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MSS Weeks 3-5 > Bone Tumors > Flashcards

Bone Tumors Flashcards

1
Q

Malignant bone tumors comprise __% of all types of cancer

A

Malignant bone tumors comprise **~0.2% **of all types of cancer

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2
Q

What are potential clinical presentations for bone tumor?

A
  1. Pain
    • any tumor
    • osteoid osteoma: severe pain, worse at night, relieved by aspirin
  2. Mass
    • parosteal OS: painless, hard growing mass in popliteal fossa
  3. Pathologic fracture
  4. Asymptomatic
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3
Q

What are some diagnostic factors to consider for bone tumors?

A
  • Age
  • Sex
  • Skeletal localization
  • Specific bone
  • Specific area of bone
    • Medullary cavity, cortex, juxtacortical
    • Epiphysis, metaphysis, diaphysis
  • Radiographic appearance
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4
Q

How does age correlate with the following tumors?

  1. Osteosarcoma, Ewing’s sarcoma ⇒
  2. Giant cell tumor ⇒
  3. Chondrosarcoma ⇒
A
  1. Osteosarcoma, Ewing’s sarcoma ⇒ childhood, adolescence
  2. Giant cell tumor ⇒ young adults
  3. Chondrosarcoma ⇒ elderly
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5
Q
  1. Sclerotic margin is generally an indication of:
  2. Ill-defined margin is generally an indication of:
  3. Solid, ivory-like pattern is generally seen in:
  4. Rings and arcs are generally seen in:
A
  1. Sclerotic margin is generally an indication of:
    • benign, slowly-growing neoplasm
  2. Ill-defined margin is generally an indication of:
    • malignant, rapidly-growing neoplasm
  3. ​Solid, ivory-like pattern is generally seen in:
    • malignant bone matrix-forming tumors
  4. Rings and arcs are generally seen in:
    • chondroid matrix-forming tumors
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6
Q

What are characteristics of osteiod osteoma?

A
  • Long bones, femur & tibia
  • < 2 cm
  • Night pain
  • Responds to aspirin
  • Radiolucent lesion within sclerotic cortex
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7
Q

What are the characteristics of osteoblastomas?

A
  • Vertebrae or long bone metaphysis
  • > 2 cm
  • Painful
  • Not responsive to aspirin
  • Expansile radio-lucency with mottling
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8
Q

What is the most common sarcoma of bone?

A

osteosarcoma

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9
Q

What kind tumor is osteosarcoma?

A

Malignant mesenchymal tumor in which cells produce osteoid or bone

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10
Q

Where does an osteosarcoma typically present? Where does it typically spread?

A
  • Metaphysis of long bones
    • Femur, tibia, humerus (56%); flat bones, spine (older patients)
    • May be polyostotic (not common)
  • Hematogenous spread to lungs is common
    • Also mets to pleura, other bones & CNS
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11
Q

Describe the posssible pathogenesis of osteosarcoma:

A
  • Inherited mutant allele of RB gene
    • Hereditary RB: marked increase (1000X) in OS
  • Mutation of p53 suppressor gene
    • Li-Fraumeni: bone and soft tissue sarcomas, early onset breast cancer, brain tumors, leukemia
  • Overexpression of MDM2 (5-10%); INK4 and p16
  • Sites of bone growth/disease (i.e. Paget dz.)
  • Prior irradiation
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12
Q

What are the morphologic features of osteosarcoma?

A
  1. Poorly delineated
  2. Bone destruction
  3. Cortical disruption
  4. Bone matrix
  5. Soft tissue extension
  6. Codman’s triangle
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13
Q

Osteosarcoma

  • Pathology:
  • Treatment:
A
  • Pathology:
    • Infiltrative tumor, extending into soft tissue
    • Malignant cells producing osteoid
  • Treatment:
    • Neo-adjuvant chemotherapy and surgical resection
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14
Q

Osteosarcoma: Prognosis

A
  • Post chemotherapy: 60-65% 3-5 yr. survival for patients with non-metastatic disease
  • En-bloc resection following chemotherapy: >90% necrosis ⇒ near 90% survival
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15
Q

What is the most common benign tumor of bone?

A

osteochondroma

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16
Q

Where does an osteochondroma typically present? What is the genetic alteration associated with this tumor?

A
  • Metaphysis of long bones
  • Malignant transformation is rare (<1%), but increased risk (~40%) in hereditary multiple exostoses (multiple osteochondromas)
  • Autosomal dominant, most commonly secondary to mutations in EXT-1 (8q24)
17
Q

What is an enchondroma? What is a perisoteal chondroma?

A

Benign hyaline cartilage lesion

  • Enchondroma = intramedullary chondroma
  • Periosteal chondroma = juxtacortical chondroma
    • located on the cortical surface under the periosteum
18
Q

Describe the characteristics associated with an enchondroma.

  1. How does it appear on x-ray?
  2. How does it appear microscopically?
  3. How is it treated?
A
  • Usually asymptomatic, incidental finding
  • Appendicular skeleton
    • _​_small bones of hands and feet
  1. X-Rays: Lytic, lobulated, cortical thinning
  2. Micro: lobules of hyaline cartilage, minimal atypia
  3. Treatment: none, unless lesion shows changes:
    • Symptomatic (onset of acute pain is felt to be circumstantial evidence that lesion is malignant)
    • Evidence of recent growth after skeletal maturity
19
Q

What can cause multiple chondromatosis? What are the types of multiple chondromatosis?

