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Oral Pathology Exam 3 > 1. Bone Disease & Neoplasms > Flashcards

1. Bone Disease & Neoplasms Flashcards

1
Q

What is the other name for Brittle Bone Disease?

A

Osteogenesis Imperfecta

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2
Q

What are the etiologies of Osteogenesis Imperfecta?

A

Group of Disorders of Type I Collagen Maturation

  • > 90% Autosomal Dominant
    • COL1A1, COL1A2 mutation
  • Autosomal Recessive
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3
Q

What are the characteristics of the Autosomal Dominant form of Osterogenesis Imperfecta? (3)

A
  • More common
  • Compatible with survival
  • History of varying degrees of bony fractures and deformity
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4
Q

What are the characteristics of the Autosomal Recessive form of Osteogenesis Imperfecta? (2)

A

Infantile - Malignant Osteopetrosis

  • Severe form
  • Results in still birth or severe deformity
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5
Q

What are the Clinical Characteristics of Osteogenesis Imperfecta? (7)

A
  • Weakened bones fracture easily and don’t heal well leading to excessive callus formation
  • Mistaken for Child Abuse
  • Long bone/spine deformities, bowing, fractures
  • Blue Sclera
  • Other Features
    • _​_Hearling loss = Hypoacusis
    • Hyper-extensible joints
    • Wormian Skull Bones
      • Like Cleidocranial Dysplasia
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6
Q

What are the oral findings in Osteogenesis Imperfecta? (3)

A
  • Opalescent Teeth
    • Blue-brown translucence of teeth
    • Pulpal obliteration or “shell teeth”
    • Clinically and radiographically identical to Dentinogeneisis Imperfecta but due to a different mutation
    • Crowns tend to be bulbous
  • Class III Malocclusion
    • ​Due to hypoplastic maxilla
    • Also in Crouzon Sx
  • Mixed (RL-RO) Jaw Lesions
    • Rarely occur
    • Look like Cemento-Osseous Dysplasia
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7
Q

What is the Histopathology of Osteogenesis Imperfecta? (2)

A
  • Failure of woven bone to mature into lamellar bone
    • Resulting in immature bone throughout life
    • Never gains full strength, leads to fractures
  • Dentin Abnormalities
    • ​Similar to Dentinogenesis Imperfecta
      • Dentin is made of collagen, so it doesn’t form well, specially the dentin tubules
      • Calcifications within the tubules with amorphous qualities
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8
Q

What is the treatment of Osteogenesis Imperfecta?

A
  • No cure, symptom management
    • Surgery for fractures/physiotherapy
    • Bisphosphonates used in moderate to severe cases
  • Dental Considerations
    • Severe attrition of teeth and tooth loss
      • Managed like DI with FPD
      • Implants used with caution
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9
Q

What are the other names for Marble Bone Disease?

A
  • Osteopetrosis
  • Albers-Schonberg Disease
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10
Q

What is the pathogenesis of Osteopetrosis?

A
  • Osteoclasts lack the ability to remodel bone (breakdown/resorb the bone being laid down) causing the obliteration of marrow space
  • Bone deposition without resorption
    • Thickening of cortical bone and sclerosis of cancellous bone
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11
Q

What is the other name for Infantile Osteopetrosis?

A

Malignant Osteopetrosis

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12
Q

What is the etiology of Infantile Osteopetrosis?

A

Autosomal Recessive

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13
Q

What are the clinical features of Infantile Osteopetrosis? (4)

A
  • Marrow Failure
    • Liver and spleen take over, and make bone marrow cells, causing them to both enlarge
    • Myelophthisic Anemia resulting in Extramedullary Hematopoiesis with Hepatosplenomegaly
  • Constriction of Nerve Ostea
    • Deafness + blindness (=​Paget’s Disease)
  • Delayed Tooth Eruption
    • _​_Need good bone remodeling for movement of teeth, they can’t erupt through sclerotic bone
  • Increased Bone Density
    • Tubular bones become solid, brittle and fracture - can’t handle “shear stress”
    • No distinction between cortical and cancellous bone = solid white bone
      • Obscured tooth roots
      • No PDL space ​
    • Dense bones are easily infected due to granulocytopenia** causing **osteomyelitis
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14
Q

What is the treatment for Infantile Osteopetrosis? (4)

A
  • Bone Marrow Transplant
  • IFN gamma-1b with:
    • Calcitriol (Vitamin D)
    • Restricted calcium intake
  • Steroids (not preferred tx)​
  • Aggressive tx of Osteomyelitis (debridement, bacterial C/S with IV antibiotics)
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15
Q

What is the etiology of Intermediate Osteopetrosis?

