exam 3 Flashcards

1
Q

not malignant, favorable for recovery

A

benign

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2
Q

occurring within bone

A

central

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3
Q

surrounded by a capsule of fibrous CT

A

encapsulated

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4
Q

abnormal increase in the NUMBER of normal cells

A

hyperplasia

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5
Q

likely to cause the death of the host

A

malignant

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6
Q

transport of neoplastic cells to parts of the body remote from the primary
tumor and establishment of a new tumor there

A

metastasis

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7
Q

ormation of tumors by the uncontrolled proliferation of cells

A

neoplasia

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8
Q

benign, localized overgrowth of melaoncytes

A

nevus

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9
Q

study of tumors or neoplasms

A

oncology

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10
Q

occurring in various forms

A

pleomorphic

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11
Q

increase in quantity

A

proliferation

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12
Q

malignant tumor of epithelium

A

carcinoma

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13
Q

disordered growth

A

dysplasia

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14
Q

– staining more intensely than normal

A

hyperchromatic

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15
Q

infiltration and active destruction of surrounding tissue

A

invasion

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16
Q

cancer; resistant to tx and causes death

A

malignant tumor

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17
Q

tumor developing in a spot different from a primary tumor

A

metastitic tumor

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18
Q

tumor

A

neoplasm

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19
Q

tooth forming

A

odontogenic

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20
Q

original tumor source for matastitis

A

primary tumor

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21
Q

malignant tumor of CT

A

sarcoma

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22
Q

means new growth in which cells exhibit uncontrolled proliferation

A

neoplasia

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23
Q

a mass of cells exhibiting uncontrolled proliferation

A

neoplasm

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24
Q

study of tumors

- causes of neoplasia: chemicals, viruses, and radiation

A

oncology

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25
Q

remain localized and does not have the ability to spread to distant sites

A

benign tumor

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26
Q

invades and destroys surrounding tissue and has the ability to spread
throughout the body “CANCER”

A

malignant tumor

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27
Q

benign fat tumor

A

lipoma

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28
Q

bone tumor

A

osteoma

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29
Q

– malignant tumors of epithelium

i. 10x more common than sarcomas

A

carcinoma

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30
Q

malignant tumors of C.T.

A

sarcoma

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31
Q

treatment of tumors

A
  • Benign – surgical excision; Malignant – surgery, chemotherapy, radiation or combo
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32
Q

3 types of epithelial tumors

A
  • 3 types:
    i. tumors from squamous epithelium
    ii. tumors from salivary gland epithelium
    iii. tumors from odontogenic epithelium
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33
Q

Tumors of Squamous Epithelium

A
  • Papilloma – benign tumor of squamous epithelium that appears clinically as a relatively small exophytic pedunculated or sessile growth
    i. Cauliflower-like in appearance
    ii. Occur at any age
    iii. Most commonly found in soft palate
    iv. Tx: surgical excision
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34
Q

white plaque-like lesion of the oral mucosa that cannot be rubbed off and cannot be diagnosed as a specific disease

i. Histologically it may show epithelial dysplasia, or squamous cell carcinoma
ii. When found on floor of the mouth or lateral border of tongue – biopsy

A

leukolpakia

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35
Q

an oral mucosal lesion that may appear as a smooth red patch or a granular red and velvety patch

i. A lesion showing a mixture of red and white areas is called Speckled Leukoplakia
ii. Less common than leukoplakia

A

erythroplakia

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36
Q

i. Histological dx
ii. Pre-malignant condition
iii. Preceeds SCC
iv. Presents clinically as leukoplakia, erythroplakia, or speckled leukoplakia
v. “Carcinoma in Situ”
vi. dysplasia – means disordered growth

A

epithelial dysplasia

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37
Q

squamous cell carcinoma SCC

A

i. A malignant tumor of squamous epithelium
ii. Most common malignancy of oral cavity and it can metastasize to distant sites (nodes then lungs/liver)
iii. Usually an exophytic ulcerative mass
iv. Essential feature of SCC is the invasion of tumor cells through the epithelial basement membrane into the underlying CT
v. Most common sites is floor of the mouth, lateral borders of tongue, soft palate, tonsillar pillar, and retromolar areas
vi. Prognosis for SCC of the lips/skin is better than SCC of oral cavity
vii. Majority of SCC occurs in older patients (40’s)
viii. Risk factors – tobacco and alcohol
ix. Tx: surgical excision with or without radiation or chemo ( 95% 5 yr survival rate if detected early)
1. smaller the lesion the better the prognosis

