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94 Cards in this Set

  • Front
  • Back
How common are bone tumors relatively?
- Relatively rare compared to carcinomas and hematopoietic tumors
- ~0.2% of all types of cancer
Are bone tumors potentially curable?
Yes, they represent an important percentage of potentially curable cancers following multimodal therapy
What is the general clinical presentation of bone tumors?
Nonspecific:
- Pain
- Mass
- Pathologic fracture
- Asymptomatic
What kind of tumor is characterized by severe pain that is worse at night? How can the pain be relieved?
Osteoid Osteoma - relieved by aspirin
What kind of tumor is characterized by being painless, with a hard growing mass in the popliteal fossa?
Parosteal Osteosarcoma
What are some diagnostic factors for bone tumors?
- Age
- Sex
- Skeletal localization (specific bone or specific area of bone)
- Radiographic appearance
How can bone tumors be distinguished based on their skeletal localization?
- Medullary cavity vs cortex vs juxtacortical
- Epiphysis (ends) vs metaphysis (growth plate) vs diaphysis (shaft)
- Which specific bone it is in
- Medullary cavity vs cortex vs juxtacortical
- Epiphysis (ends) vs metaphysis (growth plate) vs diaphysis (shaft)
- Which specific bone it is in
Which kind of bone tumors are more common in children?
- Osteosarcoma
- Ewing's sarcoma
Which kind of bone tumors are more common in young adults?
Giant cell tumor
Which kind of bone tumors are more common in the elderly?
Chondrosarcoma
Which kind of bone tumors are found in the diaphysis?
- Fibrous cortical defect
- Osteoid Osteoma
- Ewing's Sarcoma
- Enchondroma
- Chondrosarcoma
- Fibrous cortical defect
- Osteoid Osteoma
- Ewing's Sarcoma
- Enchondroma
- Chondrosarcoma
Which kind of bone tumors are found in the metaphysis?
- Osteochondroma
- Chondromyxoid Fibroma
- Osteosarcoma
- Osteoblastoma
- Osteochondroma
- Chondromyxoid Fibroma
- Osteosarcoma
- Osteoblastoma
Which kind of bone tumors are found in the epiphysis?
- Chondroblastoma
- Giant Cell Tumor
- Chondroblastoma
- Giant Cell Tumor
Which kind of bone tumors are found in the medullary cavity?
- Ewing's Sarcoma
- Enchondroma
- Chondrosarcoma
- Ewing's Sarcoma
- Enchondroma
- Chondrosarcoma
What is usually indicative of a benign, slowly growing bone neoplasm?
Sclerotic margin / rim (bone has time to heal and form a circumscribed capsule)
Sclerotic margin / rim (bone has time to heal and form a circumscribed capsule)
What is usually indicative of a malignant, rapidly growing bone neoplasm?
Ill-defined margin (bone has no time to heal and form a circumscribed capsule)
Ill-defined margin (bone has no time to heal and form a circumscribed capsule)
What radiologic finding is generally seen in malignant bone matrix-forming tumors?
Solid, ivory-like pattern
Solid, ivory-like pattern
What radiologic finding is generally seen in chondroid matrix-forming tumors?
Rings and arcs (cloud-like or popcorn-like appearance)
Rings and arcs (cloud-like or popcorn-like appearance)
What are the types of benign bone-forming tumors?
- Osteoid Osteoma
- Osteoblastoma
How do osteoid osteoma and osteoblastoma compare in the location of tumors?
- Osteoid Osteoma: long bones, femur, and tibia
- Osteoblastoma: vertebrae or long bone metaphysis
How do osteoid osteoma and osteoblastoma compare in the size?
- Osteoid Osteoma: < 2 cm
- Osteoblastoma: > 2 cm
How do osteoid osteoma and osteoblastoma compare in the amount of pain and the treatment of pain?
- Osteoid Osteoma: night pain especially, responds to aspirin
- Osteoblastoma: painful but not responsive to aspirin
How do osteoid osteoma and osteoblastoma compare in the radiologic findings?
- Osteoid Osteoma: radioluscent lesion w/in sclerotic cortex
- Osteoblastoma: expansile radio-lucency w/ mottling
What kind of neoplasm is this?
What kind of neoplasm is this?
Osteoid Osteoma - radiolucent (transparent) lesion within sclerotic cortex of diaphysis (benign because well circumscribed)
Osteoid Osteoma - radiolucent (transparent) lesion within sclerotic cortex of diaphysis (benign because well circumscribed)
What kind of neoplasm is this?
What kind of neoplasm is this?
- Osteoid Osteoma - will-cricumscribed in sclerotic bone (thickened)
- Benign because well-circumscribed
- Found in diaphysis, < 2 cm
- Osteoid Osteoma - will-cricumscribed in sclerotic bone (thickened)
- Benign because well-circumscribed
- Found in diaphysis, < 2 cm
What kind of neoplasm is this?
