ch 14 Bone Pathology Flashcards
Most common type of inherited bone disorder
Osteogenesis imperfect
Osteogenesis imperfecta is an impairment of what tissue maturation
type I collagen
What parts of the body are affected by OI
bone Dentin
Sclera Ligaments Skin
What is the bone character in O.I.
thin cortex, fine trabeculations, diffuse osteoporosis
How will bone heal in O.I.
heal, but with abundant callus
What are the radiographic hallmarks of O.I.
osteopenia Bowing
Angulation/deformed long bones Multiple fractures
Wormian skull bones
What are wormian bones
10 or more sutural bones that are 6X4mm in diameter or larger and mosaic patterned, but not specific to O.I.
O.I. can appear dentally identical to what other disorder
Dentinogenesis imperfect
How will the dentition look in both Osteogenesis Imperfecta and Dentinogenesis Imperfecta
blue to brown opalescent teeth
What should you call teeth that have the characteristic brown or blue opalescence? Should it be D.I. or O.I outright
No, just opalescent teeth until determined if only D.I.
What is the characteristic malocclusion in O.I. and why
1.) Class III due to maxillary hypoplasia
What is the most common, mildest form of O.I.
Type I
What is the most severe form of O.I.
Type II
Other physical characteristics of O.I.
Blue sclera
Hypoacusis(hearing loss)
Long bone & spine deformaties Joint hyperextensibility
Most severe form of O.I. noted in patients beyond
the perinatal period and has moderately severe to severe bone fragility
Type III
Type III O.I. patients die from cardiopulmonary complications from what
kyphoscoliosis = abnormal spin curvature in coronal and sagittal plane
O.I. type associated with mild to moderate bone fragility
Type IV
What happens to bone throughout life in O.I.
Cortical bone is attenuated
Reduced bone matrix production
Bone immature throughout life
Woven bone never transforms to lamellar
What is the primary goal of O.I. treatment
No cure, goal to improve symptoms (bisphosphonates later in life)
rare hereditary skeletal disorder characterized by marked increase in bone density
Osteopetrosis
Osteopetrosis cause
defect in osteoclast function
What causes the thickening of cortical bone and sclerosis of cancellous bone
defective osteoclast function + continued osteoblast function + endochondral bone formation
2 types of osteopetrosis
Infantile/Malignant Adult/Benign
Character of Infantile/Malignant Osteopetrosis
diffusely sclerotic skeleton Marrow failure
Frequent fracture (accusations of child abuse)
Cranial nerve compression (blind/deaf/facial paralysis)
Facial character of Infantile/Malignant Osteopetrosis
broad face Hypertelorism
Snub nose
Frontal bossing
Tooth eruption in Infantile/Malignant Osteopetrosis
Delayed
Common complication of tooth extraction in Infantile/Malignant Osteopetrosis
osteomyelitis
What are common in Infantile/Malignant Osteopetrosis even though bone is dense
pathologic fractures
How will bone look radiographically in Osteopetrosis
increased radiographic density with loss of distinction between cortical and cancellous bone
O.I with opalescent teeth normally show what
severe attrition
Which part of skeleton has significant sclerosis in Adult/Benign Osteopetrosis
axial skeleton, long bones not as sclerotic
When is Adult/Benign Osteopetrosis diagnosed and how
dental radiographs by increased radiopacity of medullary portions of bone
How do patients that are symptomatic with Adult/Benign Osteopetrosis present
with pain
Most common treatment with best prognosis for Infantile Osteopetrosis
bone marrow transplant
Secondary treatment for Osteopetrosis that decreases bone mass, decrease infection prevalence, and lower frequency of cranial nerve compression
Interferon gamma 1b with calcitriol
Cleidocranial dysplasia affects what type of bone formation
endochondral ossification
Most prominent abnormalities of Cleidocranial dysplasia affect what bones
clavicles and skull
Absent/hypoplastic clavicles with underdeveloped neck muscles allowing shoulders to be approximated in front of the chest is characteristic of
cleidocranial dyplasia
Physiologic appearance of pt with Cleidocranial dysplasia
short stature
Large head
Prominent frontal and parietal bossing Hypertelorism
Broad nasal base and depressed nose bridge
Skull bone character in Cleidocranial dysplasia
delayed or never closed fontanels with sutures featuring wormian bones
Palate character in Cleidocranial dysplasia patient
narrow, high arched palate
Increased prevalence cleft palate
Teeth character of Cleidocranial dysplasia