A

Autosomal Recessive

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16
Q

What are the clinical features of Intermediate Osteopetrosis? (3)

A
  • Asymptomatic @ birth
  • Fractures by 10 yrs
  • Anemia and Extramedullary Hematopoiesis occur but bone marrow failure is rare
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17
Q

What is the etiology of Adult Osteopetrosis?

A

Autosomal Dominant

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18
Q

What is the most common type of Osteopetrosis?

A

Adult Osteopetrosis

Has less severe manifestations

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19
Q

What are the features of Adult Osteopetrosis? (4)

A
  • Axial skeleton shows sclerosis, but long bones exhibit little defect
  • Bone pain often reported BUT 40% are asymptomatic
  • Variable presence of fracture and/or nerve compression
  • Need to rule out other causes of widespread sclerosis of jaws (DD)
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20
Q

What is the other name for Paget’s Disease?

A

Osteitis Deformans

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21
Q

What is the etiology/pathogenesis of Paget’s Disease?

A
  • Unknown etiology
  • Increased or uncontrolled bone remodeling
    • Net acumulation of bone
    • Bones get bigger but not stronger
    • Bones don’t mature - results in distortion and weakening of bone
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22
Q

In what population is Paget’s Disease prominent?

A
  • Older adults of Anglo-Saxon ancestry
    • Rare < 40 yrs
  • 2:1 Male predilection
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23
Q

When Paget’s Disease affects the jaws, which does it prefer?

A
  • Maxillary (2:1)
  • Jaws affected in 17% of cases
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24
Q

What classifies most cases (85%) of Paget’s Disease?

A

Polyostotic = affecting multiple bones

  • Most commonly
    • Pelvis, Femur, Tibia, Lumbar vertebrae, Skull
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25
Q

What are the Clinical Features of Paget’s Disease? (6)

A
  • Dull, non-specific pain
  • Symmetric Enlargement
    • Increase in hat size
    • “Maxillary denture won’t fit”
  • Simian stance - bowing of legs
  • Spinal compression fractures
  • Deafness and blindness due to narrowing of nerve ostea
    • Also in Infantile Osteopetrosis
  • Extensive Hypercementosis of teeth
    • Entire arch not just an isolated tooth or quadrant.
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26
Q

What is the clinical feature of early lesions of Paget’s Disease?

A

Decreased RO with coarse trabeculae

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27
Q

What is the clinical feature of late lesions of Paget’s Disease?

A
  • Patchy sclerosis “Cotton-wool” appearance with thickened cortices
    • Dense RO next to RL areas
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28
Q

What are the laboratory features of Paget’s Disease?

A
  • Markedly elevated total serum Alkaline Phosphatase (non-specific)
    • Marker of osteoblastic activity (bone turnover)
  • Use serum bone-specific alkaline phosphatase if pt has Liver Disease
  • Calcium and Phosphate levels are usually normal
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29
Q

What is the histology of the Osteolytic phase of Paget’s Disease?

A
  • Initial resorption with numerous enlarged osteoclasts (multinucleated) surrounding irregular trabeculae
    • Bone looks more RL
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30
Q

What is the histology of the Osteoblastic phase of Paget’s Disease?

A
  • Increased osteoblastic activity, then occurs the likely “Cotton-wool” appearance, lacking lamellar pattern
    • Entire skull is affected, not just an isolated area like in Osteoblastoma
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31
Q

What is the histology of the Osteosclerotic (burned out) phase of Paget’s Disease?

