Bone Tumors - Exam 1 Flashcards
What are the 2 different kinds of bone tumors? What are the major dividing characteristics?
benign: confined and doesn’t spread
malignant: invades tissues and metastasizes
What are the 2 different kinds of malignant bone tumors?
primary: arise from bone cells
secondary: metastasized and spread to bone
What 5 kinds of cancer like to spread to the bone?
breast, prostate, lungs, thyroid and kidneys
On the cellular level, what leads to adnormal bone cell growth?
presence of oncogenes AND mutated tumor suppressor genes
______ = promotes normal cell growth
______ = overstimulates cell growth
proto-oncogenes
oncogenes
How will most benign tumors present? What are the 2 exceptions?
most are asymptomatic, DO NOT weaken bones and have NO constitutional symptoms
2 benign types that weaken bones are osteoid osteoma and osteochondroma
How will malignant tumors present?
with a dull ache that is worse with activity and progresses over time
+/- soft tissue mass firmly attached to bone
Constitutional symptoms are late findings (think mets)
aka Jory said patients that limp without an apparent cause
Benign or malignant?
benign
benign or malignant
malignant
_____ is the best test for assessing metastatic disease in the thorax
CT scan
What 3 reasons would you order an MRI if you suspect a bone tumor?
Determines tumor size
Assesses extent of intraosseous and extraosseous involvement
Add contrast for biopsy planning
Osteoid osteoma arises from what bone tissue? Who is the MC pt? What location?
osteoblasts
males, 10-35 in the FEMUR (then tibial and humeral shaft)
What is the pathophys behind an osteoid osteoma?
As bone tissue proliferates, a nidus surrounded by sclerotic bone is formed
The nidus secretes prostaglandins leading to the pain associated with this tumor
What is a nidus? What kind of bone tumor?
Nidus: a centrally located disorganized mixture of small blood vessels, trabecula (tiny rods of bone), and osteoid (unmineralized bony tissue)
osteoid osteoma
Why is an osteoid osteoma painful?
The nidus secretes prostaglandins leading to the pain associated with this tumor
What is the presentation of osteoid osteoma? When is it worse? **What makes it better? Where on the body will the pt say it hurts?
Localized, deep, constant, aching pain, +/- palpable mass, tenderness overlying the lesion, neurologic symptoms
worse at night
improves with NSAID or ASA
Atypical juxta-articular presentation
Pain in joint (hip - MC) with walking with limp
Referred pain to the knee
**What will the xray show for an osteoid osteoma?
sclerosis around a lucent nidus (< 1.5 cm) 1.5cm is important
**aka a punched out lesion surrounded by sclerosis
In osteoid osteoma, a calcified nidus leading to a radiopaque point is called a _______. What can a nidus close to the bone surface appear like?
bell
nidus close to the bone surface can appear like a fracture
What is the arrowing pointing to?
bell
What am I?
lucent nidus
**What is the preferred imaging following an XR for osteoid osteoma?
**CT w/ IV contrast
What are the 3 indications for a CT w contrast in osteoid osteoma?
X-ray appears abnormal, but the nidus isn’t visible
Residual or recurrent tumor is present
Tumor is located in a critical area (spine or femoral neck)
When a bone scan is used in osteoid osteoma, what finding is diagnostic? Are bone scans more or less sensitive than xrays?
“Double density” sign is said to be diagnostic
bone scans are MORE sensitive than xray
Why are MRIs LESS accurate than CT in osteoid osteoma? When are they used?
Less accurate than CT due to reactivity of bone from the edema surrounding the lesion
Often used to assess cases not confirmed by x-ray or CT
What is the management for osteoid osteoma that is well controlled with OTC therapy?
then f/u with serial imaging every 4-6 months
What is the management for osteoid osteoma that is NOT well controlled with OTC therapy? How is the pain resolved?
refer to ortho for surgical intervention
Removal of the nidus results in resolution of pain
What is the prognosis for osteoid osteoma?
Untreated lesions will resolve spontaneously over several years
What are the defining features of a osteoblastoma?
slow growing and more aggressive than osteoid ostoma
nidus > 2cm this is the biggest difference
Posterior column of the spine
Who is the MC pt with osteoblastoma?
male 10-20 years old in the posterior column of the spine
Will osteoblastoma be relieved with NSAIDs? How will the pt present?
NO!! pain is unrelieved by NSAIDs
limp, spinal cord/nerve root compression, scoliosis
What are 2 xray findings that are consistent with osteoblastoma?
