Bone and Cartilage Tumors Flashcards
What bone conditions might show lesions in the brain?
Paget’s disease or osteoma
What benign bone neoplasms can present on the epiphysis of a bone?
Chondroblastoma or giant cell tumors
What benign bone neoplasms can present on the metaphysis of long bones?
- osteoblastoma
- osteochondroma
- Non-ossifying fibroma
- osteoid osteoma
- Chondromyxoid fibroma
- Giant cell tumor
What benign bone neoplasms can present on the diaphysis of long bones?
Enchondroma or Fibrous dysplasia
What malignant bone neoplasma can present on the diaphysis of long bone?
Ewing’s sarcoma or Chondrosarcoma
What malignant bone neoplasma can present on the metaphysis of long bones?
Osteosarcoma or Juxtacortical osteosarcoma
What are the most common primary bone neoplasms?
- MM
- osteosarcoma
- chondrocytomas
- Ewing’s sarcoma
Note that by far most bone tumors are metastatic carcinomas
Age rule with bone neoplasms
under 30= benign, over= malignant usually
Metastasis of bone neoplasms is usally via ____
venous circulation
Patient population for osteoma?
Adults and children (male slightly more than female)
Describe osteomas
bone formaing tumors composed of compact or mature trabecular bone that are confined almsot exclusively to cranial-facial bones (paranasal sinuses) and present with pain, headache, and vision changes
Osteomas are associated with what?
Gardner syndrome
What is Gardner syndrome?
a variant of familial adenomatous polyposis caused by APC gene mutations on chromosome 5q21
MOI of Gardner syndrome?
AD
What is gardner syndrome characterized by primarily?
multiple colon polyps
Where else can extracolonic tumors present in Gardner syndrome?
- osteomas in the skull
- thyroid cancers
- epidermoid cysts
- fibromas
- desmoid tumors in approx. 15% of patient s
What case the epidermoid cysts seen in Gardner syndrome?
doubling of the squamous layer of skin with keratin debris in middle
Describe osteoid osteomas
benign tumors of young (less than 25) males associated with osteoblasts that produce osteoid surrounded by a rim of reactive bone (common in cortex of long bones of the leg)
less than 1.5cm in diameter
How does osteoid osteoma present?
(may be nocturnal) bone pain in the leg of a young male that resolves with aspirin
How are osteoid osteomas treated?
radiofrequency ablation
Describe osteoblastomas.
similiar to an osteoid osteoma but is LARGER (2+ cm), and is associated with dull bone pain but IS NOT relieved by aspirin
How else are osteoblastomas differentiated from osteoid osteomas?
more commonly found in the VERTEBRAE, in female and children, and NOT relieved by aspirin
How are osteoblastomas treated?
curettage or excision
What bone neoplasm is characterized by a MALIGNANT proliferation of osteoblasts?
osteocarcomas
What patient population is common for osteosarcomas?
60% male, ages 10-20
Associations with osteocarcomas?
Paget disease and post-radiation in older patients
as well as Rb gene (retinoblastoma)= poor prognosis
Microscopic presentation of osteocarcoma?
spindle cell appearance with unmineralized/osteoid bone
How common is metastasis with osteocarcoma?
20% will have lung METs at diagnosis
Common spots for osteosarcoma?
- knee metaphysis (60%)
- hip (15%)
- shoulder (10%)
- nasal (8%)
Buzzword on xray with osterosarcoma?
Codman Triangle- periosteal reaction (indicates an aggressive tumor)
Buzzoword on bone biopsy with osteosarcoma?
‘lace like’
5 yr survival of osteosarcoma?
about 75% in children and goes down with secondary disease
Acquired genetic abnormalites associated with increased risk of osteosarcoma?
Rb, a critical negative regulator of the cells (mutation= 1000x risk for OS.
TP53 mutation (li-Fraumeni syndrome or sporadic)
INK4a, (incodes tumor suppressors)
MDM2 and CDK4, cell cycle regulators that inhibit p53 and RB function, respectively
What are the cartilage forming tumors?
- chondroma
- osteochondroma
- chondrosarcoma
What are the types of chondroma?
benign cartilaginous tumor that is classified as either:
1) enchondroma (arise from diaphyseal medullary cavity)
2) subperiosteal/juxtacortical chondroma
3) soft tissue chondroma
What genes have been ID’d in the chondrocytes if syndromic and solitary enchondromas?
