Odontogenic Cysts Flashcards

1
Q

What are the two types of Odontogenic Cysts?

A

Inflammatory
or
Developmental

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

List the type of Inflammatory cysts

(4)

A
  • Periapical (radicular)
  • Residual periapical
  • Buccal bifurcation
  • Paradental
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

List the types of Developmental Cysts?

(9)

A

‐ Dentigerous
‐ Eruption
‐ Gingival cyst of newborn
‐ Gingival cyst of adult
‐ Lateral periodontal
‐ Glandular odontogenic
‐ Odontogenic keratocyst
‐ Orthokeratinized odontogenic
‐ Calcifying Odontogenic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

All of the following are histologically the same because they are all what?

-Periapical (radicular)
‐ Residual periapical
‐ Buccal bifurcation
‐ Paradental

‐ Dentigerous
‐ Eruption
‐ Gingival cyst of newborn
‐ Gingival cyst of adult

A

squamous epithelial lined cysts

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What are the sources of epithelium
within the jaw bone ?

(6 sources)

A

▪ Epithelial rests of Malessez
▪ Reduced enamel epithelium
▪ Fissural cysts – when 2 pieces of bone come together
▪ Odontogenic cysts
▪ Epithelial component of odontogenic tumors
▪ Salivary gland inclusions – rare, incorporated in development

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

radicular cyst, inflammatory cyst are other names for

A

Periapical Cysts

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

▪ The most common cyst of the jaws

A

Periapical Cysts

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Periapical Cysts

Demographic and location

A

▪ Any age (peak in 3rd ‐ 6th decades, rare in 1st decade)
▪ No sex predilection
▪ MX > MD (anterior MX most common)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Tooth vitality and Periapical Cysts

A
  • Involved tooth usually non‐vital/non‐responsive with thermal and electric pulp testing
  • Should test vitality of tooth if see radiolucency in apex\
  • If tooth vital, and still see radiolucency ► should do biopsy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Periapical Cyst

(Radiographic)

A
  • Usually appears as well‐circumscribed periapical radiolucency with widening of the PDL space and/or loss of lamina dura
  • Typically small (< 1 cm) but can grow to large dimensions if left untreated
  • Radiographic findings can NOT be used for definitive diagnosis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Why the Radiographic findings of Periapical Cyst can NOT be used for definitive diagnosis?

A

‐ similar appearance with:

  • periapical granuloma
  • odontogenic tumors
  • early COD {Cemento Osseous Dysplasia}
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Lateral radicular cyst appears on the lateral surface of the root of a non‐vital/non‐responsive tooth
‐ A differential for which cyst?

A

lateral periodontal cyst

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is this radiographic finding?

A

Periapical Cysts

►Would need to test both teeth for vitality.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is this radiographic finding?

A

Periapical Cyst

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is this radiographic & clinical findings?

A

Periapical cyst

shows inflammation at site
abscess developed fistula tract thru
soft tissue. Pt will have pain until
pressure is released

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

The wall of which cyst?

A

Periapical Cyst

Open clear areas = Cholesterol clefts where fat
used to be. Multinucleated cells (purple dots)
trying to break down cholesterol

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What is this and what is it associated with?

A

keratin pearl – can be associated w/SCC

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Periapical Cyst

treatment

A
  • endodontic therapy or extraction of involved teeth
  • larger lesions may require biopsy along with endodontic therapy
  • lesions which fail to resolve should be biopsied
  • follow-up at 1-2 years
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Residual Cyst

Etiology

A
  • After tooth extracted, not properly cleaned ► the residual cells of the cyst lining and inflammatory cells continue to proliferate
  • Has to be at site where tooth was previously removed
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Residual Cyst

Radigraphically

A
  • well defined round to oval radiolucency in the site of a previous extraction
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Residual Cyst

Histologically is identical to which cyst?

