Bone Disorders Flashcards
most common primary bone malignancy
osteosarcoma
most common general location of osteosarcoma
long bones
most common dental relevant location of osteosarcoma
mandible
most common age for osteosarcoma
10-25 (jaw lesions a decade later)
osteosarcoma symptoms
- pain, paresthesia in mandible
- nasal obstruction, epistaxis (nose bleeds) in maxilla
radiographic features - osteosarcoma
- sunburst radiopaque lesions
- PDL widening
osteosarcoma histology
- sarcomatous stroma
- production of osteoid and bone
osteosarcoma - differential diagnosis
- chondrosarcoma
- osteoblastoma
- ossifying fibroma
how can you tell the difference between osteosarcoma and chondrosarcoma?
- histologically chondrosarcoma has cartlaginous structures and does NOT have osteoid and bone in the stroma
- chondrosarcoma does not show distinctive deomographics like osteosarcoma
osteosarcoma - treatment
radiation & chemotherapy
chondrosarcoma
malignant neoplasm devoid of osteoid and bone with cartlagenous structures
is chondrosarcoma common or rare?
rare
chondrosarcoma clinical features
- expansion
- loose teeth
- nasal discharge
- pain may be present
chondrosarcoma histological features
- fully developed cartilage
- NO osteoid or bone
- very large nuclei
chondrosarcoma radiographic features
- “cotton wool” calcifications
- widened PDL may be present
mesenchymal chondrosarcoma
dental relevant subtype of chondrosarcoma - more commonly occurs in the mandible
fibrosarcoma
malignant neoplasm of fibroblastic cells
fibrosarcoma: common or rare
rare
fibrosarcoma - age
over 40
what is an important clinical treatment that could potentially cause fibrosarcoma?
radiation therapy
clinical features of fibrosarcoma
- same as any malignancy - NOT characteristic
- mass or swelling common
- pain, paresthesia
- trismus
- MIMICS: SCC which is much more common
can fibrosarcoma occur in children
yes (childhood head and neck fibrosarcoma is a distinct entity)
fibrosarcoma - histological features
- interlacing connective tissue cells (looks like interlaced cable wires)
- cells arranged in fascicles often with a “heringbone” pattern
what are the two forms of fibrosarcoma in children
- desmoplastic
- medullary
which childhood form of fibrosarcoma behaves like the adult form?
desmoplastic fibrosarcoma
which childhood form of fibrosarcoma is less aggressive
medullary fibrosarcoma
how do you treat fibrosarcoma?
complete excision (get it out, radical resection advised)
fibrosarcoma prognosis
not good
langerhans cell histocytosis (LCH)
neoplastic proliferation of langerhans cells with a wide spectrum of biologic behavior
what are the three categories of LCH?
- chronic focal/”eosinophilic granuloma”/unifocal
- chronic disseminated/”Hand-Schüller-Christian disease”/multifocal unisystemic
- acute disseminated/”Letterer-Siwe disease”/multifocal multiple system
need to know the different names
characteristics of the chronic focal/ “eosinophilic granuloma”/unifocal type of LCH
- solitary granuloma
- no soft tissue or organ involvement
- easily treatable
- M > F
- children & young adults
- radiographically - teeth appear to “float in air”, unilocular lesions in skull appear “punched out”
- simple excision or curettage
characteristics of chronic disseminated/ “Hand-Schüller-Christian disease”/multifocal unisystemic type of LCH
- multifocal (affects multiple bones & organs)
- multiple lesions
- multiple sites
- diabetes insipidus
- hisotlogiccally - histocytes & eosinophils
- surgery, radiation, chemotherapy, steroids
characteristics of acute disseminated/”Letterer-Siwe syndrome”/mulitifocal multiple system type of LCH
- infants
- lethal form
- multiple organ involvement
- histologically - “foamy” cytoplasm
- chemotherapy
immunohistochemistry - LCH will stain positive for?