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38
Q

i. A form of SCC that has a better prognosis
ii. Slow growing exophytic tumor with a pebbly white/red surface
iii. Tx: surgical excision

A

verrucous carcinoma

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39
Q

i. A malignant skin tumor composed of basal cells derived from squamous epithelium
ii. Assoc. with sun exposure
iii. Appears clinically as a nonhealing ulcer of the skin with a characteristic rolled borders
iv. Does not occur in the oral cavity
v. Recommend biopsy if not healed within 10 days

A

basal cell carcinoma

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40
Q
  • minor salivary glands tumors are most commonly located at the junction of the
A

hard/ soft palate

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41
Q

benign tumor of salivary gland

A

adenoma

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42
Q

adenocarcinoma

A

malignant tumors of salivary glands

i. specific names:
1. adenoid cystic carcinoma
2. mucoepidermoid carcinoma

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43
Q

i. Benign
ii. Most common (90%) of all benign tumors
iii. Histologically – encapsulated tumor of mixed tissues (epithelium and CT)
iv. Extraoral site – parotid gland
v. Intra oral site – palate
vi. * slowly enlarging, nonulcerated, painless, dome-shaped mass
vii. usually older (40’s)
viii. Tx: surgical removal

A

pleomorphic adenoma

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44
Q

i. Benign encapsulated salivary gland tumors that are much rarer than pleomorphic adenomas
ii. Occurs in adults in upper lip
iii. Tx: surgical excision

A

monomorphic adenoma

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45
Q

warthins tumor adenolymphoma

A

i. Type of monomorphic adenoma with 2 tissue types (epithelial and lymphoid)
ii. Presents as a painless, soft, fluctuant mass of the parotid gland
iii. Bilaterally
iv. Tx: surgical excision

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46
Q

adenoid cystic carcinoma (cylindroma)

A

i. Malignant tumor of salivary gland origin that can originate from either major or minor salivary gland tissue
ii. Slow growing
iii. Painful because they tend to surround nerves
iv. More common in women
v. Complete surgical excision
vi. Poor prognosis if metastasizes

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47
Q

mucoepidermoid carcinoma

A

i. Malignant salivary gland tumor
ii. Most likely associated with parotid
iii. Slowly enlarging mass
iv. Can appear within bone – uni or multilocular (RL) in mandibular PM and Molar
v. Most common malignant salivary gland tumor in children
vi. Tx: complete excision with long term follow-up for metastisis
1. low grade – 92% 5 year survival rate
2. high grade – 49 % 5 year survival rate

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48
Q

derived from tooth forming tissues

A

odontogenic tumors

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49
Q

ameloblastoma

A
  • Benign, slow growing aggressive epithelial odontogenic tumor occurring in both max. and mand. (when in the maxilla – DEATH)
  • Radiographically – multilocular soap-bubble-like (honeycombed) (RL)
  • 80% in mandible – in the MOLAR-RAMUS area
  • may cause expansion of the bone
  • slowly developing, asymptomatic swelling of the affected bone
  • Tx: complete surgical removal
    i. Recurrence common – follow-up with PAX in 6 months
50
Q

Calcifying epithelial odontogenic tumor CEOT

A
  • also known as a Pindborg tumor
  • benign epithelial odontogenic tumor that occurs less frequently than an ameloblastoma
  • tumor is composed of islands and sheets of polyhedral epithelial cells
  • radiographically – uni or multilocular (RL) with (RO) areas
  • usually adults
  • occurs 2x more in mandible – Premolar/molar area
  • Tx: complete surgical excision
51
Q

odontogenic myxoa

A
  • benign nonencapsulated infiltrating tumor composed of pale-staining mucopolysaccharide substances with dispersed cells having long processes
  • young people – 10 – 29 years
  • multilocular (RL) with poorly defined margins – can cause tooth displacement
  • Tx of Myxoma – complete removal – 25% reoccur
52
Q