What kind of neoplasm is this?
Osteoblastoma (found in vertebrae, >2cm)
Osteoblastoma (found in vertebrae, >2cm)
What are the types of malignant bone-forming tumors?
Osteosarcoma
What kind of neoplasm is an Osteosarcoma? Where does it occur?
- Malignant, mesenchymal tumor in which cells produce osteoid or bone
- Metaphysis of long bones (femur, tibia, humerus (56%); flat bones, spine (older patients))
- Hematogenous (via blood) spread to lungs
- Malignant, mesenchymal tumor in which cells produce osteoid or bone
- Metaphysis of long bones (femur, tibia, humerus (56%); flat bones, spine (older patients))
- Hematogenous (via blood) spread to lungs
What is the most common sarcoma of bone?
Osteosarcoma (35%)
How common is Osteosarcoma? Who does it affect most?
- ~2000 cases/yr
- Most common sarcoma of bone
- M > F
- 60% ages 10-20; second peak 55-80
What can predispose someone to getting Osteosarcoma?
- Inherited mutant allele of RB gene (retinoblastoma) - 1000x higher in OS
- Mutation of p53 suppressor gene (Li-Fraumeni)
- Overexpression of MDM2 (5-10%); INK4 and p16
- Sites of bone growth/disease (Paget dz)
- Prior irradiation
What are the characteristics of an Osteosarcoma neoplasm?
- Poorly deliminated (malignant)
- Bone destruction
- Cortical disruption
- Soft tissue extension
- Codman's triangle (triangular area of new subperiosteal bone that is created when a lesion raises the periosteum away from bone)
- Very ivory ...
- Poorly deliminated (malignant)
- Bone destruction
- Cortical disruption
- Soft tissue extension
- Codman's triangle (triangular area of new subperiosteal bone that is created when a lesion raises the periosteum away from bone)
- Very ivory = radio dense
What kind of neoplasm is this?
What kind of neoplasm is this?
Osteosarcoma:
- Poorly delimited neoplasm, bone destruction, cortical disruption
- Codman's triangle (raised periosteum away from bone d/t new subperiosteal bone)
- Soft tissue extension
- Starts at metaphysis / medullary cavity of long bone (...
Osteosarcoma:
- Poorly delimited neoplasm, bone destruction, cortical disruption
- Codman's triangle (raised periosteum away from bone d/t new subperiosteal bone)
- Soft tissue extension
- Starts at metaphysis / medullary cavity of long bone (femur)
What is the prognosis for Osteosarcoma?
- Prior to 1970 (before chemo), 5 yr survival 20%
- Most relapsed in lung w/in 6 months after primary amputation
- Metastases to lung, pleura, other bones, CNS
- Post chemotherapy: 60-65% 3-5 yr survival (for non-metastatic dz)
- En-bloc resection (surgery of entire neoplasm) following chemo: >90% necrosis --> near 90% survival
What are the benign cartilaginous tumors?
- Osteochondroma
- Enchondroma
- Multiple chondromatosis (Ollier's disease and Maffucci's syndrome)
What is the most common benign tumor of bone?
Osteochondroma
What kind of neoplasm is Osteochondroma? Where does it affect?
- Benign cartilaginous tumor of bone (malignant transformation is rare, <1%, but increased risk in hereditary multiple osteochondromas)
- Metaphysis of long bones
How can Osteochondroma be inherited? Implications of hereditary version?
- Autosomal dominant - most commonly secondary to mutations in EXT-1 (8q24)
- Multiple osteochondromas --> increased risk (~40%) of malignant transformation
What is the progression of formation of Osteochondromas?
- Growth at cartilaginous growth plate
- Forms osteochondroma
- Migrates and connects to medullary cavity
- Growth at cartilaginous growth plate
- Forms osteochondroma
- Migrates and connects to medullary cavity
What kind of neoplasm is this?
What kind of neoplasm is this?
Osteochondroma
Osteochondroma
What kind of neoplasm is an Enchondroma?
- Benign hyaline cartilage lesion
- Intramedullary chondroma
What kind of neoplasm is Periosteal Chondroma?
- Benign hyaline cartilage lesion
- Juxtacortical chondroma (located on cortical surface under periosteum)
What are the symptoms of an Enchondroma? Where does it affect?
- Usually asymptomatic, incidental finding
- Appendicular skeleton; small bones of hands and feet
What does an enchondroma look like on X-ray?
Lytic, lobulated, cortical thinning
What does an enchondroma look under microscope?
Lobules of hyaline cartilage, minimal atypia
How are enchondroma treated?
