Ch14 Bone Path (Part V) Flashcards

1
Q

_______ are benign tumors composed of mature compact or cancellous bone.

A

Osteomas

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2
Q

They may Osteomas arise in what three areas?

A
  1. Surface: periosteal,peripheral, or exophytic osteomas 2. Medullary bone (endosteal or central osteomas) 3. Extraskeletal osteomas ( muscle or the dermis of the skin (osteoma cutis)
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3
Q

Common palatal tori, mandibular tori, and buccal exostoses are NOT considered to be ______, although they are histopathologically identical

A

osteomas

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4
Q

Osteomas: age, location

A

adults, mandibular body / condyle…LINGUAL surface adjacent to premolars and molars

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5
Q

condylar hyperplasia vs condylar osteoma: osetoma is typically is ________, whereas a hyperplastic
condyle usually retains its original shape

A

lobulated

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6
Q

________ osteomas are even more common than gnathic lesions.

A

Paranasal sinus

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7
Q

Smaller endosteal osteomas are difficult, if not impossible, to differentiate radiographically from _______, focal chronic sclerosing osteomyelitis, or idiopathic osteosclerosis..The true nature of these osteomas can be confirmed only by documentation of ________.

A

condensing osteitis…continued growth

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8
Q

________ osteomas are composed of normal-appearing, dense bone with minimal marrow

A

Compact

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9
Q

________ osteomas are composed of bony trabeculae and fibrofatty marrow

A

Cancellous

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10
Q

Gardner syndrome represents a variant of _________

A

familial adenomatous polyposis

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11
Q

Both classical familial adenomatous polyposis and theGardner syndrome variant are HIGHLY penetrant _______ disorders caused by mutations in the _______ tumor suppressor gene on chromosome ____.

A

Autosomal Dominant….adenomatous polyposis coli (APC)…5q21

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12
Q

What % of Gardner syndrome pts are new mutations?

A

30%

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13
Q

Garnder syndrome generally refers to cases in which the _________ manifestations are especially prominent

A

extraintestinal

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14
Q

The estimated frequency of Gardner syndrome ranges from about _________ live births.

A

1 : 8,300 to 1 : 16,000

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15
Q

GARDNER Syndrom: Colorectal polyps typically develop by the _____ decade of life

A

2nd

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16
Q

What are the # main maifestations (think by body system) of Gardner disease?

A
  1. colorectal (GI) polyps 2. osteomas 3. dental abnormalities (odontomas, supernum, impacted teeth) 4. skin lesions (epidermoid cysts, lipomas, NFs, fibromas, Leios 5. DESMOID TUMORS 6.thyroid carcinoma 7.adrenal adenoma/carcinoma 8.hepatoblastoma 9.pancreatic adenoca 10. nasopharyengeal angiofibrom 11. brain tumos 12. congenital hypertrophy of the retinal pigment epithelium
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17
Q

What are the 3 dental manifestations of Gardner Syndrome?

A

1)odontomas 2) supernumerary teeth 3) impacted teeth

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18
Q

What % of pts with Gardner Syndrome develop DESOMID tumors? What gender predilection? What scenario do they typically present in?

A

12-18%…female…forms in abdominal scar status post colectomy

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19
Q

What % of Gardner Syndrome (FAP) pts have congenital hypertrophy of the retinal pigment epithelium?

A

58-88%

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20
Q

Without treatment, 50% of Gardner Syndrome pts will develop colorectal cancer by age ___ and 100% by age ___

A
  1. fuck. 50..fuck
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21
Q

_____ exhibits a unique tumor nidus with a high concentration of peripheral nerves and prostaglandins

A

osteoid osteoma

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22
Q

osteoid osteoma exhibits more limited growth potential than _________

A

osteoblastoma

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23
Q

Classically, the distinction depends on lesion size, with osteoid osteomas measuring less than a threshold of _____ in diameter, whereas osteoblastomas are larger.

A

1.5 cm or 2 cm

24
Q

cytogenetic studies have identified recurrent alterations on the long arm of chromosome __ in some osteoid osteomas and aggressive osteoblastomas.