Well-circumscribed radiolucent lesion (nidus) > 2 cm
Spinal lesions will likely extend into the soft tissue due to the limited size of the bone to contain the tumor
What is the next imagine you should order if you suspect osteoblastoma?
CT with contrast
indicated in ALL patients, will define the size and extent of the lesion
When would you order an MRI in osteoblastoma?
Best for lesions that extend into surrounding soft tissues, bone marrow, and spine
(Osteoid osteoma/osteoblastoma) typically have a surrounding, intense, reactive zone of bone
osteoid osteoma WILL have this reactive zone of bone
osteoblastomas will NOT have this reactive zone of bone
After imaging, _____ is needed to confirm. Then refer to ____? Then ______ is usually recommended in all patients
core needle bx vs open bx
oncologic ortho surgeon
surgical resection or marginal resection, +/- post-surgical radiation
What is the recurrence rate of osteoblastoma?
10-20% and more likely if section was not adequately resected or in the spine
_______ is the MC solitary benign bone tumor. What will it present like?
OSTEOCHONDROMA
Benign, cartilage-capped bony PROJECTION on the external surface of the bone
Who is the MC pt population for osteochondroma? Where are the 2 MC body sites?
males in their 20s
around the knee and proximal humerus occurs adjacent to the epiphyseal growth plate
Besides the knee and proximal humerus, name 2 additional sites for osteochondromas? When does the tumor usually stop growing?
pelvis and scapula
Growth of tumor ceases when growth plates close
If the pt has multiple osteochondromas, likely linked to _____ mutation. What genes specifically?
A genetic mutation in the tumor suppressor genes EXT1 or EXT2
How will an osteochondroma present? ______ may also occur
Most are asymptomatic and found incidentally on imaging,
Painless mass near a joint or on the axial skeleton
can be painful in the stalk fractures due to trauma
-> Growth plate dysfunction can occur with varus or valgus deformity and shortening of long bone
What is the xray finding you will see with osteochondroma?
Bone spur that extends away from joint
What is the management for an asymptomatic osteochondroma?
Asymptomatic lesions
No intervention
Monitor with annually with clinical exam
If change in exam, → MRI
What is the management for an symptomatic osteochondroma?
Surgical excision indications if large lesion, associated with deformity and functional limitations
Osteochondromas have ____ risk of becoming malignant and may turn into _____. What increases risk?
<1% risk
chondrosarcoma
if you have the HMO genes
What would make you suspect the pt’s osteochondroma has transformed into a chondrosarcoma?
New onset growth of lesion
New onset pain
Rapid growth of lesion
What is the recurrence rate of solitary osteochondromas? What if the pt has Hereditary multiple osteochondromas (HMO)?
Local recurrence rate of less than 2%
HMO: Monitor frequently for growth defects and neurologic changes which may be associated with a spinal lesion
What is an enchondroma? What does the tumor do?
A benign cartilage forming tumor that develops in the bone marrow of long bones
Lesions replace normal bone with hyaline cartilage
What are the 2 MC cites of involvement for enchondroma?
hands and feet
What is enchondromatosis? What age does it usually present?
A non-hereditary, acquired genetic mutation resulting in multiple enchondromas, often with a unilateral predominance
before age 10
How will enchondroma present?
asymptomatic (found incidentally)
Widening of the bone
Angular deformity
Limb-length discrepancy
pain from pathologic fracture in weight bearing bones
What will an enchondroma present like on xray?
centrally located
Round or oval
Well circumscribed
Sclerotic border
Lobulations are often seen inside the lesion
What will an enchondroma present like on a bone scan? Why should you order additional imaging?
Test negative in uncomplicated enchondromas
aka they will NOT have a signal because they are NOT vascular
CT/MRI: May be ordered to rule out ddx of chondrosarcoma or bone infarct
Why would you bx an enchondroma?
If painful lesion without fracture must rule out chondrosarcoma
What is the management for small, asymptomatic enchondroma lesions at low risk for pathologic fracture?
monitor with exams and serial imaging
F/u frequency depends upon the size, location, and number of lesions
What is the management for symptomatic enchondromas?
Curettage & bone grafting but fx should be allowed to heal first before curettage
What are the pathologic fracture risk factors?
Weight bearing bone
> 25 mm in diameter
Involving > 50% of the diameter of the cortex
What is the prognosis for enchondromas?