prolonged deciduous retention, permanent eruption failure, numerous unerupted supernumand permanent teeth with dilacerated roots or abnormal crowns
What do Cleidocranial dysplasia teeth lack that may be associated with eruption failure
Lack of secondary cementum
Cleidocranial dysplasia mandible character on radiograph
coarse trabeculation
Narrow ascending rami
Narrow pointed coronoid process
Cleidocranial dysplasia maxilla character on radiograph
Thin zygomatic arch
Hypoplastic maxillary sinus
Profile of Cleidocranial dysplasia patient as they age
short lower face height
Acute gonial angle
Anterior incline of mandible
Prognathic
Treatment of choice for Cleidocranial dysplasia dentally
removal of primary and supernum teeth followed by exposure of permanent teeth to be extruded orthodontically
An area of hematopoietic marrow that is sufficient in size to produce an area of radiolucency that may be confused with an intraosseous neoplasm (i.e. it is not a pathologic process)
Focal Osteoporotic Marrow Defect
Demographics of Focal Osteoporotic Marrow Defect
Post menopausal females, posterior mandible, endentulous areas
Is there expansion in the mandible from Focal Osteoporotic Marrow Defect
No
How treat Focal Osteoporotic Marrow Defect
incisional biopsy for diagnosis, if it is FOMD, then no further treatment required
Focal area of increased radiodensity that is of unknown cause and cannot be attributed to any inflammatory, dysplastic, neoplastic, or systemic disorder
Focal Idiopathic Osteosclerosis
What must do to the tooth if see a radiopaque lesion at the root apex
Test Tooth vitality
If teeth are nonvital with testing then what should be the diagnosis of the radiopacity at the root apex
condensing osteitis or focal chronic sclerosis osteomyelitis
When does Focal Idiopathic Osteosclerosis begin and end
begins 1st to 2nd decade and ceases with bone maturity ~ 4th decade
Where does Focal Idiopathic Osteosclerosis normally appear
90% mandible, most common near 1st molar, but also common in 2nd PM and 2nd Molar
What should be included in the differential diagnosis if more than one area of osteosclerosis is noted
multiple osteomas associated with Gardner’s syndrome
Radiographic character of Focal Idiopathic Osteosclerosis (size, shape, rim)
radiopaque 3mm-2cm, well-defined round to irregular with no rim
Is root resorption common in Focal Idiopathic Osteosclerosis
No
Radiographic character of Focal Idiopathic Osteosclerosis
dense lamellar bone with scant fibrofatty marrow
Treatment for Focal Idiopathic Osteosclerosis
serial radiographs until growth stops, then no treatment required
What would be an indication for biopsy with focal idiopathic osteosclerosis
if cortical expansion occurs (indicates a neoplasm)
Abnormal and anarchic resportion and deposition of bone resulting in distortion and weakening of the affected bones
Paget’s Disease (Osteitis Deformans)
Paget’s disease demographics
more common in Britain, after 4th decade, Males > women, Whites > blacks
Most commonly affected bones in Paget’s
lumbar vertebrae
Pelvis
Skull
Femur
Can Paget’s jump a joint space to a previously unaffected bone
No
Can just the mandible be affected in Paget’s
No, maxilla must be involvd if mandible involved
Paget affected bone character
thickened, enlarged, weakened
Characteristic stance of Paget’s disease and why
weight bearing bones bow, resulting in Simian stance
Paget’s disease head circumference
Large head circumference
Term for extreme cases of maxillary expansion in Paget’s disease
leotiasis ossea (gross expansion of midface)
Phase I of Paget’s Disease is osteolytic or osteoblastic
Osteolytic
What is osteoporosis circumscripta
waves or fronts of progressive demineralization occur in skull in Paget’s disease
What is the cause of the increased cranial circumference in Paget’s disease
expansion of diploic table of skull
Phase II of Paget’s Disease is osteolytic or osteoblastic
osteoblastic
Descriptive term of the radiographic appearance of Phase II Paget’s Disease with patchy areas of sclerotic bone
cotton wool appearance
Character of Phase III Paget’s Disease
mixed osteolytic/osteoblastic, but no coordination between the two
What is pathognomonic for Paget’s Disease
Generalized hypercementosis
What type of root resorption is seen in Paget’s Disease
external root resorption
Change in teeth due to Osteoblastic (Phase II) of Paget’s disease
diastema
What other pathology is included in the differential when diagnosing Paget’s Disease
cement-osseous dysplasia
Consideration of Pagetoid bone
hypervascular, therefore poor hemostasis, sensitive to inflammation, high output cardiac failure (?)