- No treatment unless lesion shows changes:
- Symptomatic (onset of acute pain = malignant)
- Evidence of recent growth after skeletal maturity
What kind of neoplasm is this?
What kind of neoplasm is this?
Enchondroma (lytic lesion surrounded by fibrous capsule, benign, found in small bones of hands, which is common)
Enchondroma (lytic lesion surrounded by fibrous capsule, benign, found in small bones of hands, which is common)
What kind of neoplasm is this?
What kind of neoplasm is this?
- Enchondroma (lytic lesion surrounded by fibrous capsule, benign, found in small bones of hands, which is common)
- Shiny, white appearance indicative of hyaline cartilage
- Enchondroma (lytic lesion surrounded by fibrous capsule, benign, found in small bones of hands, which is common)
- Shiny, white appearance indicative of hyaline cartilage
What would cause mulitple chondromas ("multiple chondromatosis")?
Frequent point mutations in IDH1 or IDH2 (isocitrate dehydrogenase)
What are the types of multiple chondromatosis?
- Ollier's disease
- Maffucci's syndrome
What are the characteristics of Ollier's disease?
- Multiple enchondromata
- Regional distribution
- ± Severe skeletal malformation
What are the characteristics of Maffucci's syndrome?
- Multiple enchondromata + angiomata
- Severe skeletal malformation
- Higher incidence of malignant transformation
What is the malignant cartilaginous tumor?
Chondrosarcoma
What kind of neoplasm is a Chondrosarcoma?
Malignant tumor in which neoplastic cells produce a purely cartilaginous matrix
What kind of malignant tumor has cells that produce bone and cartilage? Just bone? Just cartilage?
- Both = osteosarcoma
- Just bone = osteosarcoma
- Just cartilage = chondrosarcoma
What is the second most common bone sarcoma? Who is affected by them?
Chondrosarcoma; mainly older adults (>40-50 years); peaks during 6th-7th decades
Where are chondrosarcomas commonly located?
- Central skeleton: pelvis and ribs (45%); humerus, femur (metaphysis and diaphysis)
- Medullary location originally, but cortical erosion or destruction too and occasional soft tissue extension
How do chondrosarcomas appear on imaging?
- Ring and arc appearance (popcorn-like)
- Frequently present calcifications, which tend to be lost in grade 3 tumors
- Ring and arc appearance (popcorn-like)
- Frequently present calcifications, which tend to be lost in grade 3 tumors
What kind of neoplasm is this?
What kind of neoplasm is this?
Chondrosarcoma:
- Ring and arc appearance = cartilage
- Started in medulla (shiny white) --> spreads and destroys cortex --> spreads to periosteum and surrounding soft tissues
Chondrosarcoma:
- Ring and arc appearance = cartilage
- Started in medulla (shiny white) --> spreads and destroys cortex --> spreads to periosteum and surrounding soft tissues
What kind of cellular changes in Chondrosarcomas?
- More cellular than enchondromas
- More pleomorphic nuclei than enchondromas
- Binucleation is frequent (not sufficient for malignant diagnosis)
- Myxoid change of chondroid matrix (mucousy)
How many nuclei/lacuna in benign cartilaginous neoplasms? In malignant cartilaginous neoplasms?
- Benign = 1 nuclei/lacuna
- Malignant = 2 nuclei/lacuna
How does the size of the neoplasm affect the outcomes of Chondrosarcoma?
If the neoplasm is >10 cm there are worse outcomes
What is the 5 year survival for chondrosarcoma?
- Grades 1,2 = 80-90%
- Grade 3 = 29% (pulmonary metastases)
What are the variants of Chondrosarcoma?
- De-differentiated
- Myxoid
- Clear cell
- Mesenchymal
- Juxtacortical
What are the types of Fibrous Bone Tumors?
- Non-Ossifying Fibroma
- Fibrous Dysplasia
What is the most common space-occupying lesion of bone? How common?
Non-ossifying fibroma (1 in 4 individuals)
Where do Non-Ossifying Fibromas typically occur? When?
- Tibia, Femur (metaphysis)
- 1st-3rd decades
What are the characteristics of Non-Ossifying Fibromas? How are they discovered?
- Eccentric, lytic, peripheral sclerosis
- Incidental finding or pathological fracture
- Eccentric, lytic, peripheral sclerosis
- Incidental finding or pathological fracture
What type of bone tumor looks like "starry night" histologically?
What type of bone tumor looks like "starry night" histologically?
Non-Ossifying Fibroma
What is Fibrous Dysplasia?
Benign tumor in which all components of normal bone are present but they fail to differentiate into mature structures (developmental arrest)
What are the types of Fibrous Dysplasia? Which are more common?
- Monostotic = most common
- Polyostotic
- McCune-Albright Syndrome = least common
Who is most likely to get the different types of Fibrous Dysplasia? Where?
