Malignant Neoplasia of Bone Flashcards
Primary malignant neoplasia of bone tissues account for ___% of all bone malignancy in the U.S.
<30%
What are the most common primary malignancies of bone?
Bone forming tumors: osteosarcoma
Cartilage forming tumors: chondrosarcoma
Tumors of other origin: Ewing sarcoma, multiple myeloma
Secondary malignant neoplasia of bone tissues accounts for ___% of all bone malignancy in the U.S.
70%
What would secondary malignant neoplasia of bone tissues be secondary to?
Metastatic disease
Osteosarcoma accounts for ___% of primary bone cancers
20%
What is the most common primary bone tumor in children?
Primary osteosarcoma (75% in individuals younger than 20 years)
What kind of osteosarcoma is more common in older adults with predisposing conditions such as Paget’s, previous radiation therapy, or bone infarcts?
Secondary osteosarcoma
Which bones are most commonly affected by primary osteosarcoma?
Metaphyseal region of long bones; knee accounts for 50% of cases
Which bones are most commonly affected by secondary osteosarcoma?
Flat bones
Which genes are mutated in the pathogenesis of osteosarcoma?
Tumor suppressor and oncogenes:
- RB
- TP53
- INK4a
- MDM2 and CDK4
osteosarcoma
What is the RB gene?
Tumor suppressor gene; negative regulator of cell cycle
osteosarcoma
What is the TP53 gene?
Tumor suppressor gene; encodes p53 which plays an important role in promoting DNA repair and apoptosis of damaged cells
osteosarcoma
What is the INK4a gene?
Gene that encodes 2 tumor suppressors (p16 and p14)
osteosarcoma
What are the MDM2 and CDK4 genes?
Inhibit p53 and RB function; overexpressed in many osteosarcomas
Is osteosarcoma blastic or lytic?
Mixed blastic and lytic mass; formation of bone by tumor is diagnostic in biopsy
Osteosarcoma features ___ margins
irregular
How might periosteum be affected by osteosarcoma?
May lift or penetrate periosteum; reactive bone formation
Does osteosarcoma invade other tissues?
Yes, frequently metastasizes to the lung, brain, or other bones
What is the 5-year survival rate for osteosarcoma?
60%
What are clinical manifestations of osteosarcoma?
- Pain and swelling around the site of the tumor
- Possibly palpable tumor
- Pathological fracture
- Abnormal labs
What abnormal lab findings are present with osteosarcoma?
- Increased ESR/C-reactive protein
- Increased alkaline phosphatase
If the following radiographic characteristics are found, what is suspected?
- Pathological fracture
- Radiopaque mass with spiculated pattern
- Periosteal lifting/disruption in Codman’s triangle pattern
- Long zone of transition
Osteosarcoma
What are the radiographic characteristics of osteosarcoma?
- Pathological fracture
- Radiopaque mass with spiculated pattern
- Periosteal lifting/disruption in Codman’s triangle pattern
- Long zone of transition
What is a malignant bone forming tumor?
Osteosarcoma; produces unmineralized osteoid or mineralized woven bone
What is a malignant cartilage forming tumor?
Chondrosarcoma
Chondrosarcomas are subclassified by ___
tissue and location
The conventional type of chondrosarcoma is most common
What cartilage is produced by this cancer?
Hyaline cartilage
What are the classifications of chondrosarcoma by location?
- Central (intramedullary)
- Juxtacortical (peripheral)
At what rate do chondosarcomas grow?
Very slow growing
What histological differences can be found in chondrosarcoma?
- Malignant chondrocytes
- Necrosis
- Hemorrhage
- Stippled calcifications
- Cystic change
What are common bone locations for chondrosarcoma?
- Pelvis
- Ribs
- Long bones (especially femur and humerus)
At what ages are most chondrosarcomas diagnosed?
40-60 years of age
(exception: clear and mesenchymal subtypes in teens or 20s)
What is the distribution of chondrosarcoma among sexes?
Male > Female (2:1)
Where is chondrosarcoma most likely to metastasize?
- Lungs
- Liver
- Kidneys
- Brain
Depending upon differentiation, what is the 5-year survival rate for chondrosarcoma?
20-80%
What are the clinical manifestations of chondrosarcoma?
- Insidious onset; up to 2 years
- Pain at affected site
- Possible palpable soft tissue mass
If the following radiographic findings are present, what is suspected?
- Localized bone destruction
- Poorly-defined borders with cortex perforation
- Radiopacities due to calcification or endochondral ossification
- Soft tissue masses may be visible
Chondrosarcoma
What are the radiographic findings of chondrosarcoma?
- Localized bone destruction
- Poorly-defined borders with cortex perforation
- Radiopacities due to calcification or endochondral ossification
- Soft tissue masses may be visible
Drug and radiation therapy are ___ effective in treating chondrosarcomas than other malignant bone tumors
less
(cartilage isn’t well vascularized)
What is a cause of malignant tumors of bone marrow cells?
Multiple myeloma
What is the most common primary malignant bone tumor?
Multiple myeloma
What is the average age of diagnosis for multiple myeloma?