A
  • Marrow space replaced by vascular fibrous CT
    • In OP it is replaced by dense cortical bone
  • Dense RO bone masses with prominent reversal lines create a “mosaic bone”
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32
Q

What is the treatment for Paget’s Disease? (3)

A
  • NSAIDs for mild pain
  • Bisphosphonates (oral 2-6 months) or IV forms as a single dose to limit amount of resorption
  • Pts should be monitored for the development of Giant Cell Tumor of Bone as well as malignant bone tumors, especially Osteosarcoma (1%)
    • ​Very rare to get osteosarcoma > 40 yrs old, if so it is probs Paget’s Disease
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33
Q

What are the Dental Considerations for Paget’s Disease? (5)

A
  • Pt may report “black triangle” or diastemas
  • Difficult extractions due to hypercementosis and ankylosis
  • Place implants with caution
  • Surgical bleeding risk duing the osteolytic phase, because it is more vascular.
  • Poor wound healing with risk for Osteomylelitis during osteosclerotic phase.
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34
Q

With an Osteoma, what involvement is common?

A

Paranasal sinus - frontal most common

bone growth inside sinuses

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35
Q

What location are the jaw lesions of Osteoma’s associated with?

A
  • Condylar Area
  • Lingual Posterior Mandible - near premolars and molars
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36
Q

What needs to be ruled out with Osteomas of the jaw?

A

Gardner Syndrome

due to colon polyps that turn into cancer

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37
Q

What is the etiology of Gardner Syndrome?

A

Autosomal Dominant

  • Mutation in the APC Tumor Suppressor Gene
    • Makes colon polyps when TSG is knocked out
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38
Q

What is Gardner Syndrome characterized by?

A

Osteomas of Facial Bones

looks like giant bulges on pts faces

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39
Q

If a pt has Osteomas of the facial bones, what else should we look for?

A

Impacted Supernumerary Teeth or less commonly, Odontomas

Gardner Syndrome

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40
Q

What are the skin lesions of Gardner Syndrome?

A
  • Epidermoid Cysts
  • Desmoid Tumors
    • Locally aggressive fibrous neoplasms of soft tissue, like a Fibromatosis
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41
Q

What is the most significant aspect of Garnder Syndrome?

A

Development of precancerous polyps of the colon

  • 50% develop colon cancer by age 30
  • 100% affected later in life
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42
Q

What do pts with Gardner Syndrome have an increased risk for?

A
  • Thyroid Carcinoma
    • Often gets overlooked
  • Adenocarcinoma of the Colon
    • 100% affected later in life
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43
Q

What is the Treatment for Gardner Syndrome?

A
  • Prophylactic Colectomy
  • Removal of cosmetically problematic cysts and osteomas
  • Genetic Counseling
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44
Q

What is the Histology of Osteoid Osteoma and Osteoblastoma? (4)

A
  • Identical to each other and Cementoblastoma
  • Mineralized material with prominent reversal lines
    • So does Paget’s Disease in the osteosclerotic phase
  • Large sheets of irregular trabeculae of bone with multi-nucleated osteoclast-like giant cells and numerous osteoblasts
  • Loose fibrous CT with scattered dilated vascular spaces
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45
Q

What is the treatment for Osteoid Osteoma and Osteoblastoma?

A

Excision or curettage

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46
Q

What is the prognosis for Osteoid Osteoma and Osteoblastoma? (4)

A
  • Good, with low recurrence rate
  • Aggressive variants of Osteoblastoma in older pts = higher recurrence rates
  • Rare transformation of Osteoblastoma to Osteosarcoma
  • Osteoid Osteoma has no potential for malignant change
47
Q

Which occurs in younger pts, Osteoid Osteoma or Osteoblastoma?

A

Osteoblastoma (<30 yrs)

Osteoid Osteoma (avg 25)

48
Q

What is the size of Osteoid Osteomas?

A

< 2cm small

49
Q

What is the size of Osteoblastomas?

A

large

2-4cm –> 10cm

50
Q

What is associated with nocturnal pain, relieved by salicylates, becuase tumor makes prostaglandins?