A
  • identical to the radicular cyst (periapical cyst)
  • Should biopsy to rule out other causes
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Residual Cyst

Treatment

A

-Removal

  • Enucleation if small
  • Marsupialization if large
  • Note:*
  • Enucleation* means: removal of an organ or other mass intact from its supporting tissues

Marsupialization means: surgical technique of cutting a slit into an abscess or cyst to empty its contents and suturing the edges of the slit to form a continuous surface from the exterior surface to the interior surface of the cyst or abscess.
Promotes Decompressing and shrinkage.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What are these radiographic findings?

A

Residual Cysts

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What is the radiographic finding?

A

Residual Cyst

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Paradental Cyst

Etiology

A

Some controversy over this designation
‐ some think they are inflammatory cyst
‐ some think they are developmental cysts
▪ Etiology: remains unclear

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Paradental Cyst

Radiographically

A
  • Radiolucent area noted
  • most frequently, along the distal aspect of an impacted or partially erupted third molar
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Which cyst has been associated w/ enamel extensions into furcation areas of the
involved teeth?

A

Paradental Cyst

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Paradental Cyst

Treatment

A

Extraction of the tooth along with the lesion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

What is the radiographic finding?

A

Paradental Cyst

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

What is the radiographic finding?

A

Paradental Cyst

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Buccal Bifurcation Cyst

is similar to what Cyst ?

A

Similar to a paradental cyst

EXCEPT: location is central on the buccal of mandibular first molars

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Buccal Bifurcation Cyst

Etiology

A

unclear

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Buccal Bifurcation Cyst is most commonly seen with eruption of what tooth?

A

The eruption of the permanent first molar

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

Buccal Bifurcation Cyst

Clinically

A

seen as

  • swelling
  • tenderness of soft tissue over involved area
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Which

Radiograph type is best to see

Buccal Bifurcation Cyst?

A

▪ Radiolucency best seen with an occlusal radiograph

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

What is the radiographic finding?

A

Buccal Bifurcation Cyst

as seen in occlusal radiographs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

What is the radiographic finding?

A

Buccal Bifurcation Cyst

as seen in occlusal radiographs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

Buccal Bifurcation Cyst
Treatment

A

Enucleation of cyst; tooth extraction unnecessary
▪ Some cases resolve w/o surgery
▪ Some resolve w/ daily irrigation of buccal pocket with saline/hydrogen peroxide

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

Dentigerous Cyst
also known as ?

A

Follicular Cyst

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

What is most common type of developmental odontogenic cysts?

20% of all epithelial lined cysts of the jaw

A

Dentigerous Cyst

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

Dentigerous Cyst

Origin & Etiology

A

Originates: by the separation of the follicle from the crown of an unerupted tooth

Pathogenesis: accumulation of fluid between the tooth and the reduced enamel epithelium

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

Dentigerous Cyst

Clinically

A

▪ Small cysts typically asymptomatic and picked up
on routine radiographic exam
▪ Large lesions may show expansion of bone
▪ Cysts may become infected, especially if partially erupted
tooth

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

Dentigerous Cyst

Demographics & Location

A
  • Mostly mandibular 3rd molars (rarely unerupted deciduous teeth)
  • Most commonly present in 2nd and 3rd decades
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

What is a key characteristic of Dentigerous Cyst location?

A
  • Attached to the tooth at the CEJ
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

Small Dentigerous Cyst
are hard to differentiate radiographically from —?

A

enlarged/hyperplastic follicle

Rule of thumb:

  • If 4‐5mm or more of radiolucency ► dentigerous cyst
  • If <4mm of radiolucency► can be hyperplastic follicle
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

dentigerous cyst or
follicle ?

A

_dentigerous cys_t
b/c *attachment at CEJ

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

What is the radiographic finding?

A

Dentigerous Cyst

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

What are these radiographic findings?

A

dentigerous cyst

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

What are these radiographic findings?

A

dentigerous cyst

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

What is the radiographic finding?

A

dentigerous cyst

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

What is this gross finding?