Solitary enchondromas usually are self-limiting but continued growth may occur
Recurrence after curettage and bone graft is rare
Enchondromas of ______ & ____ may be complicated by malignant transformation to ________. Occurs in up to ___% of patients with enchondromatosis. Rare in _____
long bones & pelvis
chondrosarcomas
50%
rare in solitary lesions
What is a chondroblastoma? Who is the MC pt population
A benign cartilage-forming tumor that usually arises in the epiphyses or apophyses of long bones
males in their 20s
Where are the 3 common sites of involvement for chondroblastomas? Which one is highlighted?
Epiphysis of the proximal humerus
distal femur
proximal tibia
What will be in a chondroblastoma pt’s history? **What is the highlighted phrase I should remember about chondroblastomas?
Chronic pain that is mild and gradually progressive
Pain is often constant and unrelated to activity
Joint stiffness and swelling
pain is often CONSTANT and UNRELATED TO ACTIVITY
What will the PE look like for a chondroblastoma?
Localized tenderness
Decreased range of motion
Joint effusion
Muscle atrophy may occur
Limp in weight bearing lesions
What will the xray reveal in a pt with chondroblastoma?
Small, well-defined lesions with a sclerotic border that may cross the physis (growth plate)
When would you bx a chondroblastoma?
Indicated before surgery if lesion is atypical on imaging
What is the management for a chondroblastoma?
Surgery is the primary and definitive treatment
Curettage and bone grafting
What is the prognosis of chondroblastoma?
Arthritis may occur if the lesion involves the articular surface
Recurrence rate of 20%
What is the complication of chondroblastoma? What should you do next?
Benign pulmonary metastases may occur
Obtain a CXR in all patients; follow with CT w/ IV contrast if any suspicious areas are present
These pulmonary lesions are resectable and curable
What are the 3 different types of fibrous lesions?
fibrous dysplasia
ossifying fibroma
nonossifying fibroma
______ is abnormal fibrous tissue and trabecular bone replaces normal bone marrow and bone tissue leading to bone weakness. Are these lesions fast or slow growing? When do they often appear?
FIBROUS DYSPLASIA
slow growing
often appears during periods of bone growth
Who is the MC pt population for fibrous dysplasia? What genetic condition is it associated with?
male early teen/adolescent/early adulthood year
McCune-Albright Syndrome
What are the 4 common sites for fibrous dysplasia?
Proximal femur- MC of the common sites
Tibia
Skull/maxilla
Ribs
What is the presentation of fibrous dysplasia?
asymptomatic but pain/tenderness can occur as the fibrous tissue expands into bone or a fx develops
bone deformity may occur
bone swelling
waddling gait
What is a Shepard’s Crook deformity? What type of boney lesion is it associated with?
Varus deformity of the proximal femur
fibrous dysplasia
What am I?
fibrous dysplasia
What will fibrous dysplasia show up like on CT?
Note the spongy-like appearance
What will fibrous dysplasia show up like on xray?
Lytic lesion with “ground-glass” appearance
bone expansion
bowing
cortical bone thinning
Why would you order a bone scan in fibrous dysplasia? Do you always need to bx?
Indicated after initial dx to exclude polyostotic disease
bx: in atypical presentations
What is the management for asymptomatic fibrous dysplasia patients? Symptomatic?
Asymptomatic patients can be monitored with serial exams and imaging
symptomatic: Curettage & bone grafting is an option AND IV bisphosphonates
When would you want to AVOID curettage and bone grafting in symptomatic fibrous dysplasia? Why?
Avoided in pediatric patients due to high rate of recurrence
If polyostotic lesions are found and firbous dysplasia is the cause, what should you do?
refer to endo
need to r/o McCune Albright syndrome
What is the prognosis of fibrous dysplasia?
Recurrence is high after curettage
Monostotic lesions stabilize with skeletal maturity
< 1 % convert to osteosarcoma, fibrosarcoma, or even chondrosarcoma – most often occurring in adulthood
What is another name for ossifying fibroma? What are the 3 words used to describe it?
Osteofibrous Dysplasia
A destructive, deforming, slow growing benign fibro-osseous lesion
What are the 2 MC locations of ossifying fibroma in children? What age range?
kiddo: tibia and fibula
Generally, in children less than 10 y/o – peak between 1-5 y/o
What is the MC location of ossifying fibroma in adults? What is the gender preference?
mandible in adults
Male = Female
What is the presentation of ossifying fibroma? What is the MC clinical sign?
Localized, firm swelling in the affected area- MC clinical sign
Tibia is frequently bowed anteriorly or anterolaterally
usually painless unless fx is present
What is the xray finding associated with ossifying fibroma?