A

Osteoid Osteoma

51
Q

What is the radiographic appearance of Osteoblastoma? (3)

A
  • Expansile, pushing teeth apart
  • Cotton-wool appearance
    • Don’t confuse with Paget’s which will be diffuse and is rare in pts < 40 yrs
  • Reactive sclerosis not always present
52
Q

What is the radiographic appearance of Osteoid Osteoma? (3)

A
  • Non-expansile
  • Well-circumscribed, RL outer rim, with variably thick reactive sclerosis in center
  • Central RO nidus (target-like)
    • COD, but it isn’t painful
53
Q

What is the most common primary bone malignancy?

A

Osteosarcoma

2x as common as Chondrosarcoma

54
Q

What is the pathogenesis of Osteosarcoma?

A
  • Production of osteoid by malignant mesenchymal cells
    • Must have: malignant cells + osteoid
55
Q

What is the skeletal presentation of Osteosarcoma?

A
  • Fast growing mass around knees in children and young adults (mean = 18 yrs)
56
Q

What is the classic radiographic appearance of Osteosarcoma in the long bones?

A

Sun-burst opacities

57
Q

What leads to Osteosarcoma of the long bones, in older pts?

A
  • Paget’s Disease
  • Radiated Bone
58
Q

What is the most common bone sarcoma to happen in bone that has been irradiated?

A

Osteosarcoma

59
Q

What is the mean age for pts to develop Osteosarcoma of the jaws?

A

33 yrs

Skeletal is younger @ 18 yrs

60
Q

What is typically the initial complaint in pts with Osteosarcoma of the jaws?

A
  • PAIN, followed by:
    • Swelling
    • Loose teeth due to PDL attachment loss
    • Paresthesia
61
Q

What is the radiographic appearance of Osteosarcoma of the Jaws? (3)

A
  • Symmetrically widened PDL
    • ​Tumor grows around the PDL and destroys it, leads to loose teeth
  • Malignant bone growth above the crestal ridge, wispy, ill-defined
  • Fuzzy bone - mixed lesion with ill-defined borders
62
Q

What is the presentation of Parosteal Osteosarcoma? (2)

A
  • Firm, bony, mushroom shaped growth, off the side of the jaw (red/purple)
    • DD: Kaposi sarcoma, lymphomas, leukemia
  • Obliterates periosteum
63
Q

What is the presentation of Periosteal Osteosarcoma? (3)

A
  • Elevates periosteum around it
  • Forms spicules of bone radiating out - classic
  • Onion skinning, layering of the periosteum
64
Q

What is the Histology of Osteosarcoma? (3)

A
  • Infiltrating sheets of malignant spindle-shaped or angular lesional cells
  • Direct production of osteoid or bone required by malignant cells
  • 3 Subtypes
    1. Osteoblastic
    2. Chondroblastic
      • Most common
    3. FIbroblastic
65
Q

What is the treatment for Osteosarcoma of the Jaws?

A
  • Wide surgical excision with initial complete removal ​
    • ​Most important prognostic factor
  • Chemo for jaw lesions has no added benefits
66
Q

What is the prognosis for Osteosarcoma?

A
  • 60-70% 5yr survival
  • Death is usually due to uncontrolled local ds
67
Q

What is the treatment for Osteosarcoma of the long bones?

A
  1. Neoadjuvant chemo
    • Trying to see how much necrosis will occur​
  2. Surgery
  3. Adjuvant Chemo
    • If they saw alot of necrosis with the neoadjuvant chemo
68
Q

Where does Osteosarcoma metastasize to?

A

Lung and Brain

69
Q

Which lesions of Osteosarcoma metastasize more frequently?

A

Long bone lesions

70
Q

Where is it common for a Chondroma to occur?

A

Small bones of the hands + feet

Rare in Jaws, any mature cartilaginous jaw tumor is potentially a well differentiated chondrosarcoma

71
Q

What are the 2 instances where you can have mature cartilaginous metaplasia (above the crest of the ridge), anything else is malignant = Chondrosarcoma?

A
  1. Periosteum irritated by an ill-fitting denture = Osseous and Chrondromatous Metaplasia
    • ​​Periosteum is a fibrous tissue, that here is differentiating into bone and cartilage
  2. Normal development, normal pieces of cartilage can be left over = Cartilaginous Rests
    • In the anterior maxilla, mandibular symphysis, coronoid process + condyle
72
Q

What population is typically affected by Chondrosarcoma?