A

Grossly image of

Dentigerous Cyst

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

Dentigerous Cyst

Treatment

A
  • Decompression: Try to open window in the jawand put tube into cyst lumen and have pt irrigate a few times a day for a few weeks ► release pressure and allows bone to grow back ► cyst will shrink
  • If get rid of whole area surgically► c_an risk_ fracturing the jaw
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q

What is the Soft tissue counterpart of a dentigerous cyst?

A

Eruption Cyst

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q

Eruption Cyst also known as

A

eruption hematoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

Eruption Cyst

Etiology

A
  • Results from accumulation of fluid in the follicular space when the tooth has erupted over the alveolar bone *NOT in bone*
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

Eruption Cyst

Demographic & Location

A

▪ Usually seen in 1st decade (children)

▪ Most often involves 1st permanent molar and maxillary incisors

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
57
Q

Eruption Cyst

Clinically

A

Frequently normal mucosal color, BUT surface trauma (ex. chewing) may result in bleeding into the cystic space► may look purple or blue
▪ Usually soft or fluctuant(like a balloon) upon palpation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
58
Q

Eruption Cyst

Treatment

A
  • Unless symptomatic, no treatment required, cysts resolve upon eruption of teeth
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
59
Q

What is the clinical finding?

A

Eruption Cyst

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
60
Q

What is the clinical finding?

A

Eruption Cyst

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
61
Q

Cysts of the Newborn

can either be — or —

A

Palatal cysts

or

Gingival cyst

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
62
Q

Palatal cysts

Types

&

Location

A

‐ Bohn’s nodules: scattered over HP (hard palate), often junction of HP and SP (soft palate)

‐ Epstein’s pearls: along median palatal raphe

63
Q

Cysts of the Newborn:

Palatal cysts

Demographics

A
  • Seen in 60‐80% of neonates
64
Q

Cysts of the Newborn:
Palatal cysts

Clinically

A
  • 1‐3 mm cream to white papules (keratin filled cysts)

*NOT in bone*

65
Q

Cysts of the Newborn:
Palatal cysts

Treatment

A

No treatment is required
‐ Resolve (degenerate or rupture) on their own in a
few months
‐ Once baby eats solid foods, will go away

66
Q

What is the clinical finding?

A

Cysts of the Newborn:
Palatal cysts

67
Q

Cysts of the Newborn:
Gingival cyst of the Newborn

Also known as

A

Dental lamina cysts

68
Q

Gingival cyst of the newborn

demographics & Location

A
  • Found superficially on the alveolar ridge mucosa
  • MX > MD
  • Rarely seen after 3 mos. of age
69
Q

Gingival cyst of the newborn

Treatment

A

▪ No treatment is necessary

▪ Spontaneously resolve (degenerate or rupture)

70
Q

Gingival cyst of the newborn

Clinically

A
  • 1‐3 mm creamy white papule (keratin filled cysts)
  • *NOT in bone*
71
Q
A

Gingival cyst of the newborn/ Dental lamina cysts/Cysts of the Newborn-gingival

72
Q

What is the soft tissue counterpart of the lateral periodontal cyst ?

A

Gingival Cyst of the Adult

73
Q

Gingival Cyst of the Adult

Origin

A

Derived from dental lamina rests
‐ Rests of Serres

74
Q

Gingival Cyst of the
Adult

Demogrophic & Location

A

▪Uncommon lesion
▪ 60‐75% mandibular canine/premolar area
‐ most common location on the facial or buccal aspect
5th and 6th decade most common

75
Q

Gingival Cyst of the
Adult

Clinically

A
  • Painless, dome‐like swellings up to 5 mm in diamete
  • Often with a bluish or grayish hue
76
Q

Gingival Cyst of the
Adult

has similar histology to which cyst?

A

lateral periodontal cyst

77
Q

Gingival Cyst of the
Adult

Treatment

A
  • simple surgical excision
  • Unlikely to recur/come back
78
Q

What is the clinical finding?

A

Gingival Cyst of the
Adult

79
Q

What is the clinical finding?

A

Gingival Cyst of the
Adult

80
Q

What is the clinical finding?

A

Gingival Cyst of the
Adult

notice the bluish hue

81
Q

Lateral Periodontal Cyst represents the intrabony counterpart of which cyst?