Malignant bone tumor associated with Paget’s disease (because it is rare to find in someone older than 40)
Osteosarcoma
What is required for diagnosis of Paget’s disease
clinical, radiographs, lab tests (high alkaline phosphatase among other things)
What should patient be sent for once diagnosed with Paget’s disease
Bone scintography to detect skeletal involvement
What is histologically characteristic of Paget’s disease
numerous basophilic reversal lines forming mosaic or jigsaw pattern
What replaces marrow in Pagetoid bone
highly vascular fibrous connective tissue
Common treatment for the bone pain common in Paget’s disease
NSAID
What can result due to compression of cranial nerves during Phase II (osteoblastic) Paget’s
deafness, blindness
What can be prescribed to reduce bone resorption
parathyroid hormone antagonist (calcitonin or bisphosphonates)
What pagetoid bone is an osteosarcoma likely to occur in
pelvis or lower extremities
What tumors normally develop in Pagetoid bone in the craniofacial skeleton
benign and malignant giant cell tumors
Character of anterior Mandibular Central Giant Cell Granuloma
anterior can cross midline
Central Giant Cell Granuloma commonly found before what age and in what gender
before age 30, females
majority presentation of Central Giant Cell Granuloma
asymptomatic, but have swelling
Are central giant cell granulomas aggressive, fast growing, perforate cortical plate, and do they cause root resorption
No, nonaggressive slow growing with no cortical expansion and no root resoprtion (Nonaggressive Central Giant Cell Granuloma)
Can Central giant cell granulomas be aggressive and grow quickly causing perforation of the cortical plant and root resorption
Yes (Aggressive Central Giant Cell Granuloma)
Central Giant Cell Granulomas can be what flavor (unilocular or multilocular)
Either unilocular or multilocular
Central Giant Cell Granulomas are radiopaque or radiolucent
radiolucent with well-defined rim, but rim is not corticated (does not have a definite white line)
What is a differential diagnosis for a multilocular radiolucent lesion of the jaw
Central Giant Cell Granuloma
Ameloblastoma
Unilocular radiolucent lesion of the jaw differential diagnosis
Central Giant Cell Granuloma
Periapical Granuloma
Cyst
Giant Cell Granulomas are histopathologically identical to what tumor associated with hyperparathyroidism
brown tumors
Common histology in all Central Giant Cell Granulomas
multinucleated giant cells in background of ovoid to spindle mesenchymal cells & round Macrophages
Before preceeding with treatment of Central Giant Cell Granuloma, what disease must be first ruled out
hyperparathyroidism (because brown tumor of hyperparathyroidism is histologically identical to Central Giant Cell Granuloma)
What disease should be investigated in a child that has multiple foci of apparent central giant cell granuloma and why
Cherubism. Because Central Giant Cell Granuloma normally occurs as 1 lesion while Cherubism is bilateral lesions presenting commonly at age 2-5
Treatment of nonaggressive Central Giant Cell Granuloma
Curettage (has 15%-20% recurrence)
3 alternatives to surgery for Aggressive Central Giant Cell Granuloma
Corticosteroids
Calcitonin
Interferon alpha-2a
Where do giant cell tumors normally occur in the body
epiphysis of long tubular bones
Rare developmental condition of painless bilateral expansion of the posterior mandible involving the angles and ascending rami
Cherubism
Cherubism: genetic or random mutation
can be autosomal dominant genetic transfer or spontaneouls mutation
Cherubism usually manifests at what age range
2-5 years
Besides the chubby cheeks, what is the other physiologic feature that leads to the naming of Cherubism
“eyes upturned to heaven) appearance due to infraorbital rim and orbital floor involvement tilting eye up while pulling lower lid down exposing more sclera below iris
When will the lesion regress
stabilize and slowly regress at puberty