CD1
what is the name of the staging system for LCH
Kemp staging system
central giant cell granuloma of jaw
distinct diagnostic entity
central giant cell granuloma - demographics
- children and young adults
- F > M
central giant cell granuloma - location
anterior mandible (may cross midline, can occur in maxilla)
central giant cell granuloma - clinical features
- local swelling
- pain
- paresthesia - UNCOMMON
central giant cell granuloma - radiographic features
- root displacement or resorption
- unilocular or multilocular
- expansion without perforation of cortial plates
central giant cell granuloma - histology
multinucleated giant cells (unique but not specific)
central giant cell granuloma - differential diagnosis
- Hyperparathyroidism “Brown Tumors”
indistinguishable from central giant cell granuloma
this is systemic
MUST differentiate before treatment!
- Aneurysmal Bone Cyst - cavernous blood filled spaces
central giant cell granuloma
curettage and complete excision
cherubism
rare hereditary disease of mandible
cherubism - demographics
- children
- M > F
cherubism - preferred location
mandible
(bilateral rami; bilateral rami & posterior mandible; maxilla and madible)
cherbuism - radiographic features
thinig of corticies
(easy to diagnose if in all for quadrants of MX & MN, otherwise may require genetic screening, thins the cortex but rarely penetrates)
cherubism - treatment
slow regression after puberty, don’t over treat
may move teeth, but tends to reslove itself
radiotherapy contraindicated
fibrous dysplasia
skeletal abnormaliy of poorly mineralized bone (bone never gets hard - spongiosum replaced with fibro-osseous tissue)
two patterns of fibrous dysplasia
- monostotic
- polystotic
monostotic fibrous dysplasia
one bone
polystotic fibrous dysplasia
several bones
associated with Albright syndrome (several bones + endocrine abnormalities - may see precocious puberty)
which form of fibrous dysplasia is most common
monostotic
fibrous dysplasia - age
late childhood
become quiesent after puberty
fibrous dysplasia - genetics
GNAS1 gene
fibrous dysplasia - clinical findings
- unilateral
- painless
- slow onset
fibrous dysplasia - preferred location
mandible
fibrous dysplasia - radiograpic features
- eliptically shapped (unilaterally)
- “gound glass,”“orange peel,” fuzzy appearance
- diffuse, like “driven snow”
fibrous dysplasia - histology
non-specific histology (requires combo of histo, radiology, and clinical findings)
osteoid and calcified bone (“Chinese characters”)
fibrous dysplasia - prognosis
good
fibrous dysplasia - treatment
monitor
commonly “burns out” around puberty
sugery only if it becomes obstructive
fibrous dysplasia - differential diagnosis
ossifying fibroma
- ossifying fibroma - the cortex is hard and has to be drilled through to get to lesion
- fibrous dysplasia - the cotex is soft and can easily be cut through to get to lesion
can you treat fibrous dysplasia with radiation therapy?
NO
radiation therapy is contraindicated
osteoma
rare slow growing neoplasm of compact or cancellous bone
osteoma - M?F
males
what syndrome are osteomas associated with?
Gardner’s syndrome
(associated with multiple osteomas)
osteoma - differential diagnosis
- osteoblastoma
- osteoid osteoma
these grow more rapidly, show more cellularity, and are associated with pain compared to osteomas
do osteomas contain tooth remants/structures
yes
osteoma - treatment
monitor and meaure risk
excsision if necessary (i.e. obstruction)
exostoses/tori
not a neoplasm
reaction to bone stress
ossifying fibroma
slow growing, well-demarcated, benign fibrou-osseous lesion of bone
ossifying fibroma - origin
PDL cells
ossifying fibroma - preferred location
mandible
(posterior to canine)
aggressive form of ossifying fibroma
juvenile active ossifying fibroma
(ages 6-20, can grow very rapidly, need to monitor closely)
aggressive intervention required
ossifying fibroma - M?F
females
ossifying fibroma
lyctic lesion with radiopaque foci, usually has radiopaque border
looks like a marble
ossifying fibroma - histology
mixture of fibrous and calcified tissue
“cementoid” calcifications
ossifying fibroma - differential diagnosis
fibrous dysplasia
ossifying fibroma - treatment
excision, curettage, enucleation
radiotherapy contraindicated
osteoblastoma
rare, benign neoplasm associated with pain
(need to recognize that although incredibly rare if you are delaing with something that looks like an osteoma but is painful you could be dealing with this rare neoplasm)