A

gingival cyst of the adult?

82
Q

Lateral Periodontal
Cyst

Origins

A
  • Developmental cyst believed to arise from dental lamina rests
83
Q

Lateral Periodontal cyst is diagonsed when cysts occur in the lateral periodontal region and after what have been excluded?

A
  • an inflammatory origin cysts or the diagnosis of odontogenickeratocyst have been excluded
84
Q

Lateral Periodontal Cyst

Charcterstics and tooth vitality

A

▪ Commonly asymptomatic and found on routine radiographic exam

▪ Associated teeth tests vital/responsive with electric pulp test

85
Q

if you see a radilucency Lateral to a teeth

how would you know if it’s

Lateral Periodontal Cyst

or

Lateral Radicular Cyst

or

Lateral OKc

A

If pulp alivelateral periodontal cyst or Lateral Okc ( if huge lesion)

‐ If pulp dead► lateral radicular cyst

86
Q

Lateral Periodontal Cyst

Demographic and Location

A

▪ Most likely found after age 30
▪ Males>Females
▪ ~65% mandibular canine/premolar area
‐ Can also be seen between canine and lateral incisor

87
Q

Lateral Periodontal Cyst

Radiographically

A

Present as well circumscribed, unilocular radiolucencies between 2 teeth, located lateral to tooth root
▪ Most often 0.5‐1.0 cm in diameter
▪ Radiographic features are NOT diagnostic

88
Q

Which is here is

Lateral Periodontal Cyst

Lateral Radicular Cyst

Lateral Odontogenic Kertocyst

A
  • Could be differential for lateral Odontogenic keratocyst, except this does not grow in size
  • Lateral radicular cysts from an accessory canal if tooth is non vital
  • or it could be Lateral Periodontal Cyst if tooth is vital!
89
Q

What is the radiographical finding?

A

Lateral Periodontal
Cyst

90
Q

What is the radiographical finding?

A

Lateral Periodontal
Cyst

91
Q

What is the histological finding?

A

Lateral Periodontal Cyst

see the alternating
thin to thick epithelium

a characteristic of these cysts

92
Q

What is the histological finding?

A

Lateral Periodontal Cyst

93
Q

Lateral Periodontal
Cyst

Treatment

A
  • consists of conservative enucleation
94
Q

What cyst is a variant of lateral periodontal cyst?

A

Botryoid Odontogenic
Cyst

95
Q

Botryoid Odontogenic
Cyst

Grossly and Microscopically

A

shows a grape‐like cluster of small
individual cysts

96
Q

Botryoid Odontogenic
Cyst

Radiographically

A

▪ Either unilocular or multilocular on radiographs, depending on size of the lesion
▪ Cyst lining similar to lateral periodontal cyst

97
Q

What is the radiographical finding?

A

Botryoid Odontogenic
Cyst

well circumscribed, between 2 teeth (similar to
lateral odontogenic cyst), multilocular

98
Q

What is the radiographical finding?

A

Botryoid Odontogenic
Cyst

99
Q

Glandular Odontogenic
Cyst

Charcterstics

A
  • A rare odontogenic cyst which exhibits features of glandular differentiation within the epithelium
  • Presumably represents the pluripotentiality of odontogenic epithelium
100
Q

Glandular Odontogenic
Cyst

Demographics

A

▪ Wide age range from 2nd to 9th decades
mean age 49
▪ ~ 80% of cases in mandible
▪ Anterior lesions

‐ More common
May cross the midline

101
Q

Glandular Odontogenic
Cyst

Radiographically

A

▪ Uni‐ or (more often) multilocular radiolucency

Well‐defined with a sclerotic border

102
Q

Glandular Odontogenic
Cyst

reccurance rate

A

(~ 25% recurrence rate)

Can be locally aggressive

103
Q

Glandular Odontogenic
Cyst

Clinically

A

▪ Usually asymptomatic unless inflamed

104
Q

“Primordial” Cyst

why it is controversial!

