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Neville and Damm > Ch14 Bone Path (Part III) > Flashcards

Ch14 Bone Path (Part III) Flashcards

1
Q

This is ridiculous…what are the 5 alternate names for Simple Bone Cyst?

A

1.Traumatic Bone Cyst 2. Hemorrhagic bone cyst 3. Solitary bone cyst 4. idiopathic bone cavity 5. unicameral bone cyst

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2
Q

Why is a simple bone cyst not really a cyst?

A

It lacks a lining, thus really a pseudocyst and not a true cyst

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3
Q

The trauma-hemorrhage theory for the cause of a simple bone cyst is that a ________ forms but does not organize and repair, just liquefies

A

hematoma

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4
Q

Simple bone cysts have been reported in almost EVERY bone in the body..but what are the two most common sites?

A

proximal humerous and proximal femur

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5
Q

What is the most common location of a simple bone cyst that shows up in the jaw?

A

marked mandibular predominance..PM, molar, and symphyseal regions

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6
Q

Simple bone cyst: age? gender?

A

young, peak in 2nd decade…no gender preference (extragnathic has male predilection)

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7
Q

What % of simple bone cysts in the jaw present with swelling?

A

20%

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8
Q

What is a HIGHLY SUGGESTIVE (but not clearly diagnostic) feature of a simple bone cyst on a radiograph?

A

scalloping between tooth roots

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9
Q

What may arise in conjunction with a simple bone cyst? What age and gender demographic is this most likely?

A

cemento-osseous dysplasia…older females

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10
Q

Simple bone cyst: in lieu of an epithelial lining there may be what 2 features?

A
  1. a thin vascular connective tissue membrane adjacent to the bone 2. a thickened myxofibromatous proliferation
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11
Q

Clinically, when opened 1/3 of simple bone cysts will be ______ and 2/3 will have _______

A

1/3 empty…2/3 small amounts of serosanguinis fluid

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12
Q

Interesting…How are EXTRAGNATHIC simple bone cysts treated? What is the recurrence rate?

A

aggressively: curettage, cryosurgery, decompression, intralesional steroid injection, bone substitiute/marrow injection/grafting…29% (range 12-48%)

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13
Q

How are simple bone cysts of the jaw treated? Recurrence rate?

A

surgical exploration and curettage, usually stimulates bone regeneration..1-2% (some report as high as 27%)

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14
Q

What increases the chance of recurrence for a simple bone cyst in the jaw? (2)

A

multiple cysts and assoc w COD

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15
Q

How long does it take for simple bone cysts to resolve post enucleation?

A

12-17 months post surgery

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16
Q

The ___________ is an intraosseous accumulation of variable-sized, blood-filled spaces surrounded by cellular fibrous connective tissue and reactive bone. Because the lesion lacks an epithelial lining, it represents a _______.

A

aneurysmal bone cyst… pseudocyst

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17
Q

What % of aneurysmal bone cysts form in association with other lesions?

A

20-30%

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18
Q

GENETICS for aneurysmal bone cysts indicate it is a neoplastic process…what gene is involved? Chromosome?

A

Ubuiquitin-specific protease 6 (USP6) (or Tre-2/TRE17)…Chromosome 7p13

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19
Q

Where are the typical site for ABCs? Age? Gender? What % of cases involve the jaws and what age?

A

long bones and vertebrae, pts younger than 30 then vertbre…no sex predilection (possible slight female predilection).. 2% and peak in 2nd decade

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20
Q

When an ABC shows up in the jaw, where is it most likely? Where does it not show up?

A

Common in ascending ramus and posterior body, but NOT in condylar or coronoid process

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21
Q

What is the MOST COMMON clinical manifestation of an ABC?

A

RAPID swelling (pain is variable, paresthesia, crepitus noted variably)

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22
Q

ABC: Radiographic buzzwords…

A

Balooning or “blow out” distension of the affected bone

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23
Q

ABC: Buzzword for clinical appearance during enucleation…

A

“blood soaked sponge” (venous-like bleeding)

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24
Q

ABC Histology: characterized by ______ that LACK ______…sourrounding these structures is fibroblastic tissue with what three features?

A

blood-filled spaces…lack endothelial or epithelial lining…giant cells, osteoid, and woven bone

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25
Q

What two entities are most commonly associated with ABCs when discovered histologically?

A

fibro-osseus lesions or giant cell granulomas

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26
Q

How long does an ABC take to heal post enucleation?

A

6mo to 1 year

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27
Q

What is the recurrence rate for ABCs?