What is the diagnostic (pathognomonic) radiography for Cherubism
multilocular expansile radiolucency with a bilateral symmetrical presentation
Side effects of the bilateral symmetrical jaw expansion of cherubism
displaced teeth, delayed eruption, impaired mastication and speech
What is contraindicated for Cherubism treatment and why
No radiation therapy, rick of post irradiation sarcoma
Benign empty or fluid-filled cavity within bone that is devoid of epithelial lining
simple bone cyst
Where do Simple Bone Cysts commonly occur in the body
long bone
Where do simple bone cysts commonly occur in the head and in what age range
mandible, between 10-20 years
What is the radiographic appearance of Simple bone cyst (radiolucent or opaque, uniloc or multiloc)
unilocular well-defined radiolucency
What is highly suggestive of simple bone cysts radiographically
superior border of cyst scallops up and down between adjacent tooth roots
Are teeth adjacent to Simple bone cysts vital or non-vital
vital
Is there root resorption with simple bone cyst
no root resorption
Does a simple bone cyst have an epithelial lining
no, may have a thin vascular connective tissue membrane adjacent to the bone
Treatment for Simple bone cyst
curettage and enucleation with submission of any recovered tissue to pathology
How will the walls of the simple bone cyst appear on surgical exploration
smooth and shiny
What will happen to simple bone cyst after curettage and enucleation
bony fill within 6 months
Intraosseous accumulation of variable-sized, blood-filled spaces surrounded by cellular fibrous connective tissue that is often admixed with trabeculae of reactive woven bone
Aneurysmal bone cyst
Aneurysmal bone cysts commonly located
shaft of long bone
Vertebral column
Patient age normal for aneurysmal bone cyst in long bones and vertebral column
<30
Patient age for aneurysmal bone cyst in jaw
children and young adults, mean age 20
Aneurysmal bone cyst in head normally occur in what region of what bone
posterior segments of mandible (mandibular molar region)
Most common clinical symptom of aneurysmal bone cyst
rapid facial swelling
What is the radiographic appearance of an aneurysmal bone cyst
unilocular or multilocular, radiolucent, marked cortical expansion and thinning
Treatment of aneurysmal bone cyst
enucleation and curettage, can look like a blood soaked sponge
What does the aneurysmal bone cyst do to the surrounding bone
blow out or balloon
Does the aneurysmal bone cyst have an epithelial lining
No, just spaces filled with clotted blood surrounded by fibroblastic tissue with gian cells and trabeculae of osteoid
Odontogenic neoplasm of cementoblasts
cementoblastoma
Most common area for cementoblastoma
mandibular molar and mandibular premolar region
What age is cementoblastoma commonly found
late teens to 30’s
What has occurred in a cementoblastoma that does not allow you to follow the root outline to the apex
root resorption has occurred and lesion is fused with tooth
What is fused onto the root surface
sheets or thick trabeculae of mineralized cementum or osteocementum
what is the cell type that lines the trabeculated sheets attached to the tooth root in cementoblastoma
blast cells
Radiographic appearance of cementoblastoma
radiopaque mass fused to one or more tooth roots surrounded by thin radiolucent rim
Treatment of Cementoblastoma
surgical extraction of tooth and attached mass, does not recur
Primary malignant tumor of bone composed of small undifferentiated round cells of questionable etiology
Ewing’s sarcoma
What is thought to be the origin of the round cells of Ewing’s sarcoma
neuroectodermal
what are the top three most common osseous malignancies (in order)
- Osteosarcoma
- Chondrosarcoma
- Ewing’s Sarcoma
Demographics of Ewing’s sarcoma
20’s, white, male
Most common symptom of Ewing’s sarcoma
pain and swelling
What does the tumor or Ewing’s sarcoma do to the cortical bone and what results
perforates cortical bone and soft tissue mass lies over the affected bone