A
  • Mixed up with OkC
  • Originally meant to describe cyst which develops in bone at a site where a tooth was meant to develop (usually a third molar)
  • If this lesion exists, it is truly rare and would have histology distinct from OKC
    • In the current literature, reference has been almost nonexistent
105
Q

What is the radiographical finding?

A

“Primordial” Cyst

Assuming histologically it is different from OKC

106
Q

“Primordial” Cyst

is not a true ——-

A
  • lesion, was actually some other type of cyst
    • it is now thought that most of the reported Primordial cysts were actually OKCs
107
Q

Odontogenic
Keratocyst

OKC

Also known as

A

keratocystic odontogenic tumor (KOT) -2005 WHO

but now it’s back to OKC

108
Q

Odontogenic
Keratocyst (OKC)

Etiology

A
  • Growth and expansion of this lesion due not only to osmotic effects/pressure, but to unusual gene expressions
109
Q

Which unusal gene expression causes growth and expansion of OKC ?

A
  • Expresses Ki‐67 (high rate of cell proliferation)
  • O_verexpression of Bcl‐2_ (antipoptotic protein)
  • Overexpression of MMP’s 2 and 9 (thought to allow growth into connective tissue)
  • Mutation of PTCH, a tumor suppressor gene
    • when PTCH is non‐functional → cell proliferation
110
Q

Odontogenic
Keratocyst (OKC)

Demographic & Location

A
  • ~ 60% present in 2nd and 3rd decade, but can occur at any age
  • Mandible affected in 60‐80% of cases
    • tendency to occur in posterior mandible and ramus
  • 25‐40% of cases involve an unerupted tooth
  • 5% of patients have multiple cysts
111
Q

Odontogenic
Keratocyst (OKC)

differes from

Meloblastoma

in its growth pattern

A

Odontogenic Keratocyst (OKC) :grows in anterior to posterior manner before causing cortical expansion

while

Meloblastoma: causes cortical expansion early

112
Q

Which cyst make up ~10‐15% of all odontogenic cysts?

A

Odontogenic
Keratocyst (OKC)

113
Q

5% of Odontogenic
Keratocyst (OKC)
are associated with which syndrome?

A

nevoid basal cell carcinoma
syndrome
(Gorlin syndrome)

114
Q

What are the site distribution of OKC?

A

Most of OKC in
posterior region

115
Q

Odontogenic
Keratocyst

Reccurance Rate

A
  • HIGH Recurrence Rate
  • Benign, but locally aggressive biologic behavior
  • Solitary OKCs have ~10% recurrence rate with appropriate treatment
  • _Multiple OKCs hav_e ~ 30% recurrence rate
116
Q

Odontogenic
Keratocyst

(OKC)

Reccurance Rate Order

from highest to lowest reccurance rate

A

Syndrome OKC > Multiple OKC > Solitary OKC > Conventional odontogenic cysts

117
Q

Odontogenic
Keratocyst

OKC

Radiographically

A
  • Usually a well‐circumscribed radiolucency with smooth, often corticated margins

▪ Cysts may be
Unilocular (most common)
Multilocular (larger lesions)

118
Q

Odontogenic
Keratocyst

OKC

clinically

A

Small cysts are typically asymptomatic and picked up on routine radiographic exam
Larger cysts may or may not be asymptomatic
▪ *Cysts tend to grow in an antero‐posterior direction prior to lateral growth ►therefore cysts are usually quite large when they start to expand the cortical plate

119
Q

Odontogenic
Keratocyst

OKC

Has similar Radiographic findings with ?

A
  • dentigerous cyst
  • ameloblastoma
  • and others
120
Q

Odontogenic
Keratocyst
OKC

Treatment

A

Marsupialization (decompression)
Peripheral ostectomy
‐ Carnoy’s solution
Resection
Medications targeted to PTCH
Long term follow‐up

121
Q

What is the radiographical finding?

A

Odontogenic
Keratocyst
OKC

122
Q

What is the radiographical finding?

A

Odontogenic
Keratocyst
OKC

123
Q

What is the radiographical finding?