cementifying and ossifying fibromas

A
  • cementifying fibroma is a benign well-circumscribed tumor composed of fibrous CT and rounded or globular calcifications resembling cementum
  • considered a fibro-osseous lesion because of fibrous CT and calcifications
  • ossifying fibroma – calcifications resemble bone trabeculae
  • cemento-ossifying fibromas – calcifications are a mixture of cementum and bone trabeculae
  • radiographically – well-defined (RL) or (RO) lesion
  • Tx: surgical excision
53
Q

benign cementoblastoma

A
  • cementum producing lesion that is fused to the root of a tooth
  • well defined (RO)mass in continuity with the root or roots of the affected tooth and obliterates the apex of the tooth
  • surrounded by a (RL) line
  • young adults
  • mand. molar or PM
  • PAIN is a frequent symptom
  • Tx: removal of lesion and tooth
54
Q
  • common condition of unknown cause that affects periapical bone
  • “Cementoma” – but is not a neoplasm
  • not a premalignant condition
  • asymptomatic found on routine PA x-ray
  • more common in black females in older population
  • teeth vital
  • fibro-osseous lesion
  • Tx: none
A

periapical cemental dysplasia

55
Q

florid osseous dysplasia

A
  • fibro-osseous lesion ( mixed RL/RO lesion )
  • a condition of disordered cementum and bone development
  • involves multiple quadrants
  • dense, sclerotic masses of bone or cementum appear as large (RO) areas
  • older black women (40’s)
  • Tx: none
    i. Complication – osteomyelitis in patients wearing C/C or RPD/RPD
56
Q

amelobastic fibroma

A
  • benign, nonencapsulated odontogenic tumor composed of both strands and small islands of odontogenic epithelium and tissue that resembles dental papilla
  • young children (less than 20 years)
  • mandibular PM – Molar region
  • radiographically – well defined or poorly defined uni or multilocular (RL)
  • Tx: surgical removal
57
Q

Adenomatoid odontogenic tumor

A

(AOT or OAT)

  • encapsulated, benign epithelial odontogenic tumor that has a distinctive age, sex, and site distribution
  • does not reoccur
  • duct-like structure in make-up
  • adenomatoid – gland-like
  • 70% OATS in females less than 20 years old and 70% in anterior max.
  • radiographically – well-circumscribed (RL)
  • OATS extend past CEJ and can involve 50 – 60% of the root
  • Tx: enucleation – removed in its entirety
    i. Rare reoccurance
58
Q

calcifying odontogenic cyst

A
  • nonaggressive cystic lesion lined by odontogenic epithelium with associated ghost cell keratinization
  • ghost cells – round structures with clear centers
  • most commonly seen in under 40
  • Tx: surgical enucleation – doesn’t recur
59
Q
  • odontogenic tumor composed of mature enamel, dentin, cementum, and dental pulp
  • most common odontogenic tumor
  • 2 types:
    i. compound – collection of numerous small teeth
    ii. complex – mass of enamel, dentin, cementum, and pulp that doesn’t resemble a normal tooth
  • compound – anterior maxilla
  • complex – posterior mandible
  • most small but can become large and cause displacement of teeth
  • Tx: surgical excision
A

odontoma

60
Q
  • exophytic lesion occurring only on the gingiva probably from cells of PDL
  • composed of cellular CT interspersed with scattered bone or cementum
  • well-demarcated sessile or pedunculated lesion originating from interdental papilla
  • young females
  • Tx: surgical excision with thorough scaling of adjacent teeth
  • Most common odontogenic tumor occurring in the gingiva
A

peripheral ossifying fibroma

61
Q

– benign tumor of mature fat cells

  • yellowish mass surfaced with thin overlying epithelium
  • Tx: surgical excision
A

lipoma

62
Q

tumors of nerve tissue

A
  • neurofibroma and schwannoma are benign tumors derived from nerve tissue
  • neurilemmoma – also called a shwannoma
  • both are derived from Schwann cells
  • tongue is most common intraoral site for these tumors
  • Tx: surgical excision
  • Neurofibromas occur in multiple neurofibromatosis syndrome called Von Recklinghausen’s disease – numerous neurofibromas on the skin
63
Q