Majorityu of the cases contain what in the cytoplasm of the Ewing’s sarcoma tumor cells
glycogen
Treatment for Ewing’s sarcoma
chemotherapy, multidrup chemotherapy to address micrometastasis, surgery. No radiation due to post irradiation sarcoma risk
Most common form of cancer involving bone
Metastatic Carcinoma
Carcinomas of what organs or tissues give rise to the majority of gnathic metases
Breast, Lung, Prostate, Colon, Thyroid, Renal
How do the primary carcinomas metastasize to the jaw
hematogenous route
Majority of metastatic tumors of the jaw are what type and occure where and in what age
intraosseous, posterior body of mandible, condyle, anterior maxilla, 5th – 7th decade
In general, metastatic tumors of the jaw are found in areas of what (think of the route of transmission)
areas of red marrow
Symptoms found in metastatic tumors of the jaw
pain, swelling, mass, tooth mobility, paresthesia, numb chin syndrome
In general, the metastatic lesion in the jaw appears how radiographically
radiolucent with moth eaten appearance and possible PDL widening
Metastatic process in jaw particular to breast and prostate cancer as primary site
osteoblastic
If the metastasis is osteoblastic, how will it appear radiographically
radiopaque or mixed density
In the 25% of the metastases to the oral cavity that don’t go to bone, where do they go
soft tissue: gingival or dorsal tongue
2 things a metastatic tumor to the Oral cavity can be confused for
pyogenic granuloma
PGCG
Fibroma
Why is the prognosis dismal if it is a metastatic tumor or the jaw
because metastasis automatically places pt in Stage IV, survival generally 1 yr
diverse group of processes that are characterized by replacement of normal bone by fibrous tissue containing a newly formed mineralized product
Fibro-osseous lesions
Developmental tumorlike condition characterized by replacement of normal bone by an excessive proliferation of cellular fibrous connective tissue intermixed with irregular bony trabeculae
Fibrous dysplasia
What is the character of the abnormal fibro- osseous tissue
poorly formed
Functionally inadequate
By what process does the abnormal fibro-osseous tissue arise in Fibrous dysplasia
Metaplasia
when Fibrous dysplasia is confined to 1 bone
Monostotic (80% all cases)
Most common symptom of Monostatic fibrous dysplasia
painless swelling at site
Part of oral cavity most affected by Fibrous dysplasia
Maxilla
Fibrous dysplasia that involves 2 or more bones
polyostotic
Polyostotic fibrous dysplasia associated with café au lait pigmentation
Jaffe-Lichtenstein Syndrome
Maxillary fibrous dysplasia normally involves more bones than just the maxilla, so it is commonly called
Craniofacial fibrous dysplasia
Polyostotic fibrous dysplasia with café-au lait and multiple endocrinopathies (e.g. sexual precocity, pituitary adenoma, hyperthyroidism)
McCune-Albright Syndrome
This fibrous dysplasia syndrome also has affectation or effeacement of various neural foramina at base of skull in CT
McCune-Albright Syndrome
What is the character of the café au lait pigmentation in McCune Albright syndrome
uniform pigment with irregular border
What is the radiographic character of Fibrous dysplasia
highly variable from radiolucent to radiopaque
2 radiographic clues for Fibrous dysplasia
1) cortical persistence of inferior border mandible with bone enlargement
2) Superior displacement of IAN canal
What is the most common radiographic appearance of Fibrous dysplasia and what does it indicate
ground glass pattern(end of slide or decanter stopper). Indicates lesion is hard & will be difficult to remove surgically
2 pathognomonic radiographic returns for Fibrous dysplasia
peau d’orange
Fingerprint
Fibrous dysplasia lesions in youth versus adult
Youth: Hypervascular
Adult: avascular
Concern with surgery in youth fibrous dysplasia
Hemostasis due to hypervascularity
Concern with adult fibrous dysplasia
avascular bone so more susceptible to infection
Only fibrous dysplasia type that has risk of malignant transformation