A

Odontogenic
Keratocyst
OKC

124
Q

What is the radiographical finding?

A

Odontogenic
Keratocyst
OKC

125
Q

What is the radiographical finding?

A

similar to
lateral
periodontal cyst

but is actually
OKC

126
Q

What is the histological finding?

A

Odontogenic
Keratocyst

Histology

Notice the daughter cysts

127
Q

Nevoid Basal Cell Carcinoma
Syndrome

is also known as —– ?

A

Basal Cell Nevus or Bifid Rib Syndrome

or

Gorlin syndrome

128
Q

Which cyst is assoicated with

Nevoid Basal Cell Carcinoma
Syndrome

?

A

Odontogenic Keratocyst
“OKC”

129
Q

Nevoid Basal Cell Carcinoma
Syndrome

(Gorlin syndrome)

_modes of inheritanc_e

A

Autosomal dominant inheritance

130
Q

Which Gene mutation and pathway

associated with

Nevoid Basal Cell Carcinoma
Syndrome

(Gorlin syndrome)

A
  • Mutation of PTCH (tumor suppressor gene)
  • in the Sonic Hedge Hog pathway
131
Q

Nevoid Basal Cell Carcinoma
Syndrome

Prognosis

A

■ Prognosis depends on progression of skin tumors

132
Q

Nevoid Basal Cell Carcinoma
Syndrome

Treatment

A

✎Surgery (typically MOHS)
✎Sometimes curette them
✎ Radiation therapy (RT) is typically not the first line of therapy with small lesions RT
✎Cryotherapy which means they just use a little liquid nitrogen and freeze them
✎Photodynamic therapy with photosensitizer and topical medications
■ New medication: Vismodegib inhibits sonic hedgehog pathway by binding smoothened (SMO)

  • suppressive rather than curative cause it seems to work for short time and after ~7-8 months ..may also helps suppress growth of OKC
133
Q

What is this radiographic finding?

A

✎A patient who has Nevoid Basal Cell Carcinoma
Syndrome

✎We can see multiple cystics areas and lesions in
the jaws, maxillary and mandible
Both 3rd molar displaced in the maxilla because of
the cyst

134
Q

What is this radiographic finding?

A

Multiple lesions, impacted 3rd molar in mandible
and displaced 3rd molar up into the sinus,
✎These too many lesions hard to manage the issue
with a surgery
✎This large area on the left mandible – good example of why we do
decompression because if you just remove this lesion
and the entire area is left open, this would be an area
risk for fracture

135
Q

What is this called

which can be seen with

Nevoid Basal Cell Carcinoma
Syndrome

A

✎An example of the pitting that can be seen palmar
and plantar

~ This is a side of a hand
✎This is an early stage of basal carcinoma which
never goes on (like it is aborted)

136
Q

What are these findings that is associated with

Nevoid Basal Cell Carcinoma
Syndrome?

A
  • thousands of basal cell carcinoma is developing on the skin

-very difficult to manage with surgery,
~ That’s why they remove
the larger ones, the deeper ones ~ They leave the one
that’s less as an issue until they get to a larger size to
be removed

137
Q

Why Basal Cell Carcinoma is very problematic ?

A

It’s not the lesion themselves causing metastasis
that’s the issue, it’s the lesion growing deeply and in affecting adjacent structures that really is the
issue with basal carcinoma

138
Q

What is the Most common type of skin cancer?

A

Basal Cell Carcinoma
(BCC)

139
Q

Basal Cell Carcinoma
(BCC
)

Demographics

A
  • 2-3 million cases a year
  • About 3 out of 4 skin cancers are basal cell carcinomas
140
Q

Basal Cell Carcinoma

Growth and location

(BCC)

A
  • Develop in the lowest layer of the epidermis, called the basal Layer
  • Develops on sun-exposed areas: cumulative DNA Damage
    - Slow-growing
    • If not treated, basal cell cancer can grow into nearby areas
    and invade the bone or other tissues beneath the skin
141
Q

Basal Cell Carcinoma

Progrssion

(BCC)

A

within 5 years of being diagnosed with
BCC►35%-50% of people develop a new skin cancer

142
Q

Calcifying Odontogenic Cyst

​COC

also known as

?