A

Adult Males, ~ 51 yrs

Osteosarcoma ~30s

73
Q

Where do you typically find Chondrosarcoma?

A
  • Pelvis, Femur, Humerus, and Ribs
  • Rare in Jaws
    • < 10% occur in H/N region
74
Q

What are the symptoms of Chondrosarcoma?

A
  • Swelling, loose teeth
  • No pain associated
    • Differs from Osteosarcoma
75
Q

How do you radiographically distinguish Osteosarcoma from Chondrosarcoma?

A

Check the PA for PDL Widening = Osteosarcoma

76
Q

How does Chondrosarcoma act, compared to Osteosarcoma?

A
  • Slow growing, but very invasive
  • Rarely mets
  • Osteosarcoma is faster growing and can mets to brain and lungs
77
Q

What is the histology of a Chondrosarcoma?

A
  • Invasive lobules of atypical cartilaginous differentiated cells
  • NO BONE, only cartialge
    • If you see bone it is Chondroblastic Osteosarcoma
78
Q

What is the treatment for Chondrosarcoma?

A

Radical surgery with wide margins

79
Q

What is the prognosis for Chondrosarcoma? (3)

A
  • 75% overall 5 yr survival, depends on:
    • Location, size, and grade (differentiation)
  • Death is usually due to direct extension of tumor, involving vital structures
    • Same is true for Osteosarcoma
  • Difficult to remove, with frequent recurrences up to 20 yrs later
    • Follow For Life!
80
Q

At what size do chondrosarcomas have a more aggressive prognosis?

81
Q

What location of a Chondrosarcoma has a better prognosis?

A

Appendicular is better than axial

82
Q

What is the prognosis of a Grade 1 Chondrosarcoma?

A

90% 5yr survival

No mets

83
Q

What is the prognosis of a Grade 2 Chondrosarcoma?

A

81% 5 yr survival

84
Q

What is the prognosis of a Grade 3 Chondrosarcoma?

A

Prognosis drops significantly

  • 29% 5yr survival
  • Mets: lung, lymph nodes
85
Q

What is the common location for Mesenchymal Chondrosarcoma?

A

Jaws (25%)

  • Only cartilage tumor that favors the jaws
  • May develop in bone or soft tissue
86
Q

What age group is most commonly affected by Mesenchymal Chondrosarcoma?

A

2nd - 3rd decade (early 20’s)

Chondrosarcoma presents in an older population, males ~51 y.o.

87
Q

How does Mesenchymal Chondrosarcoma present?

A
  • Fast growing + aggressive
  • ​ Pain + swelling (most common symptoms)
    • Osteosarcoma is fast growing, with initial symptom of pain
    • Chondrosarcoma has no assoc pain, and grows slowly, but it is invasive
88
Q

What is the radiographic appearance of a Mesenchymal Chondrosarcoma?

A

Mixed, but mostly RL = ill-defined RL with or without stippled calcifications

Chondrosarcoma will have focal opacities

89
Q

What is the Histology of Mesenchymal Chondrosarcoma?

A
  • Highly cellular proliferation of undifferentiated small cells, alternating with zones of differentiated cartilaginous tissue
  • Cartilage cells + mesenchymal cells
90
Q

What is the treatment for Mesenchymal Chondrosarcoma?

A
  • Radical surgery + possible radiation and chemo
  • It is a more aggressive lesion
91
Q

What is the prognosis for Mesenchymal Chondrosarcoma?

A
  • Poor
    • 5 yr survival ~50%
    • 10 yr survival ~ 30%
  • Long term follow-up needed as local recurrence or mets (often lungs) may occur up to 20 yrs later
    • Just like Chondrosarcoma
92
Q

What is the pathogenesis of Ewing Sarcoma?

A
  • Tumor of undifferentiated neuroectodermal cells
  • 90% due to EWSR1-FLI1 translocation
    • ​Detect translocation in a tumor with FISH, helps differentiate from other tumors with a histo of sheets of small, dark cells (Mesenchymal Chondrosarcoma, ect.)
93
Q

What is the population most commonly affected by Ewing Sarcoma?