A

13% (has been reported between 8-70% lol)

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28
Q

What are the 4 possible categories (not specific entities) of fibro-osseuos lesions?

A
  1. developmental (hamartomatous) 2.reactive 3.dysplastic 4.neoplastic
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29
Q

What are the three specific types of fibro-osseous lesions of the jaw?

A

1.Fibrous Dysplasia 2.Cemento-osseuos dysplasia (focal, periapical, florid) 3. ossifying fibroma

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30
Q

What TYPE of lesion is fibrous dysplasia?

A

developmental tumorlike condititon lol

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31
Q

Fibrous dysplasia is characterized by replacement of normal bone by a proliferation of _________ with irregular ______.

A

cellular fibrous connective tissue….bony trabeculae

32
Q

What is the distinguishing genetic factor in fibrous dysplasia, thus separating it from OF or COD?

A

mutations in the GNAS gene

33
Q

Fibrous dysplasia: This SPORADIC condition results from _______, activating mutations in the ____ gene, which encodes the ______ subunit of a stimulatory __ protein.

A

post zygotic…GNAS gene..alpha….G

34
Q

In some cases of fibrous dysplasia, involvement of multiple bones may occur in conjunction with _______ and ______ abnormalities.

A

cutaneous…endocrine

35
Q

Buckle in: Fibrous Dysplasia–TIMING of the GNAS mutation: During EARLY embryonic development, mutation of a pluripotent stem cell can cause abnormalities in multiple cell types, including WHAT 3 cell types?

A

osteoblasts, melanocytes, and endocrine cells

36
Q

Buckle in: Fibrous Dysplasia–TIMING of the GNAS mutation: if the mutation occurs in a skeletal progenitor cell in a LATER stage of embryonic development, then only _______ will be affected.

A

osteoblasts

37
Q

Buckle in: Fibrous Dysplasia–TIMING of the GNAS mutation: if the mutation occurs during POSTNATAL
life, then ________ in only a SINGLE BONE will be affected

A

osteoblasts

38
Q

Buckle in: Fibrous Dysplasia–the PARENTAL origin of the mutated GNAS allele may affect the phenotype, in certain cell types (such as pituitary somatotrophs) genomic imprinting results in expression of only the _______ allele

A

MATERNAL

39
Q

Buckle in: Fibrous Dysplasia–Constitutive activation of _______ signaling IMPAIRS ______ differentiation in skeletal progenitor cells, STIMULATES _______ production in melanocytes, and causes HYPERPLASIA and HYPERFUNCTION of various _______ cell types.

A

G-protein…osteoblastic….melanin…endocrine

40
Q

Fibrous Dysplasia: mutated osteoblasts overexpress ______, which stimulates osteoclastic activity and may contribute to bone lesion expansion

A

interleukin (IL)-6

41
Q

What is more common: monostotic or polyostotic fibrous dysplasia?

A

monostotic…one bone affected in 70-85% of pts

42
Q

Monostotic fibrous dysplasia: age? sex? sites?

A

2nd-3rd decades..equal frequency…craniofacial bones, ribs, femur, tibia

43
Q

When monostoic fibrous dysplasia occurs in the jaw which jaw is most affected? What region of that jaw?

A

posterior maxilla

44
Q

What is typically the clinical indication of fibrous dysplasia?

A

PAINLESS unilateral swelling

45
Q

Fibrous dysplasia: typically rapid or slow onset? What happens to the teeth?

A

TYPICALLY slow onset (can be fairly rapid though)…adjacent teeth can be DISPLACED, but usually remain FIRM (so NO mobility)

46
Q

The classic radiographic finding of fibrous dysplasia is “________” with ______ defined margins

A

ground glass opacification…POORLY defined margins

47
Q

What is the more appropriate term for when fibrous dysplasia extends beyond the maxilla and into adjacent bones?

A

crainiofacial fibrous dysplasia

48
Q

Mandibular lesions of fibrous dysplasia often exhibit __________ expansion and bulging of the _______ border. There may be SUPERIOR displacement of the _________.

A

buccal - lingual….inferior…IA canal

49
Q

PAs of fibrous dysplasia show _______ of the PDL and ill-defined lamina dura

A

NARROWING

50
Q

What syndromes are associated with polyostoic fibrous dysplasia?

A

1) Jaffe-Lichtenstein syndrome 2) McCune-Albright Syndrome 3) Mazabraud

51
Q

What age is polyostoic fibrous dysplasia typically diagnosed? Gender? Up to __% of the entire skeleton can be affected…

A

BEFORE age 10…Female predilection…75%

52
Q

What are the three buzzwords for finding polyostoic fibrous dysplasia in the proximal FEMUR?

