium levels, parathyroidectomy, or renal transplant

28
Q

What is the prognosis of hyperparathyroidism?

A

fair to good (no risk of osteosarcoma like Pagets does)

29
Q

What is the most common primary bone malignancy?

A

ostosarcoma

30
Q

How common is osteosarcoma? What age?

A
  • 1,000 new cases in the USA annually; only 6-8% affect the jaws
  • bi-modal age distribution = major peak at 2nd decade and lesser peak after 6th decade
31
Q

How does osteosarcoma of the jaw differ from that of long bones?

A
  • LONG BONES: knee area or pelvis; mean age 18 years

- JAWS: maxilla = mandible; mean age 33-39 years

32
Q

What are the signs and symptoms of osteosarcoma?

A
  • initial complaint of pain, followed by swelling, loose teeth, or paresthesia
  • mixed radiopaque/radiolucent will ill-defined borders
  • symmetrically widened PDL
  • “sunburst” pattern is uncommon
  • note: wide PDL usually only seen in malignancies; PDL is entry for sarcoma
  • note: mottled expansion = bone forming malignancy
  • note: differential diagnoses = peripheral ossifying granuloma and focal cemento-osseous dysplasia
33
Q

Describe the histology of an osteosarcoma.

A
  • infiltrating sheets of malignant spindle-shaped or angular lesional cells
  • direct production of osteoid/bone
  • mitotic activity can vary field to field
  • osteoblastic, chondroblastic, and fibroblastic differentiation

*note: 3 IHC markers help diagnose

34
Q

What is the treament and prognosis of osteosarcoma?

A
  • TREATMENT: radical surgery with chemo (in some cases)
  • PROGNOSIS: 5-year survival 60-70%, death is more often due to uncontrolled local disease than metastasis (which is lower overall in gnathic vs extragnathic lesions)
35
Q

What is the condition that is a rare malignancy of cartilaginous differentiation?

A

chondrosarcoma

36
Q

Which is more common: osteosarcoma or chondrosarcoma?

A

osteosarcoma (2x more common)

37
Q

What age is most susceptible to chondrosarcoma? Gender? Area of the body? Area of the oral cavity?

A
  • adults 4th to 6th decade
  • males
  • femur, pelvis, ribs
  • posterior mandible, anterior maxilla

*note: only 0.1% of all head and neck malignancies

38
Q

What are the signs and symptoms of chondrosarcoma?

A
  • pain, swelling, or loose teeth
  • poorly defined radiolucency with variable amounts of radiopacity
  • larger lesions may appear multilocular
  • widened PDL of teeth in area of tumor (like osteosarcoma)
39
Q

Describe the histology of chondrosarcoma.

A

characterized by invasive lobules of atypical cells showing cartilaginous differentiation

40
Q

What grades of chondrosarcoma are recognized? What are most jaw lesions?

A
  • grades I-III recognized

- lower grade (I or II)

41
Q

True or false: Chondrosarcomas have a very rapid proliferation and leave areas of necrosis due to blood vessels not being able to keep up.

A

true

42
Q

True or false: Chondrosarcomas do not spread via metastasis.

A

FALSE; metastasizes via blood and can spread from the jaws to the vertebral column or elsewhere

43
Q

What is the treatment for chondrosarcoma? Prognosis?

A
  • TREATMENT: radical surgery
  • PROGNOSIS: depends on the location and histopathologic grade of the tumor
  • 5 year survival = 87%; 10-year survival = 71%
  • death is usually due to direct extension of the tumor, involving vital structures
44
Q

What is the most common form of cancer to involve the bone?

A

metastatic disease

45
Q

In metastasis, most carcinomas travel via ___ while most sarcomas travel via ___.

A
  • lymphatics
  • hematogenously

*note: not a hard and fast rule

46
Q

How do metastatic deposits from malignancies below the neck affect the jaws?

A

Batson’s paravertebral plexus of veins which lack valves

47
Q

What age is most susceptible to metastatic disease? Gender? Area of the oral cavity?

A
  • mean age 43-52 years
  • no gender predilection
  • posterior mandible (because that is where there is hematopoietic marrow so it can catch the cells)
48
Q

What are the signs and symptoms of metastatic disease?

A
  • paresthesia
  • tooth mobility
  • swelling
  • hemorrhage
  • pathologic fracture
  • trismus
  • failure of tooth socket to heal
  • poorly defined radiolucency (less commonly, mixed radiolucency/radiopacity)
49
Q

Describe the histology of metastatic disease.

A

lesions mimic the primary malignancy from which they arose

scattered clusters of lesional cells gives “seeded” effect or small deposits of cells in many different places

50
Q

What are the most common primary malignancies that cause metastatic disease to the jaws?

A
  • breast, lung, colon, thyroid, prostate, kidney, melanoma
51
Q

__% of jaw metastases represent the initial manifestation of the malignant process

A

22%

52
Q

What is the treatment for metastatic disease? Prognosis?

A
  • palliation, usually with radiation therapy, anti-resorptives
  • very poor prognosis, most patients die within 1 year