granular cell tumor

A
  • benign tumor composed of large cells with granular cytoplasm
  • occurs on tongue as a painless, nonulcerated nodule
  • surgical excision
64
Q

congenital epulis

A
  • a granular cell tumor present at birth that presents as a sessile or pedunculated mass on the gingiva in the anterior maxilla in girls
  • Tx: surgical excision
65
Q
  • extremely rare in the oral cavity
  • rhabdomyoma – benign tumor of striated muscle
  • leiomyomas – tumor of smooth muscle – very raree
  • rhabdomysarcoma – malignant tumor of striated muscle – most common malignant soft tissue tumor of the head/neck in children
  • Tx: combo of chemotherapy and surgery – poor prognosis
A

tumors of muscle

66
Q

hemangioma

A
  • benign proliferation of capillaries
  • a common vascular lesion
  • most are present at birth
  • tongue is most common intraoral location
  • Tx: varies depending on size
67
Q

lymphangioma

A
  • benign tumor composed of lymphatic vessels
  • most common in tongue
  • cystic hygroma – a cystic lymphangioma in the neck
  • Tx: surgical
68
Q

malignant vascular tumors

A
  • Kaposis sarcoma – a malignant vascular tumor
  • Multiple sites composed of spindle-shaped cells mixed with slit-like spaces containing RBC
  • Presents as multiple purplish tumors of the lower extremities in elderly men
  • Hard palate and gingiva are the most common intraoral sites
  • Associated with HIV
69
Q

melanocytic nevi

A
  • intraoral commonly located on hard palate
  • 2x more women than men
  • between ages of 20-50 years
  • Tx: surgical excision
70
Q

nevus

A

i. A tumor of melanocytes – nevus cells

ii. Pigmented congenital lesion – present at birth

71
Q

malignant melonoma

A
  • a malignant tumor of melanocytes
  • associated with sunlight exposure
  • rapidly enlarging bluish to black mass
  • aggressive tumor
  • located on palate and maxillary gingiva
  • Tx: surgical excision
    i. Poor prognosis
72
Q
  • benign lesion composed of normal compact bone
  • torus palatinus – maxillary tori
  • torus mandibularis – mandibular tori
  • Tx: none unless a denture needs to be made
A

torus

73
Q
  • small nodules excrescence of normal compact bone occurring on buccal surface of max. or mandibular molar region
A

exostosis

74
Q
  • a benign tumor composed of mature, normal-appearing bone
  • slow growing tumor
  • asymptomatic
  • can cause expansion of the involved bone
  • Tx: surgical extraction
A

osteoma

75
Q
  • benign tumor composed of cellular fibrous C.T. and bone
  • rare but when they do occur, usually adults in 30’s – 40’s
  • well defined and ranges from (RL) to (RO)
  • Tx: surgical excision
A

ossifying fibroma

76
Q

ostosarcoma

A
  • malignant tumor of bone-forming tissue
  • most common 1 malignant tumor of bone in patients less than 40 years
  • tumors occur in mandible 2x more than maxilla
  • presents as a diffuse swelling or mass that is painful
  • patient presents with a toothache or tooth mobility
  • paresthesia of the lip is common in mand. tumors
  • radiographically varies from (RL) to (RO)
  • usually destructive
  • “sunburst” pattern radiographically
  • Tx: pre-op chemotherapy and surgery
77
Q

benign tumor of cartilage

A

chondroma

78
Q

a malignant tumor of cartliadge

A

chodrosarcoma

  • Tx: wide surgical excision
    i. Chemo and radiation are not effective
    ii. Poor prognosis – 30% 5 year survival rate
79
Q

leukima

A

overproduction of atypical WBC

  • normal bone marrow is replaced by a. proliferation of immature WBC’s
  • several types of Leukemia classified according to kind of cells
    i. myelocytes, lymphocytes, or monocytes
80
Q

2 forms of leukima

A

i. acute leukemia – most common in children and young adults and is characterized by a proliferation of poorly differentiated cells
ii. chronic leukemia – exhibits a proliferation of well-differentiated cells that occurs most frequently in middle-aged adults
- common oral manifestation of monocytic leukemia is diffuse gingival enlargement with persistent bleeding
- Tx: chemotherapy, radiation therapy, and corticosteroids
i. Bone marrow transplants sometime