A
  • White (95%)
  • Males
  • 10-15 yrs
94
Q

What are the usual symptoms of Ewing Sarcoma?

A

Pain and Swelling

also in Osteosarcoma and Mesenchymal Chondrosarcoma, and Metastatic Disease

95
Q

Where are Ewing Sarcomas located?

A
  • 60% in bone of lower extremities and pelvis
    • ​Worst prognosis
  • < 1-2% affect the jaws
    • Better prognosis
96
Q

What is the radiographic appearance of Ewing Sarcoma?

A
  • Patchy, ill-defined, ragged RL
  • 1/3 show “onion skinning” on the surface of the involved bone due to subperiosteal bone formation
    • Rare in jaw lesions
    • Not pathognomonic
    • Periosteal Osteosarcoma also shows onion skinning
97
Q

What is the treatment for Ewing Sarcoma?

A

Multi-agent chemo + surgery + radiaiton

98
Q

What is the prognosis for Ewing Sarcoma?

A
  • 70% 5 yr survival for localized disease
  • 25% have metastasized @ presentation to lungs + bone
    • ​Single most important prognostic factor
    • Leading to a 25% 5 yr survival
99
Q

What lesions of Ewing Sarcoma have the worst prognosis?

A
  • Pelvic lesions have the worst prognosis
  • Proximal lesions worse than distal lesions
  • Possible better prognosis for jaw lesions
100
Q

What is the most common cancer involving bone?

A

Metastatic Carcinoma

101
Q

What population is more commonly affected by Metastatic Disease?

A

> 50% of pts are > 50 yrs old

The pts are older because they had to have already developed a primary tumor

102
Q

What locations are affected by Metastatic Disease?

A

Posterior Mandible (61%) - molar area

Maxilla (24%)

Soft tissue - gingiva (15%)

103
Q

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

A

Batson’s Paravertebral Plexus of Veins

  • Has no valves
  • Tumor cells can easily go up the spinal cord to the jaw
104
Q

What are the most common primary tumors that metastasize to the jaw? (6)

A
  • Breast or Prostate, usually appear RO
  • Lung + Kidney (clear cells)
  • Thyroid + Colon
105
Q

Where are 15% of metastatic disease of the soft tissue located?

A

Gingiva

Resembles pyogenic granuloma, but it’s firm

106
Q

In the soft tissue (gingiva) where do you most often see primary tumors from?

A

​“MLK is the breast”

  • Melanoma
  • Lung
  • Kidney
  • Breast
107
Q

What are the Signs and Symptoms of Metastatic Disease? (7)

A
  • Pain + Swelling
  • Tooth mobility mimicking periodontal ds with PDL widening
  • Trismus - if condylar area
  • Paresthesia - “numb-chin sign”
    • Mandibular metastasis with involvement of the mental nerve
  • Hemorrhage
  • Pathologic Fracture
108
Q

What is in the differential diagnosis of a painful, non-healing tooth socket with something growing out of it?

A
  • Granulation Tissue
    • Most often this
  • Lymphoma
  • Metastatic Disease
    • Most often carcinoma from another location
109
Q

What is the radiographic appearance of Metastic Disease?

A
  • Poorly defined, moth eaten RL
  • Can see some RO if mets of a primary tumor of the breast or prostate
    • These 2 carcinomas have the potential to induce bone formation
    • ISH for prostate cells
110
Q

What is the Histology of Metastatic Disease?

A
  • Looks like tissue of origin
  • “Seeded Effect”
    • ​May show diffuse infiltration or scattered tumor cells through the CT
111
Q

What is the treatment for Metastatic Disease? (2)

A
  • Palliation, usually with radiation therapy
  • Bisphosphonates
    • Slow progression of bone mets
    • Decrease bone pain and fracture risk
112
Q

At what stage does Metastatic Disease usually appears in the oral cavity?

A

Stage IV - widely disseminated

113
Q

What is the prognosis for pts with Metastatic Disease?

A

Very Poor

Most pts die within 1 yr of the dx

Oral Pathology Exam 3 (8 decks)

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