A
  • Calcifying Cystic Odontogenic Tumor
  • Gorlin Cyst ( don’t confuse it with Gorlin syndrome)
  • Ghost Cell Tumor

*

143
Q

Calcifying Odontogenic Cyst
(COC)

can present in 3 types

A
  1. ■ Cystic Unilocular COC
    • COC with odontoma (~ 20%)
    • Extraosseous/peripheral – present in older patients
  2. ■ Solid COC (odontogenic ghost cell tumor)
    • Often demonstrate a more aggressive behavior
    • WHO once considered them all CCOT now back to COC
  3. Odontogenic ghost cell carcinoma
    • very rare lesion
144
Q

Collision Tumors is a term used to describe lesions involving Calcifying Odontogenic Cyst (COC), what does that mean?

A
  • where you see both features of ameloblastoma with COC or adenomatoid odontogenic tumor with COC
145
Q

Calcifying Odontogenic Cyst
(COC)

may occur in association

with

which tumors or cysts?

A
  • Odontomas (a benign tumour linked to tooth development)
  • Ameloblastomas (rare, noncancerous (benign) tumor)
  • Adenomatoid odontogenic tumor (rare tumor of epithelial origin that is benign, painless, noninvasive, and slow-growing)
146
Q

Calcifying Odontogenic Cyst
(COC)

Demographics & Location

A

■ Peak in second decade, most before age of 40
■ Frequently presents anterior to molars
■ ~ 20% extraosseous (peripheral), found in older age group (~ 50 years of age)

■ Female > Male

~ 70% occur in MX
■ One third are associated with unerupted teeth, usually a canine

147
Q

Calcifying Odontogenic Cyst

Radiographically

(COC)

A

■ Usually a well-circumscribed unilocular radiolucency, infrequent multilocular cases
One third to one half show radiopaque structures within the radiolucency
■ When you see calcifications within a lesion, you don’t use the term uni or multi locular anymore, but they are called mixed radiolucent/radiopaque lesions

■ May cause resorption or displacement of roots
One third are associated with unerupted Canine

148
Q

What is this radiographic finding?

A
**Calcifying
Odontogenic Cyst (COC)**
  • in the mandible and you can see it well circumscribed radiolucency
  • a little bit of blunt root resorption in this area
  • No calcifications in this one yet ►so this is still unilocular radiolucency
149
Q

What are the clinical and radiographic findings here?

What is this lesion?

A
  • Clinical finding for this patient was Obliteration of the vestibule space, because the mandible is showing expansion
  • radiographically:we see radiolucency going as far as the first molar
  • This is a mixed radiolucent radiopaque lesion in developing calcifications.
  • This is an example of Calcifying Odontogenic Cyst (COC)
150
Q

The hallmark of Calcifying
Odontogenic Cyst COC Histology is

A

Ghost cells

They have that sort of polygonal shape or roundish shape with the pink that looks like the cytoplasm, but in
the location where the nucleus would have then, there’s an empty spot

151
Q

Histologically speaking, Calcifying
Odontogenic Cyst COC, basically
looks similar to what epithelium?

A

ameloblastic epithelium

152
Q

Calcifying Odontogenic Cyst COC

Treatment

A

Enucleation with peripheral ostectomy ~ Very similar to odontogenic keratosis
Follow up is long term because s_ome of the solid tumors have a more aggressive behavior_

■ Peripheral lesions are treated with excision

153
Q

When COC is associated with another tumor, ameloblastoma, how would you treat?

A

■ the treatment is based on the more aggressive tumor

~ So you would treat the ameloblastoma.

~You wouldn’t treat conservatively the COC though

154
Q

Odontogenic ghost cell carcinoma

Prognosis

A

(5 year survival ~ 70%).

  • It is rare and shows cytologic atypia histologically
  • It has an unpredictable biologic behavior