81
Q

lymphoma (non hodgkins(

A
  • a malignant tumor of lyphoid tissue
  • clinical presentation of lymphoma is the gradual enlargement of involved lymph nodes
  • most common location for oral lymphoma is the tonsillar area
  • Tx: radiation, surgery, chemo or a combination
82
Q

multiple myeloma

A
  • systemic, malignant proliferation of plasma cells causing destructive lesions of bone
  • most patients are older than 40 years
  • men affected more often
  • patients present with bone pain and swelling
  • pathologic fracture of bone can occur
  • involved bones show multiple (RL) lesions
  • mandible more involved than maxilla
  • patients have fragments of immunoglobulins present in the urine – these are called Bence Jones proteins
  • extramedullary plasmacytoma – localized tumor of plasma cells in soft tissue
  • Tx: chemotherapy and radiation
83
Q

a trait or characteristic that is manifested when it is carried by only one of a pair of homologous chromosomes

A

dominant

84
Q

the appearance of the face

A

facies

85
Q

an individual with two different genes

A

heterozygote

86
Q

permanent change in the arrangement of genetic material

A

mutation

87
Q

entire physical, biochemical, and physiologic make-up of a person

A

phenotype

88
Q

et of signs or symptoms, or both, occurring together

A

syndrome

89
Q

a pair of chromosomes with an identical extra chromosome

A

trysomy

90
Q

science that studies inheritance and expression of inherited traits

A

genetics

91
Q

hereditary units that are transmitted from one generation to another

A

genes

92
Q
  • single strand (DNA – double)
  • sugar is ribose
  • base uracil (U) replaces thymine (T)
A

RNA ribonucleic acid

93
Q

4 types of rna

A

i. Messenger RNA – blueprint of the genetic DNS for coding of proteins
ii. Transfer RNA – transfers amino acids from the cytoplasm to the messenger RNA, positioning amino acids in the proper sequence to form polypeptides and hence proteins
iii. Ribosomal RNA – combines with several polypeptides to form ribosomes
iv. Heterogeneous RNA – found within the nucleus and is the precursor of messenger RNA

94
Q

gross chromosomal abnormalities

A

• Trisomy 21 – known as “Down’s Sydrome”

i. Most frequent
ii. Facies characterized by slanted eyes (facies – appearance of the face)
iii. Usually shorter, heart abnormalities present in more than 30%
iv. Fissured tongue
v. Premature loss of teeth are common – due to increase in gingival and periodontal disease – about 90%

95
Q

trisomy 13

A

multiple abnormalities in various organs

i. 70% of infants die within 1st 7 months of life
ii. clinically:
1. bilateral cleft lip and palate
2. micropthalmia (small eyes)
3. anopthalmia (no eyes)
4. superficial hemangioma of forehead
5. growth retardation
6. mental retardation
7. * polydactyly of hands and feet (supernumerary digits)
8. * clenching of the fist with the thumb under the fingers
9. * rocker – bottom feet
10. heart malformations
11. external genital anomalies

96
Q

turners syndrome

A

i. Female phenotype (make-up and looks)
ii. Clinically:
1. short stature
2. webbing of the neck
3. edema of the hands and feet
4. low hairline
5. chest is broad with wide-spaced nipples
6. aorta is frequently abnormal
7. sparse body hair
8. ovaries are usually not developed

97
Q

klinefelters syndrome

A

i. Genetically XXY chromosomal pattern
ii. Male phenotype – not detected until after puberty
iii. Taller than normal, wide hips, and female pubic hair distribution
iv. 50% gynecomastia – development of female breasts
v. penis appears normal but testes are smaller and hard with lack of seminiferous tubules- sterile

98
Q

tramsmitted vertically from one generation to the next

A

autosomal dominant inheritance

99
Q

cyclic neutropenia

A

i. Cyclic decrease in the number of circulating neutrophilic leukocytes (neutrophils)
ii. Neutropenia – a decrease in the number of circulating neutrophils
iii. Systemic manifestations: fever, malaise, sore throat,
iv. Oral manifestations: ulcerative gingivitis or gingivostomatitis
v. Leads to severe perio disease, loss of alveolar bone, mobility, and loss of teeth

