Exam 1 Pt2. Bone Tumors Flashcards
T/F. Bone tumors are most commonly malignant, asymptomatic, and slow growing.
False. Bone tumors are MC benign (asym. and slow growting), but can be malignant too
What are risk factors for low back pain of cancerous origin?
- age 50 or older
- LBP not relieved with rest or wakes one during night
- Personal history of CA
- LBP lasting longer than 1-month duration
- Cachexia
What is the MC benign bone tumor?
osteochondroma
What are the three MC primary cancerous bone tumors?
- osteosarcoma
- chondrosarcoma
- Ewing sarcoma
In what ages are bone tumors MC benign? What about malignant?
if develop 0-30, MC benign, if develop in older adults, MC malignant
What are some risk factors for Bone tumors?
- mutations in RB or TP53 genes
- inflammation (AVN, hardware, etc.)
Where are bone tumors MC?
long bones of extremeties
If a bone tumor is cancerous what are some symptoms?
fever, fatigue, cachexia
What are two very important aspects to take into consideration when narrowing down the type of bone tumor?
Age and location, also radiographic appearance
Ex: age 10-20 at knee = osteosarcoma
T/F. Bone marrow is a common site for cancer mets; therefore, when cancer is discovered within the bone, more likely to be cancer that mets to bone.
True
What are the three ways we are categorizing bone tumors?
- Bone-forming tumors
- Cartilage forming tumors
- Tumors of unknown origin
_______ will produce bony tissue with variable levels of mineralization, most likley with poorly-mineralized osteoid and mineralized woven bone.
Bone-forming tumors
T/F. Osteoid Osteoma and Osteoblastoma are both malignant.
False, they are both BENIGN, but differ in size
What type of bone-forming tumor is benign and smaller than 2cm and create a “nest-like” appearance?
Osteoid osteomas
What sex and age group is most likely to get an osteoid osteoma?
males 2x likely; ages 10-20
T/F. Osteoid osteomas are round/oval shape are pain is not localized and not relieved by aspirin.
False, yes, they are oval shape, BUT the pain is well-localized and IS relieved by aspirin
Where do Osteoid osteomas MC develop?
cortex of bones in lower extremities; metaphysis of femur and tibia
What is an osteoblastoma?
a benign, bone-forming tumor, at least 2cm are larger
Where are osteoblastoma MC located and what type of pain occurs?
MC in vertebral column–locally destructive; and poorly-localized pain that is Unrelieved by aspirin
What age range are osteoblastomas MC? Whats the Tx?
MC ages are 10-20 years (same as osteoid osteomas); Tx as if cancer due to dangerous area on spine…excision
What is a malignant, bone-forming tumor that commonly occurs ages 10-20?
osteosarcoma; 75% of all cases occur prior to age 20
If osteosarcoma occurs in an adult, what are the likely the possible causes?
= “secondary”; >40 years
- Paget Disease
- AVN
- ionizing radiation
T/F. Females make up about 60% all cases of osteosarcomas.
false, males make up the 60%
Where are osteosarcomas MC in the body?
metaphyseal region of long bones in extremities
When an ostoesarcoma develops in a characteristic adolescent, where is the location?
ages 10-20 yrs; within metaphyseal region of distal femur or proximal tibia = ~60% all osteosarcomas
What are other common locations of osteosarcomas (other than knee)?
- proximal femur/pelvis (15%)
- proximal humerus (10%)
- mandible or facial bones (8%)
What type of pain is experienced with osteosarcomas?
painful tumors; increase pain freq, and pain intensity as tumor enlarges and invades
What is the MC place of osteosaroma to mets to?
lungs; pulmonary mets is present in ~20% of osteosarcomas when initially diagnosed
What are the x-ray appearances of osteosarcomas?
- Codman Triangle
- large bony lesion with destructive appearance/ cortical destruction, “starburst”
- osteosclerotic (brighter white) over osteolysis (darker regions) = “mixed” lytic
When osteosarcomas start to invade where do they “exit/invade”?
the cortex of bone–> called periosteal reaction
What are periosteal reactions? What does one assume when they see one?
they develop following periosteal irritation from non-invasive or invasive lesions of bone; assume aggressive CA, until proven otherwise
Describe the appearance of Codman triangle on an x-ray. What is an indication we can assume from seeing this on an x-ray?
raised triangular “shadow” between the cortex of the bone and original cortical bone; Indicates the tumor is invading through the cortex and is behaving aggressively
What gene mutations are common in someone who has osteosarcoma? What will increase an inds. risk by 1000x of getting osteosarcoma?