polyostotic
dysplastic condition at apices of anterior mandible teeth
periapical cement-osseous dysplasia
What demographic does periapical cement- osseous dysplasia have a marked predilection for
middle aged African American females
What is the status of the teeth associated with Periapical Cemento-Osseous dysplasia
vital(key for endo)
Stage I periapical cement-osseous dysplasia radiographic appearance
periapical radiolucency (do vitality testing so don’t do unnecessary endo)
Stage II periapical cement-osseous dysplasia radiographic appearance
mixed radiolucent/radiopaque lesion as woven and lamellar bone and cementum mineralize
Stage III periapical cement-osseous dysplasia radiographic appearance
large radiopaque mass due to masses coalescing to form acellular disorganized cement-osseous material
Is there any treatment indicated for periapical cement-osseous dysplasia
No, don’t want to biopsy or extract because exposing to oral environment could superimpose osteomyelitis on dysplastic bone
a subset of Periapical Cemento-osseous dysplasia that has extensive periapical cement-osseous dysplasia in multiple posterior quadrants
Florid Cemento-osseous dysplasia
Does Florid cemento-osseous dysplasia have 3 developmental stages like Periapical cement-osseous dysplasia
Yes
What is the danger with Periapical or Florid cement-osseous dysplasia
Exposing it to oral environment which can cause osteomyelitis
What is the characteristic Florid Cemento-osseous dysplasia histologically
ginger root curvilinear trabeculae
Benign, well-circumscribed, slow-growing neoplasm of craniofacial bones, predominantly in the mandibular molar and premolar region, with a predilection for Females in their 3rd – 4th decade
cement-ossifying fibroma
What radiographic feature is key for cement- ossifying fibroma
well-circumscribed, sharply defined border
How does cement-ossifying fibroma grow
equidirection expansion with centrifugal growth pattern that can thin cortical bone
What is composition of Cemento-ossifying fibroma
uniform fibroblasts and small irregular masses resembling cementum
What is a differentiating characteristic between cement-ossifying firbrom and fibrous dysplasia
cement-ossifying fibroma does not arrest at skeletal maturity like fibrous dysplasia does
Cemento-ossifying fibroma treatment
complete enucleation with larger possibly requiring surgical resection and bone grafting
Ossifying fibrom that is rapidly growing, well- circumscribed lacking continuity with adjacent bone
Juvenile aggressive ossifying fibroma
Major pattern of Juvenile/Aggressive Ossifying Fibroma that occurs in the craniofacial skeleton
psammomatoid
What part of the craniofacial skeleton does Juvenile/Aggressive Ossifying Fibroma occur most often
maxilla, psammomatoid. 70/5 in orbital, frontal bones and paranasal sinuses
Symptoms of Juvenile/Aggressive Ossifying Fibroma
nasal obstruction, pain, sinusitis, headache, proptosis (eye bulging)
Juvenile/Aggressive Ossifying Fibroma histology
psammomatoid bodies embedded in cellular stroma of stellate cells w/ hyperchromatic nuclei, no mitotic bodies. Osteoid and woven bone when mature
Treatment and recurrence of Juvenile/Aggressive Ossifying Fibroma
surgical excision and curettage, 30-58% recurrence but no malignant transformation
Most common osseous tumor of the jaw
Osteoma (benign neoplasm of bone)
Does an osteoma occur in immature or mature bone
mature lamellar bone
Do palatal tori, mandibular tori, and buccal exostoses represent osteomas
No
2 ways osteomas can arise
1) on bone surface = periosteal/peripheral/exophytic osteoma
2) In medullary bone = endosteal or central osteoma
Most common osteoma location
body of mandible or condyle
When osteoma found in the body of the mandible, where will it most likely occur
posterior to premolars, lingual side
How would one differentiate between and osteoma in the condyle and condylar hyperplasia
condylar osteoma would be lobulated, condylar hyperplasia the condyle retains its shape