100
Q

i. marked destruction of periodontal tissues (periodontoclasia) of both dentitions with premature loss of teeth and hyperkeratosis of the palms of the hands and soles of the feet (palmar and plantar hyperkeratosis)
ii. usually all permanent teeth are lost before age 14

A

papillon lefevre syndrome

101
Q

i. Component of several inherited syndromes
ii. Generally develops early in life and within a few years the teeth are completely covered ( normal healthy looking gingiva)
iii. Extensive gingival enlargement leads to protrusion of the lips

A

gingival fibromatosis

102
Q

increased distance between the eyes

A

ocular hypertelorism

103
Q

cherubism

A

i. Progressive bilateral facial swelling that appears between 1.5 – 4.5 years
ii. Ocular hypertelorism – increased distance between the eyes
iii. Radiographically – “soap - bubble” in ascending ramus of the mandible
iv. Bone lesions interfere with tooth development and eruption
v. Facial deformity can remain for life

104
Q

chondroectodermal dysplasia

A

i. Dwarfs because of distal shortening of the extremities
ii. 1/3 of these patients are mildly retarded
iii. hands show polydactyly
iv. fingernails/toenails are hypoplastic and deformed
v. 50% have congenital heart defects
vi. teeth appear conical in shape

105
Q

i. Cranium develops a mushroom shape because the fontanelles remain open which makes the face appear small
ii. Skull radiographs reveal the open fontanelles
iii. Paranasal sinuses are lacking
iv. Neck is long and narrow because of unilateral or bilateral aplasia or hypoplasia of the clavicles
1. allows patients to approximate their shoulders to the midline because of this alteration
v. many supernumerary teeth

A

cleidocranial dysplasia

106
Q

gardners syndrome

A

i. Prescence of osteomas in various bones, especially the frontal bones, mandible and maxilla
ii. Facial asymmetry
iii. Multiple odontomas can occur in the jaw bones, especially the mandible
iv. Teeth can exhibit hypercementosis
v. Prescence of intestinal polyps, which become malignant at age 30 and after

107
Q

nevoid basal cell carcinoa syndrome

A

i. Also called “Gorlin’s Syndrome”
ii. Consists of basal cell carcinomas of the skin over the nose, eyelids, cheeks, neck, arms, and trunk along with multiple adontogenic keratocysts (OKC’s)
iii. Bifurcation of the ribs
iv. Spina bifida occulta defective closure of the bone encasement of the spinal cord
v. Kyphoscoliosis – lateral curving of the spine plus a vertical curvature of the spine
1. kyphosis – “hunchback” (vertical curvature of spine)

108
Q

osteongensis imperfeca

A

(Brittle Bone Disease)

i. Defect involves collagen and results in abnormally formed bones that fracture easily
ii. Multiple bone fractures are main clinical complication
iii. Bowing of legs, curvature of the spine (kyphosis and scoliosis)
1. deformity of the skull, shortening of the arms and legs
iv. some show blue sclerae – a blue appearance of the white of the eyes
v. oral manifestations – dentinogenesis imperfecta
1. crowns, roots, and pulp chambers are generally smaller than normal and appear opalescent or translucent

109
Q

i. Occurs in about 1 in 800 births
ii. Most frequent syndrome is cleft lip-palate and congenital lip pits
1. lip pits are located near the

A

clefting palate or lip

110
Q

i. Multiple capillary dilations of the skin and mucous membranes
1. skin of face has numerous spider-like telangiectases of lips, eyelids, and nose
2. frequent and sometimes serious nose bleeds (epistaxis)

A

hereditary hemorrhagic telangiectasia

111
Q

i. Multiple neurofibromas appear as papules and growths of various sites
ii. Malignant transformations in 3 – 15% of patients
iii. Café au lait pigmentation of the skin is present from the 1st decade of life in 90% of these patients
1. Café au lait usually precedes the development of the neurofibromas

A

von recklinghauses disease neurofibromatosis

112
Q

i. Multiple melanotic macular pigmentations of the skin and mucosa which are associated with GI polyposis