IDH1 and IDH2 (also seen in gliomas and acute myeloid leukemia (AML))
IDH= isocitrate dehydrogenase
What is the most common benign tumor of bone?
osteochondroma
Patient population for osteochondroma?
mostly males, 10-20 yrs old
Describe osteochondromas
slow growing, painful benign tumors that usually stop growing at puberty and ossify
What characteristic appearance do osteochondromas take on in x-ray?
broccili/califlower appearance budding off bone
Osteochondromas rarely transform to what?
chondrosarcomas (5-10%)
How are osterochondromas treated?
simple excision
Describe osteochondroma development
intitially, at the epiphyseal plate, there is a small outpouching of cartilage, which grows proximally to the epiphyseal plate and comes to resemble a mushroom/califlower growing out of bone
What covers the mushroom projection caused by osteochondromas?
a cap of cartilage (dont fucking forget this)
Describe enchondromas
usually asymptomatic tumor that presents in the small bones of hands and feet and are mostly solitary
treatment= excision or nothing
Patient population for enchondromas?
20-49, no gender preference
Note on multiple enchondromas
may produce sever deformities; associated with chondrosarcoma transformation
What is Maffuci syndrome?
multiple enchondromas and soft tissue hemangiomas assoicated with ovarian carcinoma and brain gliomas
What is Ollier disease?
nonhereditary disease of multiple enchondromas of LONG bones and flat bones (up to 50% of skeleton) with associated skeletal deformities and often ovarian sexcord tumors
T or F. Most lesions associated with Ollier disease progress into adulthood
F. Most regress with skeletal maturation
What are chondrosarcomas?
malignant cartilage forming tumors (NO OSTEOID)
Where do chondrosarcomas arise from?
the medulla of the pelvis or central/axial skeleton
Patient population for chondrosarcomas
men in 40s
What are the fibrous and Fibroosseous Tumors?
- Fibrous cortical defect
- Fibrous dysplasia
What are fibrous cortical defects?
NON-OSSIFYING fibromas greater than 5cm with an intramedullary component that is benign and typically asymptomatic
Patient population for fibrous cortical defects?
teenagers, no gender preference
What causes fibrous cortical defects?
probably developmental defects
What causes fibrous dysplasia?
benign tumor arising during development forming a intramedullary fibrous mass (fibrous tissue instead of medullary bone) resulting in painful swollen bones prone to fracture that presents in early adolescence
What bones are most commonly affected by fibrous dysplasia?
ribs and femur
Types of fibrous dysplasia?
- monosodic
- polysodic
- McCUne-Albright Syndrome
What is McCune-Albright Syndrome?
fibrous dysplasia plus cafe-au-lait skin pigmentations and endocrine abnormalities, especially precocoius puberty
What are giant cell tumors of bone?
tumors of young adults (20-40s) arising from the epiphysis of long bones, typically the knee
The neoplastic cells of giant cell tumors express what?
high levels of RANKL
Buzzword for giant cell tumors?
soap bubble on x-ray
What tumor is commonly described grossly as ‘fish flesh’?
Ewing sarcoma (PNET)
What causes Ewing sarcoma?
malignant proliferation of poorly differentiated cells from NEUROECTODERM
second most common bone tumor in children after osteosarcoma
Where are Ewing sarcomas commonly found in bone?
diaphysis (medullary) in LONG bones
Patient population of Primitive neuroectodermal tumor (PNET) (aka Ewing)?
white male children under 15
X-ray appearance of Ewings?
onion skin
Common mutation of Ewing’s?
t(11,22) translocation (EWS-FLI1 fusion)
How is Ewing’s treated?
aggressive treated with neoadjuvant chemo followed by surgical excision with or without radiation
Prognosis for Ewing’s?
effective dchemo achieves 5-yr survival of 75% and long term cure of 50%
the amount of chemo-induced necrosis is an important prognostic finding
T or F. Metastatic bone tumors are more common than primary
T. Much
Common places that go to bone?
BLT with a Kosher Pickle
80% from breast, lung, and prostate
thyroid and kidney
Why would metastatic tumors go to axial bone?
it has a much richer blood supply
Which MET bone cancer is associated with osteoblastic (bone forming) lesions?
prostatic carcinoma
Which MET bone cancer is associated with lytic lesions?
MM (via icnreased osteoclast activation)
T or F. All MET bone cancers are Stage IV with poor prognosis
T. Except maybe lymphoma