How will an osteoma look histologically
lots of dense bone w/ little marrow and prominent osteoblastic activity
What is the demographic for osteomas
6th decade, females
How do osteomas appear radiographically
well circumscribed sclerotic mass attached by a broad base to underlying bone. Radiopaque
Can an osteoma cause root resorption
yes
Where are the two most common sinus locations for maxillary osteomas
Frontal osteoma, 80%. # 2 is ethmoid sinus
what is the problem with sinus osteomas
they are aggressive, cause obstructions and recur after initial attempts to remove
how treat an osteoma
surgical removal to secure diagnosis and treat cosmetic or functional defects
What is osteoma is small, asymptomatic, and located endosteal, how treat
observed periodically
Autosomal inherited trait with 100% penetrance or the result of spontaneous mutation on Chromosome 5 characterized gnathically by multiple osteomas and multiple supernumerary teeth
Gardner syndrome
What is another systemic manifestation of Gardner syndrome
colorectal polyps that will become malignant
What does Chromosome 5 have to do with Gardner syndrome
it is the adenomatous polyposis coli (APC) tumor suppressor gene (think colon polyps)
What will all patients with Gardner Syndrome develop in their life, 50% by age 30
aggressive colon adenocarcinoma
2 prophylactic treatments in Gardners
prophylactic colectomy
Screen other family members
when do the multiple osteomas of Gardners syndrome usually present
during puberty and precede colon polyps
Can the multiple osteomas of Gardners be treated
yes, for cosmetic reasons, but long term prognosis depends on bowel adenocarcinomas
Benign tumors composed of mature hyaline cartilage
Chondroma
Where are chondromas normally located
short tubular bones of hands and feet
) If a chondroma is going to occur in the head and neck, what is the embryonic origin of its location
where embryonic cartilaginous remnants located
Where are the embryonic cartilaginous remnants that are conducive to chondromas located in the head and neck
- Mandible: premolar area(Meckel’s cartilage), condyle, coronoid process, symphysis
- Maxilla: nasal septum, ehtmoid sinus
When are chondromas normally diagnosed
Before age 60
How do chondromas look radiographically
localized irregular radiolucent defect with punctuate or stippled calcifications
What will chondromas do to small bones
expansion and cortical thinning
If chondroma in the jaw, is there root resorption
yes
What is the dilemma with chondromas in the jaw
Some believe they are chondrosarcomas meaning they are malignant
What indicates a chondroma versus a chondrosarcoma in the jaw
Chondroma will have absence of pain, lack of disturbed cortex and surrounding bone architecture and no cytologic atypia
What does an osteochondroma consist of
cancellous bone surrounded by compact bone with a cartilaginous cap
If patient has multiple osteochondromas what will be the physical character of their bone and what are they at risk for
deformed bone
Propensity to develop chondrosarcoma
What will an osteochondroma look radiographically
radiopaque extraosseous projection
where do osteochondromas occure most frequently
condyle and coronoid process
Benign bone tumor arising from osteoblasts that is rare in the jaw
Osteoid osteoma
Demographic for Osteoid Osteoma
Males (3:1), 2nd decade
Pathonomonic clinical symptoms for Osteoid Ostemoa
- pain of increasing severity
- Worse at night
- Relieved w/in 20-30 min of aspirin/NSAID dose
How does and osteoid osteoma appear radiographically
well demarcated radiopaque nidus, round to ovoid wit h a peripheral radiolucent rim surrounded by zone of reactive host sclerosis
Is the sclerosis surrounding an Osteoid Osteoma a part of the lesion or a host response
host response that resolves once nidus is gone
When the nidus is well ossified, how can the Osteoid Osteoma look radiographically
targetoid or bulls-eye appearance (what is other pathology that has targetoid appearance?)