A

peutz jeghers syndrome

113
Q

i. Clinically presents as a white, corrugated, soft, folding oral mucosa
ii. Buccal mucosa affected and is bilateral

A

white sponge nevus

114
Q

• Amelogenesis Imperfecta

i. Group of inherited conditions affecting the enamel of the teeth and having no associated systemic defects

A
  1. Type I: Hypoplastic
    a. Characterized by tooth enamel that does not develop to a normal thickness because of failure of the ameloblasts to lay down enamel matrix properly
    b. Seven varieties with following clinical presentation:
    i. Pitted local, smooth, rough, and enamel agenesis
    ii. Pits occur on labial and buccal surfaces (most common)
  2. Type II: Hypocalcified
    a. Characterized by an enamel of normal thickness that is poorly calcified
    b. 2 varieties:
    i. autosomal dominant
    ii. autosomal recessive – more severe in its clinical manifestations
  3. teeth present a yellow to orange enamel that is very soft and rapidly lost, leaving exposed dentin
  4. radiographically – “moth eaten” appearance
  5. cervical enamel is better calcified and remains on the crown
  6. associated with anterior open bite
  7. Type III: Hypomaturation
    a. Characterized by an enamel of mottled appearance but normal thickness
    b. Composed of large amounts of enamel matrix, and therefore the enamel is softer than normal
    c. Enamel chips easily from the crown
    d. * “snow-capped” type – occlusal 1/3 of the tooth affected
  8. Type IV: Hypoplastic – Hypomaturation
    a. Characterized by its association with taurodontic teeth
    b. Thin enamel is yellow to brown and pitted
    c. Radiographically – radiodensity similar to dentin, single rooted teeth have large pulp chambers
115
Q

dentiogenesis imperfecta

A
  • 3 types: one type is associated with osteogenesis imperfecta
  • Dentinogenesis Imperfecta type II
    i. Known as hereditary opalescent dentin
    ii. Teeth have bulbous crowns with color variations
    iii. 1 teeth affected more; * (radiographically – NO pulp canal or chamber
116
Q

dentin dysplasia

A
  • Type I, Radicular Dentin Dysplasia
    i. Characterized by teeth with normal crowns and abnormal roots
    ii. Radiographically – total or partial lack of pulp chambers and root canals with shortened and blunted roots
    iii. Premature exfoliation
    iv. Periapical cysts can be associated with teeth
  • Type II, Coronal Dentin Dysplasia
    i. 1 dentition characterized by translucent teeth with an amber color
    1. radiographically – lack of pulp chambers and small root canals
    ii. permanent teeth present with normal crown formation and color
    1. radiographically:
    a. single rooted teeth pulp chambers are “thistle-shaped”
    b. molars have pulp chambers that are “bow-tie” shaped
117
Q

i. Females
ii. * most severe form of ectodermal dysplasia
iii. major components are:
1. hypodontia – partial anodontia
2. hypotrichosis – less than normal amount of hair
3. hypohidrosis – abnormally diminished secretion of sweat
4. born without lanugo – body hair present at birth
5. some die from hyperthermia – greatly increased body temperature – after exposure to the sun or heavy exercise
iv. lack hair – no eyebrows or eyelashes
v. crowns are conical in shape

A

hypohiidrotic ectodermal dysplasia

118
Q

hypophosphatasia

A

i. Decrease in serum alkaline phosphatase levels with increased urinary and plasma levels of phosphoethanolamine
ii. Alkaline phosphatase – calcification of bone and cementum
iii. Agenesis or abnormal formation of cementum
1. premature exfoliation of 1 teeth
iv. * improper formation of mature bone
1. bowed legs and fractures

119
Q

i. Low serum levels of phosphorus, rickets (vitamin D deficiency)
ii. Radiographically – large pulp chambers with very long pulp horns

A

hypophosphatemia vitain d resistant rickets

120
Q

i. Small, peg shaped max. lateral incisors

ii. 1 – 3% of population

A

pegged laterals

121
Q

i. “bulls-teeth”
ii. most frequent among Native Americans
iii. Normal crowns with short roots

A

taurodontism