RB and TP53 mutations (tumor suppressor genes); retinoblastoma syndrome (cancer in retina)
Individuals over 40 who get osteosarcoma, how is there survival rate?
typically it is lethal
What are the three main Cartilage-Forming Bone tumors?
- Osteochondroma
- Enchondroma
- chondrosarcoma
Which is MC, benign or malignant cartilage-forming bone tumors?
benign
What is a cartilage-bone forming tumor that is benign and also called “exostosis”, typically projecting out from bony cortex with a cartilaginous cap?
osteochonroma
(exostosis = “sticks out”
Osteochondromas can manifest in two ways. What are those two ways?
pedunculated mass = narrow base
sessile mass = broad base
Are osteochondromas MC inherited or sporadic?
- MC sporadic (85%)
- inherited (15%) and involve multiple tumors
What are the two ways that cartilage-bone forming tumors produce when they grow?
bony hyaline cartilage = clear, glass-like
OR
myxoid cartilage = mucus like
What is it called when an individual has multiple osteochondromas and it is inherited?
Multiple hereditary exostoses
- (usually mutated EXT1 or EXT2)–> dyregulated cartilage growth
What are the chances that osteochondroma transitions into cancer? What cancer would it transition into?
rare; <1% transition risk into chondrosarcoma
Where are osteochondromas discovered?
in metaphyseal region, near growth plate in long bones of lower extremities; project from cortex
What is the MC place for an osteochondroma to manifest? Where else can it manifest?
MC at the knee
- also as pelvis, scapula, ribs, hand/feet
What is the MC age for osteochondromas to be discovered?
ages 10-30; adolescent or young adulthood
T/F. Osteochondromas are usually very painful.
False, they are usually asymptomatic unless mass Fx, compresses soft tissue, or can be palpated through skin
Are osteochondromas MC in males of females?
males, 3-1 ratio
What type of benign cartilage-forming tumor manifests as a solitary radiolucent lesion within tubular bones of hands and feet?
enchondroma (chondroma)
When are enchondromas typically diagnosed?
ages 20-50; therefore is a benign tumor of adulthood
Are enchondromas MC in males or females?
2x as likely in males
Where do enchondromas MC manifest?
in proximal phalanges (40-50%) and metacarpals (305)
How are enchondromas diagnosed?
typically asymptomatic and detected incidentally; larger ones may weaken bone and cause pathological Fx
What type of bone tumor manifests with “nodules” of bluish-white hyaline cartilage with well-circumscribed lesions?
enchondromas
What is the then zone surrounding an enchondroma’s circumscribed lesions called?
O-ring sign
What is the central site of an enchondroma likely to form?
AVN, may cause irregular areas of opacification due to stimulated dystrophic calcification
What is the management of enchondromas?
- smaller, asymptomatic ones = no Tx yet
- larger or ones that caused pathological Fx = are removed
What is Ollier Disease? How common is it?
(enchondromatosis); involves formation of multiple large enchondromas, casing severe bony deformation; rare at 1 in 100,000 inds.
Enchondromas are named based on location on bone. What are the two ways? Which is rare?
Enchondroma = medullary
Juxtacortical chondroma = cortical; RARE
What is the 2nd MC primary bone cancer?
chondrosarcoma
MC is osteosarcoma
X-ray of a 50 year old man with a cartilage-forming bone tumor in proximal humerus that has a “popcorn” appearance. What are you thinking it is?
chondrosarcoma
What age group are chondrosarcomas most likely found in? Which sex?
ages 40-60
men 2x more likely
What type of cartilage do chondrosarcomas produce?
hyaline and myxoid cartilage
Where are chondrosarcomas most likely to develop?
bones of pelvis, proximal femur, proximal humerus, sacpula, ribs, sternum
(RARE to develop in distal extremities)
Radiographically, what tumor has “stippled” or “popcorn” calcifications?
chondrosarcoma
What does gross inspection of a chondrosarcoma look like?
large mass with a “glistening gray-white” or “translucent” cartilage
T/F. Chondrosarcomas MC develop Juxtacortical.