Well-circumscribed intracortical lesion
Cortex often is expanded and thinned, with multiple radiolucencies (lytic appearing)
What is the management for asymptomatic ossifying fibroma?
Repeat x-rays every 6 months if asymptomatic
What is the management for symptomatic ossifying fibromas? What is the prognosis, give both before and after skeletal maturity
Resection, curettage & bone grafting can be done AFTER skeletal maturity
If excision performed AFTER skeletal maturity it is generally CURATIVE
If excision is performed BEFORE skeletal maturity, there is a risk of recurrence
What is a nonssifying fibroma?
A benign, non-aggressive tumor that consists mainly of fibrous tissue
**What is the MC benign bone lesion in children?
nonossifiying fibroma
What is the presentation of nonossifying fibroma? What can larger lesions lead to?
Most are asymptomatic and found incidentally on x-ray
Larger lesions may lead to bone weakness leading to fracture
What are the 3 common sites for nonossifying fibromas?
Distal femur, distal and proximal tibia
What will nonossifying fibromas appear like on xray?
Small, well-defined, eccentric (NOT centrally located), lytic lesions
Generally, in the distal diaphysis/metaphysis and multiple lesions may be present
What should you order next in nonossifying fibromas?
CT/Bone Scan and Bx are NOT needed unless other diagnoses need to be ruled out
What is the management for asymptomatic nonossifying fibromas? What is the risk of recurrence?
Asymptomatic small NOFs do not require any intervention and they will generally fill in during adolescence
risk of recurrence is low
What are the 2 indications for surgical curettage and bone grafting in nonossifying fibromas?
Lesions > 50% of the bone diameter
Lesions in high stress area (distal femur)
What are the 2 types of cystic tumors?
unicameral bone cysts
aneurysmal bone cysts
What is a unicameral bone cyst? What age group? What are the 2 common locations?
Non-cancerous fluid filled lesions with fibrous lining
equal gender preference and between 5-15 years old
proximal humerus and proximal femur
How will a unicameral bone cysts present? What happens if the lesion occurs within a growth plate?
Asymptomatic until pathologic fracture occurs
growth plate dysfunction and limb length discrepancy
What will an unicameral bone cysts appear like on xray?
Well defined, cystic lesions at the metaphysis or metadiaphysis
Generally, involves the entire diameter of bone
Pathologic fx may have a “fallen leaf” or “fallen fragment” sign a fragment of bone falls to the bottom of the cyst
**What is a “fallen leaf” or “fallen fragment” sign? **What type of bone lesion is it associated with?
a fragment of bone falls to the bottom of the cyst
unicameral bone cysts
What happens to unicameral bone cysts after skeletal maturity?
Spontaneous resolution
What is the management for asymptomatic pts with unicameral bone cysts? What should you do if you are concerned about risk of ______?
Observation with serial x-rays every 4-6 months
Activity restrictions to avoid fractures
risk of fracture -> Aspiration of cyst and injection of methylprednisolone or bone marrow, which may hasten resolution of cyst
When do you use curettage and bone grafting in unicameral bone cysts?
Reserved for larger cysts that compromise the structural integrity of bone
What is an aneurysmal bone cysts? Usually single or multiple? What is the gender preference? What age group?
A benign, rapidly growing and destructive blood-filled lesion
usually solitary
Females > males FEMALES!!
adolescents
What are the top 3 MC sites for aneurysmal bone cysts?
tibia
femur
posterior vertebral elements
What is presentation of aneurysmal bone cysts?
Localized pain, tenderness & swelling
can limp, can be neuro s/s if in the spine, can stunt growth if at the growth plate
What will an aneurysmal bone cysts appear like on an xray?
Aggressive, expansile, lytic metaphyseal lesion with sharp borders
“Eggshell” sclerotic rim
“Soap bubble”
What type of bone lesions are “eggshell” sclerotic rim and “soap bubble” appearance associated with?
ANEURYSMAL BONE CYSTS
Why is it called a “soap bubble” apperance?
“Soap bubble” appearance due to the reinforcement of the remaining trabeculae that support the bone structure
What is the management of aneurysmal bone cysts? What is done before?
Excision, curettage & bone grafting is recommended
Adjuvant chemical cauterization or cryotherapy to ensure entire lesion is removed
Selective arterial embolization before surgery to prevent hemorrhage
aka these you need to treat!!!
What is the prognosis for aneurysmal bone cysts? What is the recurrence rate?