A

coxa vara, shepherd’s crook deformity, or hockey stick deformity

53
Q

Polyostoic fibrous dysplasia: WHAT blood test finding is common?? What is the cause?

A

Hypophosphatemia….caused by renal phosphate wasting

54
Q

Polyostoic fibrous dysplasia: Hypophosphatemia appears to be related to the release of ________ by the affected bones.

A

fibroblast growth factor 23 (FGF23)

55
Q

__________ is characterized by polyostotic fibrous dysplasia and café au lait (coffee with milk) pigmentation

A

Jaffe-Lichtenstein syndrome

56
Q

_________ is characterized by polyostotic fibrous dysplasia, café au lait pigmentation, and multiple endocrinopathies

A

McCune-Albright syndrome

57
Q

________ is characterized by fibrous dysplasia and intramuscular myxomas

A

MA-ZA-BRAUD syndrome

58
Q

What is the difference between cafe au lait pigmentation in fibrous dysplasia vs neurofibromatosis?

A

FD = irregular margins (coast of Maine)…. NF = smooth borders (coast of California)

59
Q

Since we already know that McCune-Albright syndrome is set apart by the presence endocrineopathies…what IS the #1 endocrineopathy seen? What gender?

A

sexual precocity in females (menses, breast dev, pubic hair present in first months/years of life)

60
Q

Since we all know that sexual precocity in females is the most common endocrinopathy in McCune-Albright patients, what are the 4 other possibilities?

A
  1. hyperthyroidism 2. hyperparathyroidism 3. hypercortisolism 4.excess growth hormone
61
Q

craniofacial fibrous dysplasia mainly occurring in _________, investigators have reported various dental anomalies, including tooth displacement, oligodontia, enamel hypoplasia, enamel hypomineralization, taurodontism, and retained deciduous teeth.

A

McCune-Albright syndrome

62
Q

Fibrous dysplasia histology: irregularly shaped trabeculae of ______ (_____) bone in a cellular ______ stroma.

A

immature (woven)…fibrous

63
Q

Fibrous dysplasia histology: At the PERIPHERY, the lesional bone FUSES with normal bone, without a ______ or line of demarcation.

A

capsule

64
Q

Fibrous dysplasia histology: The abnormal bony trabeculae tend to be thin and disconnected, with curvilinear shapes likened to “________”.

A

Chinese characters

65
Q

Fibrous dysplasia histology: Osteoblastic ______ is usually ABSENT or minimal, and peritrabecular ______ (artifactual retraction of the stromal from the bony trabeculae) is COMMON

A

rimming..clefting

66
Q

Fibrous dysplasia histology: In addition, tiny calcified _______ rarely may be seen but are never numerous.

A

spherules

67
Q

Fibrous dysplasia histology: In LATER stages, the WOVEN bone is replaced by ______ bone with roughly PARALLEL trabeculae

A

LAMELLAR

68
Q

Fibrous dysplasia histology: what cyst can be seen secondarily?

A

ANEURYSMAL BONE CYST

69
Q

What is a generalized difference in pattern between fibrous dysplasia and ossifying fibroma / COD?

A

FD: MONOTONOUS pattern vs OF/COD=HAPHAZARD

70
Q

What are two microscopic variations in fibrous dysplasia?

A

PAGETOID (thick, interconnected bone trabeculae) and HYPERCELLULAR (parallel bone trabeculae with numerous osteocytes and polarized osteoblastic rimming)

71
Q

Fibrous dysplasia tends to stabilize upon WHAT EVENT??, and spontaneous regression even has been reported in a few cases.

A

skeletal maturation

72
Q

Fibrous dysplasia: ___% to ___% of patients show some REGROWTH after surgical debulking…risk is greater for WHICH age group?

A

20-50%..greater risk of regrowth for YOUNG pts

73
Q

More studies needed, but: fibrous dysplasia and which blood marker to predict recurrence?

A

Serum alkaline phosphatase

74
Q

Fibrous dysplasia: what treatment is CONTRAINDICATED?

A

radiation therapy…risk of post rad sarcoma

75
Q

What is the risk of fibrous dysplasia transforming to osterosarcoma? What 3 situations INCREASE the risk?

A

less than 1%…1) radiation therapy 2)McCune-Albright Syndrome 3)MAZA-BRAUD syndrome

Neville and Damm (72 decks)

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