65 years of age (50-70)
What is the distribution of multiple myeloma among sexes?
Male > Female (2:1)
What is the etiology of multiple myeloma?
Unknown
Myeloma cells secrete ___ that recruit ___
cytokines that recruit osteoclasts
What type of bone lesions, lytic or blastic, are produced by multiple myeloma cytokine secretion?
Lytic bone lesions (osteoclasts recruited)
What is the size of multiple myeloma lesions?
Multiple 1-5mm lesions
Which bones are most frequently affected by multiple myeloma?
- Skull
- Spine
- Ribs
- Pelvis
- Femur
Histologically, multiple myeloma lesions consist of ___
sheets of plasma cells with varying degrees of differentiation
Which cardinal signs of inflammation are present in multiple myeloma?
Lack of cardinal signs
What are some clinical manifestations of multiple myeloma?
- Weight loss, cachexia (muscle loss, fat retained)
- Pain
- Hypercalcemia
- Anemia
What sort of pain presents with multiple myeloma?
- Often intermittent, becomes continuous
- Possible rapid onset following slight strain or mild trauma
- Worse during day, aggravated by activity and weight bearing
A patient reports the following about their pain:
- Rapid onset following slight strain or mild trauma
- At first, intermittent, then continuous
- Worse during day
- Aggravated by activity and weight bearing
Which malignant bone tumor is suspected?
Multiple myeloma
___ is seen in 10% of patients with multiple myeloma
Amyloidosis (amyloid deposits)
How does multiple myeloma present radiographically?
- Punched out lesions
- Rain drop skull
If a patient has production of serum and urinary proteins called Bence-Jones proteins, which malignant bone tumor is suspected?
Multiple myeloma
There is overproduction of ___ in 95% of multiple myeloma patients
immunoglobulins
Multiple myeloma may cause production of serum and urinary proteins called Bence-Jones proteins.
What may be the result of this?
Renal failure
What are treatment options for multiple myeloma?
Treatment options are still very poor
What is the average survival rate for multiple myeloma?
What is the common cause of death?
Average survival rate of 5 years
Infection or renal failure are common causes of death
What is a malignant tumor of neuroectodermal cells?
Ewing sarcoma
What is the second most common primary bone tumor in children accounting for up to 10% of primary bone tumors?
Ewing sarcoma
80% of Ewing sarcomas are diagnosed in individuals ___ years of age
<20 years of age
What is the distribution of Ewing sarcoma among sexes?
Males > Females (2:1)
What is the etiology of Ewing sarcoma?
Still poorly understood; 90% of cases show translocation between chromosomes 11 and 22
If histology presents as follows, what is suspected?
- Closely packed, small, round cells without obvious differentiation
- Arranged in uniform sheets
- Cells have little cytoplasm
Ewing sarcoma
What is the histology of Ewing sarcoma?
- Primitive neuroectodermal cells
- Closely packed, small, round cells, without obvious differentiation
- Arranged in uniform sheets
- Cells have little cytoplasm
___% of Ewing sarcoma cases are extraskeletal
10-20%
Ewing sarcoma arises in ___ and grows ___
arises in medullary cavity and grows outward
Which bones are commonly affected by Ewing sarcoma?
Femur, other long bones, and pelvis
(Mid-diaphysis of long bones)
If a patient presents with the following, which malignant bone tumor is suspected?
- Deep boring pain in enlarging masses
- Tender, warm, and or swollen site
- Mimicking infection: fever, increased ESR, leukocytosis
- Unexplained weight loss
- Weakness
- Anemia
Ewing sarcoma
What are some clinical manifestations of Ewing sarcoma?
- Deep boring pain in enlarging masses
- Tender, warm, and or swollen site
- Mimicking infection: fever, increased ESR, leukocytosis
- Unexplained weight loss
- Weakness
- Anemia
What are some treatments for Ewing sarcoma?
What is the 5-year survival rate?
Chemotherapy, radiation, and limb-sparing surgery
60-75% 5-year survival rate
If the following radiographic characteristics are present, which malignant bone tumor is suspected?
- Permeative lytic destruction
- Long zone of transition
- Cortical disruption
- Laminated or spiculated periosteal response
- May extend into soft tissue
Ewing sarcoma
What are the radiographic characteristics of Ewing sarcoma?
- Permeative lytic destruction
- Long zone of transition
- Cortical disruption
- Laminated or spiculated periosteal response
- May extend into soft tissue
Metastatic bone disease is also known as ___
secondary bone tumors
What is the most common malignant tumor of bone?
Secondary bone tumors (metastatic)
Metastatic bone tumors have most commonly metastasized from:
- breast
- prostate
- kidney
- lungs
Which components of the skeletal system are the most common sites of metastasis/metastatic tumors?
- Vertebrae
- Pelvis
- Ribs
- Skull
- Sternum
What are some clinical manifestations of metastatic bone tumors?
- Unexplained weight loss
- Anemia
- Possible fever with late-stage disease
- Abnormal labs
How do metastatic bone tumors present radiographically?
Osteoblastic, osteolytic, or mixed