25
Osteoblastoma: location, age, gender
mandibular predilection, with most examples involving the posterior regions. Approximately 85% of gnathic osteoblastomas occur before 30 years of age, and there is a slight female predominance.
26
Unlike the pain associated with ________, the pain associated with ________ usually is not relieved by NSAIDs
osteoid osteoma....osteoblastoma
27
A small subset of osteoblastomas (aggressive osteoblas- tomas) is characterized by atypical histopathologic features and locally aggressive behavior. These tumors usually occur in patients older than ___ years.
30
28
Osteoid osteoma patients diagnosed during the ____ and ____ decades of life (mean age ___ years)
2nd-3rd...25
29
The most prominent clinical symptom of a ________ is pain that is most severe at night and alleviated by NSAIDs.
osteoid osteoma
30
Differentiation between aggressive osteoblastoma and low-grade _________ may be very difficult, although infiltrative growth, marked cytologic atypia, and atypical mitotic figures favor the latter.
osteosarcoma
31
What is the only TRUE neoplasm of cementum? What does it resemble histologically?
Cementoblastoma..osteoblastoma
32
In particular, almost 50% of cases of cementoblastoma involve which tooth?
mandibular first permanent molar
33
The neoplasm predominantly affects ______ patients, with about 50% arising by ___ years of age and 75% by ___ years
young..20..30
34
Are cementoblastomas symptomatic?
Pain and swelling are present in approximately two-thirds of reported cases.
35
Interesting...cementoblastomas are considered to have a low recurrence rate, but one extensive study showed a __% risk
22%
36
Chondromas frequently harbor somatic mutations in the ______ gene.
isocitrate dehydrogenase 1 (IDH1)
37
Approximately 50% of chondromas are diagnosed in the ____,___, and ____ decades of life, and there is a female predilection.
second, third, and fourth
38
Chondroma: Most gnathic examples occur in the ______ or anterior _____ of adult patients
Most gnathic examples occur in the condyle or anterior maxilla of adult patients
39
What are the two conditions associated with chondromas?
Ollier disease (sporadic chondromatosis with unilateral tendency)...Maffucci syndrome (sporadic chondromatosis with soft tissue angiomas)
40
Microscopic distinction between a chondroma and a low-grade ________ of the jaws may be difficult
chondrosarcoma
41
chondromyxoid fibroma: cytogenetic studies are limited but have detected frequent abnormalities in chromosome ___
6
42
Among reported jaw lesions chondromyxoid fibroma, the average age at diagnosis is ___ years
28
43
Approximately three-quarters of chondromyxoid fibromas occur in the ________.
mandible
44
chondromyxoid fibromas: jaw tumors, the overall recurrence rate is about ___%.
10%
45
____________ typically proceeds through three stages. In the first stage, cartilaginous or osteocartilaginous nodules develop in the synovial lining. Subsequently, these nodules begin to detach, with some lying free in the joint space and others remaining in the synovial membrane. In the final stage, the nodules are found only in the joint space
Synovial chondromatosis
46
In synovial chondromatosis... The detached particles are called ________
loose bodies
47
Synovial chondromatosis of the TMJ occurs over a broad age range (12 to 82 years), with a peak in the ____ and ____ decades of life...gender predilection?
fourth and fifth...female
48
Within the TMJ, synovial chondromatosis primarily involves the ________ compartment, although involvement of the _______ compartment also is possible.
superior...inferior
49
_________ is considered the soft tissue counterpart of soft tissue fibromatosis (desmoid tumor)
desmoplastic fibroma
50
Most desmoplastic fibromas of bone arise in patients younger than ___ years old... mean of approximately ___ years.
30...14
51
More than 80% of desmoplastic fibromas affect the _______—most often the _____
mandible..posterior body
52
Although the desmoplastic fibroma is a benign tumor, it often behaves in a locally ________ fashion
agressive
53
The recurrence rate of a desmoplastic fibroma is approximately ___% for lesions treated by curettage or enucleation, compared with about ___% for those treated by resection.
30% .... 5%
54
It may be very difficult to distinguish desmoplastic fibroma of bone from well-differentiated ________
fibrosarcoma
55
Osteosarcomas may be classified as _______ (arising within the medullary cavity), _______ (arising in the juxtacortical region), or, vary rarely, ________ (arising within soft tissue). The vast majority of cases are ______.
central...surface..extraskeletal....central