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