What is the histopathology of Osteoid Osteoma
irregular randomly ordered trabeculae of osteoid and woven bone
What is the character of the nidus in Osteoid Osteoma
well vascularized, clearly separate from reactive bone
what is the growth potential of Osteoid Osteoma
limited growth, nidus < 2 cmdiameter
What is responsible for reactive host sclerosis, inflammatory changes in soft tissue and pain in Osteoid Osteoma
large amounts of prostaglandin E2 and prostacyclin
Treatment of Osteoid Osteoma
surgical excision of nidus and surrounding bone and curettage
Also a benign bone tumor arising from osteoblasts that occurs in Males(3:1) in 2nd decade
Osteoblastoma
Differences of osteoblastoma from Osteoid Osteoma
Osteoblastoma will grow >2cm, does not have nocturnal pain relieved by aspirin/NSAID, lacks secondary peripheral sclerosis
Where will an osteoblastoma appear in mandible radiographically
in vicinity of tooth root with a central opacity surrounded by peripheral radiolucent halo
How treat osteoblastoma
surgical en bloc excision (as a unit, all together). Nidus is well demarcated and can be removed easily from surrounding bone
Histology of Osteoblastoma (same as Osteoid Osteoma)
interlacing network of bone trabeculae evenly distributed in loose fibroblastic stroma with prominent vasculature and osteoblastic rimming
Aggressive Osteoblastoma character
occur in older patients (>30) larger than normal osteoblastoma (>4cm) severe pain, locally destructive w/ high recurrence after surgery
The most common primary malignancy of bone (excluding hematopoietic tumors)
osteosarcoma
What must an osteosarcoma be able to do
produce osteoid and immature bone
What is demographic for osteosarcoma
males, during periods of highest growth. 10-20 years and >50 years
What bones does osteosarcoma arise in normally
skeletal areas with highest growth rate: distal femur, proximal tibia, proximal humerus
At what age does an osteosarcoma in the mandible or maxilla normally occur
3rd and 4th decade
What disease and treatment can predispose for osteosarcoma
Paget’s disease
Previous irradiation
Osteosarcoma clinical symptoms
pain (weeks to months) of increasing intensity, swelling, skin warm to touch
Weight loss associated with osteosarcoma indicates metastasis to what area
Lungs
What are the radiographic features of jaw osteosarcoma
ill defined border/indistinct, spiking of teeth due to root resorption, sunburst/sunray appearance, Codman’s triangle
What causes the sunburst/sunray appearance on an osteosarcoma
osteophytic bone production on surface of lesion
What is an important early radiographic sign of a jaw osteoma
symmetrical widening of PDL around 1 or several teeth
What will be elevated in patient with osteosarcoma
alkaline phosphotase
Treatment for osteosarcoma
Neodjuvant (preop) chemo and radiation
Surgical excision
Adjuvant (postop) chemo
What is Codman’s Triangle
Tumor growing on bone surface can elevate periosteum and cause periosteal reaction of open triangle showing radiographically
What will happen to the teeth along with the external root resorption in osteosarcoma jaw lesions
mobility
Essential microscopic criterion for osteosarcoma
direct production of tumor osteoid by malignant mesenchymal cells
Secondary Osteosarcoma can arise from where
Paget’s disease Fibrous dysplasia Ionizing radiation >3000 Gys Bone infarcts Chronic osteomyelitis Metallic prosthetic implants
What are the clinical symptoms associated with sarcomatous transformation
sudden onset of pain
Rapid worsening of previous pain
Increased deformity
Character of Secondary Osteosarcomas
high grade
Aggressive
Poor prognosis
Malignant tumor characterized by the formation of cartilage, but not bone, by tumor cells. Can arise de novo or within a preexisting benign chondroma
chondrosarcoma
Second most frequent bone sarcoma
chondrosarcoma
where do chondrosarcomas occur gnathically
Maxillary anterior alveolus, orbit, nose, condyle or skull base
Can there be a periosteal reaction with chondrosarcoma
yes
Cortical disruption with significant extension into soft tissues signifies what type of chondrosarcoma
advanced lesion
What can be seen with the teeth in chondrosarcoma that was seen with osteosarcoma
widening of PDL with root divergence or resorption
Most common type of chondrosarcoma
Mesenchymal
How are chondrosarcomas graded
by histologic features
Chondrosarcoma with lobular architecture, rarely metastasizes
Grade I
Chondrosarcoma with myxoid stromaand mitotic figures, 10% metastasis rate
Grade II
Chondrosarcoma with markedly cellular, spindleform, abundant mitosis, 71% metastasis rate
Grade III
treatment for chrondrosarcoma and where contraindicated
Wide local or radical surgical excision. May not be possible in base of skull
Is radiotherapy or chemotherapy useful in chondrosarcoma treatment
No
When is prognosis for Chrondosarcoma worse
Jaws. Maxilla worse prognosis than mandible
What can a chondrosarcoma be confused for
Chondroblastic osteosarcom which would have some bone in it (radiograph would have some more opacity and greater change of periosteal reaction)
Why is it important to differentiate between a chondrosarcoma and a chondroblastic osteosarcoma
because treatment and prognosis is different. Osteosarcomas are slower growing with a reduced metastasis rate which increases their prognosis. As well osteosarcomas have other therapy options beyond surgical excision.