A

Frequent point mutations in IDH1 or IDH2

  1. Ollier’s disease
    • Multiple enchondromata
    • Tend to have regional distribution
    • ± severe skeletal malformation
  2. Maffucci’s syndrome
    1. Multiple enchondromata + angiomata
    2. Severe skeletal malformation
    3. Higher incidence of malignant transformation
20
Q

What is the second most common bone sarcoma?

What age group does usually present in?

Where does it typically present?

A

Chondrosarcoma

  • Malignant tumor in which neoplastic cells produce a purely cartilaginous matrix
    • ~26% of all bone sarcomas
  • Wide range of ages, mainly older adults
    • Mostly above 40-50 years
    • Peak during 6th- 7th decades
  • Central skeleton: pelvis and ribs (45%); humerus, femur (metaphysis, diaphysis)
21
Q

What would be seen on imaging for a chondrosarcoma?

A
  • Medullary location
  • Frequently present calcifications, which tend to be lost in grade 3 tumors
  • Cortical erosion or destruction
  • Occasional soft tissue extension
  • “popcorn-like” appearance
22
Q

What pathology is associated chondrosarcoma?

How is the size of the tumor important?

What is the grade based upon?

A
  • Generally more cellular and nuclei more pleomorphic than in enchondromas
  • Binucleation is frequent, but does not suffice for malignant diagnosis
  • Myxoid change of chondroid matrix
  • Size (< or > 10 cm) and grade correlate with behavior
  • Grading (1-3) based on degree of cellularity and atypia
23
Q

What is the 5 year survival rate for chondrosarcoma? What are the variants of chondrosarcoma?

A
  • 5 year survival:
    • Grades 1,2: 80-90%
    • Grade 3: 29% (pulmonary metastasis)
  • Variants:
    1. de-differentiated
    2. myxoid
    3. clear cell
    4. mesenchymal
    5. juxtacortical
24
Q

Non-ossifying fibroma:

  • Cause:
  • Presentation:
A
  • Cause:
    • Common developmental cortical defect
  • Presentation:
    • Tibia, femur (metaphysis); 1st – 3rd decades.
    • Eccentric, lytic, peripheral sclerosis
    • Incidental finding or pathologic fracture
25
Q

Fibrous Dysplasia:

  1. Monostotic
  2. Polyostotic
A

Developmental arrest of bone

  1. Monostotic
    • Most common, seen in adolescents
    • Ribs, mandible and femur
  2. Polyostotic
    • ​Infancy/childhood
    • Crippling deformities, craniofacial involvement common
26
Q

What is McCune-Albright syndrome?

A
  • Polyostotic FD with endocrinopathies and café-au-lait spots
    • Rare form; F>M
    • Sexual precocity, acromegaly, Cushing syndrome
  • Activating germline mutations of GNAS (GTP-binding protein) result in excess cAMP leading to endocrine gland hyperfunction
27
Q

What are the morphologic features of fibrous dysplasia?

A
  • Expansile
  • Circumscribed
  • Thinned cortex
  • “Ground glass”
  • May be multiple
28
Q

Fibrous Dysplasia:

  1. Pathology
  2. Treatment
A
  1. Pathology
    • ​​Haphazard, curvilinear, randomly oriented woven bone trabeculae (“Chinese characters”), surrounded by fibroblastic stroma
    • No significant osteoblastic rimming
  2. Treatment
    • ​​Conservative, except polyostotic form
29
Q

What is the second most common malignant bone tumor in childhood?

A

Ewing Sarcoma/PNET

30
Q

How does Ewing sarcoma/PNET typically present?

A
  • Adolescents, young adults
    • M>F
  • Present as painful, often enlarging mass
  • Diaphysis of long tubular bones, ribs and pelvis
31
Q

How does Ewing sarcoma/PNET appear on X-ray?

A
  • X-RAY:
    • Destructive moth-eaten, permeative medullary lesion with large soft tissue mass
  • “Onion-skin” pattern of periosteal reaction in response to rapid growth
32
Q

Ewing sarcoma/PNET:

​Pathology

A
  • Sheets of primitive small round blue cells with neural phenotype
  • Membranous CD99
  • t(11;22)(q24;q12)
  • Contain abundant glycogen
  • Hemorrhage and necrosis common
33
Q

Ewing sarcoma/PNET:

  • Pathogenesis
  • Treatment
A
  • Pathogenesis:
    • EWS involved in >95% of ES/PNET
    • t(11;22) present in 85% of the tumors
    • EWS on 22q fused with FLI-1 transcription factor on 11q
  • ​Treatment:
    • Chemotherapy and surgery
    • Radiation therapy may be added
    • Stage I: 5 year survival 70% with chemo/ RT
34
Q

Giant Cell Tumor of the bone

A
  • Young adults (20-40 years), older adolescents (skeletally matured)
    • F>M
  • Epiphyseal location
  • Knee, proximal humerus, radius
  • Most are benign, locally aggressive
    • May destroy cortex of bone and extend into soft tissue
35
Q

What is the most common malignant bone tumor?

A

Metastatic Bone Tumors

  • especially in adults
  • 20X more frequent than primary bone tumors
36
Q

Where do metastatic bone tumors go in the body?

A
  • Mostly multiple
  • Solitary lesions may mimic a primary bone tumor and precede discovery of its source
    • 70% go to axial skeleton (skull, ribs, vertebral column, sacrum)
    • Mostly lytic
    • May be blastic (bone-forming): breast, prostate
  • 80%: breast, lung, thyroid, prostate and kidney (BLT-KP)

MSS Weeks 3-5 (22 decks)

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