False. MC develop intramedullary
Are low-grade or high-grade chondrosarcomas more common? Describe it.
low-grade are MC; smaller, slow-growing (indolent) and thicken surrounding cortical bone
What is the prognosis for low-grade and high-grade chondrosarcomas?
low-grade = favorable, 90% 5 year survivability
high-grade = poor prognosis, 40% 5 year survivability
A chondrosarcoma on an x-ray that appears to have increase radiolucency will increase the chance of it being high or low-grade? Why?
high-grade, because eroding away the cortex
low-grade thicken cortical bone
Where is the MC place a high-grade chondrosarcoma will mets to? Via what way?
MC to lungs
or to other bone
via invasion of surrounding medullary structures or hematogenously
Which cartilage-forming tumor is more likely to destroy a joint?
chondrosarcoma
What are the bony lesions called that have been described as benign tumors of neoplastic fibroblasts or macrophages? What are they being described as more recently?
Fibrous cortical defects (FCD) and Nonossifying fribromas (NOF)
more of a reaction to trauma at the area of the periosteal/cortical reaction
What part of bone are Fibrous cortical defects (FCD) and Nonossifying fribromas (NOF) most likely to involve?
metaphysis or diaphysis of lower extremity near cortex
- tibia or femur (MC at knee)
How common are Fibrous cortical defects (FCD) and Nonossifying fribromas (NOF)?
very common, may be found in up to 40% of skeletally immature children
What is the difference between Fibrous cortical defects (FCD) and Nonossifying fribromas (NOF) ?
they are bony lesions that are histologically identical
FCD = smaller NOF = larger (>3cm)
What is the x-ray appearance of Fibrous cortical defects (FCD) and Nonossifying fribromas (NOF)?
well-defined radiolucent lesion with thin sclerosis
What can a larger Nonossifying fribromas (NOF) cause?
predispose ind to pathologic fx, by creating a large area of structurally-weak bone
What is a non-neoplastic alteration in bone, and is localized bony differentiation, commonly called “arrested development” bone?
Fibrous Dysplasia (3 clinical manifestations)
What does Fibrous Dysplasia involve?
involves replacing normal bone with fibrous stroma and areas of irregular woven bone
What do all three cases of Fibrous Dysplasia originate from?
a congenital embryological GNAS gene mutation, spontaneously
What are the three ways Fibrous Dysplasia can clinically manifest?
- Monostotic Fibrous Dysplasia (MC)
- Polyostotic Fibrous Dysplasia
- McCune- Albright Syndrome
What is the MC type of Fibrous dysplasia?
Monostotic fibrous dysplasia (~70%)
When is Monostotic fibrous dysplasia usually discovered? Bones most likely involved?
10-30 years old; asymptomatic, little bony distortion
ribs, femur, calvaria, mandible, or humerus
What does Polyostotic fibrous dysplasia usually manifest like?
- multiple bones
- more severe than monostotic FD
- severe bony deformation (enlargement)
What type of FD radiographically appears as a “ground glass” appearance, and may have osseous distortion described as a “shepherd crook”?
Polyostotic fibrous dysplasia
What bones is Polyostotic fibrous dysplasia likely to involve?
- craniofacial bones (50% cases)
- proximal femur
- pelvis
- humerus
- tibia
What age is Polyostotic fibrous dysplasia most likely detected?
late childhood/ adolescence
If someone manifests with polyostotic fibrous dysplasia, endocrine hyperfunctioning, and cafe au lait skin lesions, what do they have?
McCune-Albright Syndrome
least common of FD at 3%
T/F. The polyostotic fibrous dysplasia that manifests in McCune-Albright Syndrome is usually more severe than when it manifests as just Polyostotic fibrous dysplasia alone.
False, it is usually less severe
What may the endocrine hyperfunctioning caused in McCune-Albright syndrome develop from?
- pituitary adenoma
- adrenal hyperplasia
- hyperplasia of thyroid gland
(is early-onset puberty)
Describe the appearance of the cafe au lait skin lesions ass. with McCune-Albright syndrome.
- commonly unilateral (on one side of body)
- fairly large
- “coast of Maine”/ irreg. jagged boarders
What is the 2nd MC pediatric bone cancer?