- Monostotic = adolescents - ribs, mandible, or femur
- Polyostotic = infancy/childhood - crippling deformities, craniofacial involvement common
- McCune-Albright Syndrome = polyostotic = F > M
What is McCune-Albright Syndrome?
- Polyostotic Fibrous Dysplasia
- Endocrinopathies - sexual precocity, acromegaly (excess growth hormone), Cushing syndrome
- Cafe-au-lait spots on skin
- Affects more F > M
What is the cause of McCune-Albright Syndrome?
Activating germline mutations of GNAS (GTP-binding protein) resulting in excess cAMP --> endocrine gland hyperfunction
What kind of neoplasm is this?
What kind of neoplasm is this?
Fibrous Dysplasia
- Expansile
- Circumscribed
- Thinned cortex
- "Ground glass" appearance
Fibrous Dysplasia
- Expansile
- Circumscribed
- Thinned cortex
- "Ground glass" appearance
What are the characteristics of Fibrous Dysplasia lesions?
- Haphazard, curvilinear, randomly oriented woven bone trabeculae
- Looks like "Chinese characters"
- Surrounded by fibroblastic stroma
- No significant osteoblastic rimming
- Expansile, circumscribed, thin cortex
- "Ground glass" appearance
- Haphazard, curvilinear, randomly oriented woven bone trabeculae
- Looks like "Chinese characters"
- Surrounded by fibroblastic stroma
- No significant osteoblastic rimming
- Expansile, circumscribed, thin cortex
- "Ground glass" appearance
How should Fibrous Dysplasia be treated?
Conservatively, except polyostotic form
What is the second most common malignant bone tumor in childhood?
Ewing Sarcoma / PNET
Who is most affected by Ewing Sarcoma / PNET?
- Second most common malignant bone tumor in childhood
- Adolescents / young adults, M > F
What is the presentation of Ewing Sarcoma / PNET?
- Painful, often enlarging mass
- Diaphysis of long tubular bones, ribs, and pelvis
- Destructive moth-eaten, permeative medullary lesion w/ large soft tissue mass
- Onion skin pattern of periosteal reaction in response to rapid growth
- Painful, often enlarging mass
- Diaphysis of long tubular bones, ribs, and pelvis
- Destructive moth-eaten, permeative medullary lesion w/ large soft tissue mass
- Onion skin pattern of periosteal reaction in response to rapid growth
What kind of neoplasm is this?
What kind of neoplasm is this?
Ewing Sarcoma / PNET
- Onion-skin pattern of periosteal reaction in response to rapid growth
What is the histological presentation of Ewing Sarcoma / PNET?
- Sheets of primitive small round blue cells w/ neural phenotype
- Increased membranous CD99 expression
- Contain abundant glycogen
- Hemorrhage and necrosis common
- Sheets of primitive small round blue cells w/ neural phenotype
- Increased membranous CD99 expression
- Contain abundant glycogen
- Hemorrhage and necrosis common
What genetic abnormalities are associated with Ewing Sarcoma / PNET?
t(11;22) in 85% of tumors
- EWS on 22q fused w/ FLI-1 transcription factor on 11q
How is Ewing Sarcoma / PNET treated?
- Chemotherapy and surgery
- Radiation therapy may be added
What is the life expectancy of Ewing Sarcoma / PNET?
Stage 1 = 70% 5-year survival w/ chemo and RT
Who is affected by Giant Cell Tumors of bone?
Young adults (20-40 years), older adolescents (skeletally mature); F > M
Where do Giant Cell Tumors take place?
Epiphyseal (growth plates are closed) - knee, proximal humerus, radius
Epiphyseal (growth plates are closed) - knee, proximal humerus, radius
What kind of neoplasms are Giant Cell Tumors?
- Benign but locally aggressive
- May destroy cortex of bone and extend into soft tissue
- Benign but locally aggressive
- May destroy cortex of bone and extend into soft tissue
What kind of neoplasm is this?
What kind of neoplasm is this?
Giant Cell Tumor of Bone
What is the most common cause of malignant bone tumors?
Metastatic bone tumors (20x more frequent than primary bone tumors)
How many lesions occur in metastatic bone cancer?
- Usually multiple
- Solitary lesions may mimic a primary bone tumor and precede discovery of its source
Where do metastatic bone tumors usually occur?
70% at axial skeleton (skull, ribs, vertebral column, sacrum)
What is the origin of most metastatic bone tumors?
80% - Breast, Lung, Thryroid, Kidney, and Prostate
(BLT-KP = BLT w/ a Kosher Pickle)
What are the characteristics of metastatic bone tumors?
- Mostly lytic
- May be blastic (bone-forming): breast, prostate