Lesions may continue to grow if left untreated
Lesions may recur even after excision in 10-50 % of cases
What are the 3 kinds of primary malignant bone tumors?
osteosarcoma
chondrosarcoma
ewing sarcoma
What causes an osteosarcoma? What location of the bone? What are 3 common sites?
Arise from an overgrowth of malignant osteoblasts
MC at the metaphysis of long bones
distal femur, proximal tibia, proximal humerus
What is the MC malignant bone tumor? Why does it MC occur at the metaphysis of long bones?
osteosarcoma
Most cell division happens at these sites
What are the 2 peaks of incidence of osteosarcoma? What gender preference?
Early adolescents
Adults over 65 y/o
Males > females
What are the risk factors for osteosarcoma?
Prior irradiation or chemotherapy
Paget disease, fibrous dysplasia, and other benign bone lesions
Genetic predisposition
What is the usually the first and MC symptom of osteosarcoma? What makes it worse? Will they have systemic symptoms?
pain and swelling
Pain may be worsened with activity
may have palpable mass and limping
NOT usually present
What will the xray show of an osteosarcoma?
Both osteolytic and/or osteoblastic lesions can be seen
“moth eaten” or “starburst” appearance
Codman’s Triangle may be present
What am I? What does it mean? What type of bone lesion?
“starburst” appearance
If the tumor extends through the periosteum a spiculated reaction occurs leading to a “starburst” appearance
osteosarcoma
What is the blue arrow pointing at? What does it mean? What type of bone lesion?
Codman’s Triangle → new bone formation at periosteum
osteosarcoma
What should you order next in osteosarcoma?
Bone scan
Utilize after identifying initial lesion to look for multifocal or metastatic disease
In osteosarcoma, fter xray and bone scan, should order ____ and _____. Give rationale for each
CT and MRI
CT - best for defining bone destruction & the pattern of calcification
MRI - better for defining intramedullary & soft tissue extension
What is the management for osteosarcoma? What is NOT used in the tx plan?
refer to onco orthopedist
Core needle or open biopsy for diagnostic confirmation
Pre- and post-op CHEMO with limb sparing surgery
Tumors are typically radioresistant, so radiation has LITTLE indication in therapy
What is a chondrosarcoma? What age range? What are the 2 MC sites?
A tumor arising from chondrocytes (cartilage producing cells)
MC after 50 y/o
pelvic and shoulder girdle
What is Ollier Disease? What is it a risk factor for?
Ollier Disease (Enchondromatosis) → a cluster of enchondromas that affect the hands
chondrosarcoma
What is the presentation of chondrosarcoma?
Deep, dull aching pain that is gradually progressive
Worse at night
What is the xray findings associated with chondrosarcomas? How big are the lesions?
The bony contour appears to be thinned & expanded
Multiple surface erosions can be seen → “endosteal scalloping”
Lesion is usually > 5 cm
What is the blue arrow pointing at? What type of bone lesion is it associated with?
“endosteal scalloping”
chondrosarcoma
chondrosarcomas: ______ performed preoperatively to assess the extent of the tumor. ______ to assess for mets to the lungs
MRI with gadolinium
CT chest with contrast
Where is the MC metastatic location of chondrosarcomas?
lungs
What is the management of chondrosarcomas? When is amputation indicated?
refer for surgical excision
amputation is rarely needed but only done if advanced disease or recurrent
What is an Ewing sarcoma? Why does it occur? What is the MC pt population?
A rare, peripheral primitive neuroectodermal tumor that can proliferate in the bone and/or soft tissue
Results from a translocation between chromosomes 11 and 22
adolescent males
Where are the 2 MC sites for Ewing sarcoma? What is the presentation?
pelvis and femur
Localized pain & swelling
Pain may be worse at night or with activity
Palpable mass
may have neuro deficits if spinal cord or peripheral nerve compression
What are the xray findings associated with Ewing Sarcoma?
Poorly marginated destructive lesion
“Onion skin” appearance
What is the management of Ewing sarcoma? When is radiation used?
Multi-drug chemotherapy with local disease control including surgery &/or radiation
Radiation is used if full resection of tumor is not possible
What are good prognostic factors for Ewing sarcoma?
Younger age, no metastasis, tumors outside of axial skeleton
What are the top 5 metastatic cancers?
breast
lung
thyroid
kidney
prostate
What are 3 signs that would make you think cancer has spread to the bones?
pain over metastatic location
pathologic fractures
anemia
What is the general treatment plan for metastatic bone disease?
Most common approach is radiation and pain medication
Chemo
Bisphosphonates
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