What percentage does it represent of all bone cancers?
Ewing sarcoma
10%
What is a malignant primary bone tumor composed of primitive (undifferentiated) “small round cells”?
Ewing sarcoma
What cancer MC affects ages 10-20, males, 9x more likely in causasians, and appears at diaphysis of extremity long bones?
Ewing sarcoma
If one has bone cancer that mimics symptoms of an infection and is a painful and enlarging mass, what do we think it could be?
Ewing sarcoma
- area is warm, tender, swollen
- fever
- increase WBCs
- elevated ESR; “sed rate”
What type of bone cancer has a characteristic “onion-skin” appearance?
Ewing sarcoma
Where is Ewing sarcoma most likely to originate from? What does it “do” to bone?
medullary cavity of bone and freq. causes osteolytic area of destruction; that may invade surrounding soft tissues
What type of radiographic appearance can Ewing sarcoma have that mimics osteosarcoma? How to tell them apart?
“sunburst” appearance
Ewing sarcoma– diaphysis
Osteosarcoma– metaphyseal region
T/F. Ewing sarcomas are usually aggressive and treated with surgical excision and chemo.
True
Ewing sarcomas has a _____ 5 year survival, and a ____ complete remission.
75% 5 year survival
50% complete remisison
What is another bone tumor that is nearly identical to Ewing sarcomas, except the cells that compose it contain a degree of what?
Primitive Neuroectodermal Tumor (PNET)
- cells have some degree of neuroectodermal differentiation and manifest with Homer-Wright (pseudo) rosettes
What bone tumor is an intermediate tumor, not clearly benign or malignant?
Giant Cell tumor of Bone
What bone tumor is named for the presence of multinucleated “giant cells”? How does it manifest?
Giant Cell tumor of Bone
- locally invasive
- create large area of osteolysis
- freq. cause bony expansion/distortion
What do Giant Cell tumors of bone typicallly occur?
at the metaphyseal and epiphyseal areas of long bones in extremities
What age group are Giant Cell tumors of bone exclusive to? What are the common locations of manifestation?
adults– ages 20-40
MC at knee
- distal femur
- prox. tibia
- distal radius
- sacrum
- proximal humerus
Radiographically, what bone tumor manifests as an isolated osteolytic mass that is eccentric of an extremity long bone?
Giant Cell tumor of bone
What radiographically appearance is typically of Giant Cell tumors of bone?
thin “shell of reactive bone” surrounding bony lesion and freq/ destroy cortical bone and cause bulging tissue mass
“soap-bubble” appearance
Gaint cell tumors of bone MC develop near a joint, so what do they cause?
- decrease ROM
- arthritis-like pain
- pathological fx
What percentage of Giant cell tumors of bone return following curettage?
What percent mets and to where?
50% return
2-4% mets to lungs
Cancer in the bone is most commonly due to what?
metastasis (mets to bone is MC than primary bone CA)
What is the most common area for cancer to mets in bone?
spine
Of the cancer that mets to bone, which ones make up over 75% of adult bony mets?
- prostate
- breast
- kidney
- lung
T/F. Skeletal mets is most likely to affect a single bone.
False; most likely to be multifocal (multiple sites)
Presence of secondary bone tumor indicates what about condition of ind.?
very poor prognosis; cancer in advanced stage (stage IV)
What are the three ways skeletal mets may appear? Which is MC?
- osteolytic (dark)
- osteoblastic (whitish)
- “mixed” lytic and blastic (MC)
What is indicative of these cancers when they mets to bone:
- prostate
- breast
- lung
- osteoblastic (hence ivory vert. sign)
- osteolytic
- “mixed” osteolytic/osteoblastic
What cancer is most commonly ass. with skeletal mets in children?
- neuroblastoma
- Wilms tumor
- osteosarcoma
- rhabdomyosarcoma
- Ewing sarcoma
What are “red flags” for low back pain of cancerous origin?
- > 50 years old
- history of CA
- cachexia
- LBP unrelieved with rest
- pain lasting over a month
- failure to improve after a month of care
What is the sensitivity and specificity for an ind. what is older than 50, has history of CA, and has cachexia, to have skeletal mets?
100% sensitive
60% specific
