9) Metabolic Bone Diseases and Bone Tumors Flashcards
(116 cards)
1
Q
Tumor basics
A
- Tumors are classified based on the origin cell type
- Growing tumors generally present with pain
2
Q
Metastasis
A
- Malignant lesions are called sarcomas
- Sarcomas metastasize primarily via blood vessels
3
Q
Requirements for metastasis
A
- Invasion of blood vessels
- Detachment of cells
- Cells transported to distant locations
- Cells lodge and grow secondary tumor
4
Q
Geographic bone destruction pattern
A
- Least aggressive pattern
- Consistent with slow growth
- Well defined margins
- Generally benign
5
Q
Moth eaten bone destruction pattern
A
- More aggressive
- More rapid growth
- Less defined margins – greater zone of transition
- May be malignant
6
Q
Permeative bone destruction pattern
A
- Most aggressive
- Rapid growth
- Poorly demarcated
- Often malignant
7
Q
Periosteal reactions
A
- More dramatic in the pediatric patient
- Periosteum loosely adhered to underlying bone
- More associated with aggressive tumors
- Must distinguish from osteomyelitis
8
Q
Critical calcifications
A
- Occur with cartilaginous tumors
- Eccentric location
- Flocculent appearing
9
Q
Ossification
A
- Occurs with bone tumors
- Trabecular patterns
10
Q
MRI for bone tumors
A
- Useful for identification
- Fat and marrow emit strong signal (T1 – fat intense, T2 – water intense)
- With tumor infiltration, water content increases
- Leads to greater intensity on T2 weighted imaging
11
Q
CT for bone tumors
A
- Useful to see if soft tissue lesion has infiltrated bone
- Estimates the presence and nature of bone tumors
12
Q
CT can reveal
A
- Extent of bone destruction
- Cortical integrity
- Periosteal reaction
- Bone matrix alterations (aneurysmal bone cysts have this)
- Transition zones
- Presence of “critical” calcifications
13
Q
Technetium scan
A
- Rapid uptake via osteoblastic absorption
- Angiogram
- Blood pool
- Delayed image
14
Q
Gallium-67
A
- Binds to WBCs and plasma proteins
- Renal excretion
- 6 – 24 hours - infection
- 24 – 72 hours - tumor
15
Q
Blood and serum chemistry CBC with differential
A
- Low serum iron
- Low total iron binding capacity
- High serum ferritin
- Low lab values for hemoglobin, hematocrit, MCV
- Leukocytosis?
16
Q
Alkaline phosphatase levels
A
- Osteoblastic activity
- Malignancies (Paget’s)
- Osteitis deformans
- Multiple myeloma
17
Q
Calcium levels
A
- Levels elevated with malignant tumors
- Symptoms (>12.5mg/dl): irritability, memory loss, muscle weakness
18
Q
Connective tissue neoplasm benign staging based on
A
- Radiographic appearance
- Histological appearance
- Look very similar to surrounding cells
- Anatomic size and location are not as useful
19
Q
Connective tissue neoplasm malignant staging
A
- Classification by Enneking
- Grade: how invasive are cells
- G0 benign, G1 low grade, G2 high grade
- Site: T0 Capsulated, T1 Intracompartmental/still within area of tissue, T2 Extracompartmental/expands through cortical margins into soft tissue
20
Q
Excision techniques
A
- Intralesional
- Marginal excision
- Wide excision
- Radical excision
21
Q
Intralesional excision
A
- Removal of tumor from within capsule
22
Q
Marginal excision
A
- Removal of tumor and capsule from surrounding soft tissue
23
Q
Wide excision
A
- Removal of tumor and capsule with a margin of normal surrounding tissue
- The tumor and capsular tissue is not violated
24
Q
Radical excision
A
- Removal of the entire anatomical compartment
- Example: osteogenic sarcoma (they used to cut off entire leg for these)
25
Unicameral bone cyst
- Most common cystic lesion in foot (likes the calcaneus)
- Usually an incidental finding
- Not a painful lesion!
- May cause stress fracture
- It is not an actual tumor
- May be secondary to an inflammatory process
26
Unicameral bone cyst incidence
- First and second decades
- Male to female: 2:1
- Usually in distal ends of long tubular bones, but likes humerus
- Fallen fragment (leaf sign)
- Occurs in metaphyseal and diaphyseal locations
27
Unicameral bone cyst characteristics
- Fluid filled cyst (yellowish to reddish color, thin fibrous membrane)
- Well defined sclerotic border
- Short transition zone
- Cortical thinning from intramedullary side
- Stress fracture – fallen fragment
28
Unicameral bone cyst treatment
- None necessary unless fracture occurs
- Curettage and packing with bone chips
- May try aspiration with introduction of acetated glucocorticoids
- Process may be repeated
- Use of bone stimulator if fracture present
29
Aneurysmal bone cyst
- Painful lesion is the CC
- Associated with swelling and tenderness
- Aggressive and expansile
- May be confused with sarcomatous lesion
- May be a secondary lesion arising from a primary bone tumor
- Not an actual tumor
30
Aneurysmal bone cyst incidence
- Usually occurs in the first 2 decades, but can occur at any age
- Male to female – 1:1
- Eccentrically located
- “likes” long tubular bones
31
Aneurysmal bone cyst radiographically
- Generally solitary
- Arises from within bone
- Possesses delicate trabecular patterns
32
Aneurysmal bone cyst characteristics
- Possesses sinusoidal cavities
- Filled with blood
- Sinusoidal cavities can be imaged with CT or MRI
33
Aneurysmal bone cyst treatment
- Biopsy – rule out malignancy!
| - Curettage and bone grafting
34
Cartilage forming tumors
- Arise from bone preformed in cartilage
- Possess speckled calcifications
- Lesions may involve soft tissue
35
Endochondroma
- Benign neoplasm
- Generally asymptomatic
- Pain may suggest pathologic stress fracture or malignant transformation into chondrosarcoma
36
Endochondroma incidence
- Third to fourth decades
- Male to female – 1:1
- Arises in medullary areas of bone
37
Endochondroma radiographic findings
- Well defined medullary lesion
- May occur in cancellous bone
- “likes” the phalangeal and metatarsal-phalangeal joint areas
- May have lobulated contour
38
Endochondroma characteristics
- Bluish-white hyaline cartilage
- May be mixed with yellow cartilage
- May have calcifications
39
Endochondroma treatment
- Curettage and packing
| - Lesions may recur
40
Endochondroma associated syndromes
- Ollier disease: multiple enchondromatoses
| - Marfucci syndrome: multiple enchondromatoses with hemangiotosis
41
Chondroblastoma (Codman's tumor)
- Uncommon benign lesion
- 1% of all bone tumors
- Made up of immature chondroblasts
- Eccentric epiphyseal location
- Usually occurs when growth plate is open
42
Chondroblastoma incidence
- Second decade
| - Male to female – 2:1
43
Chondroblastoma clinical findings
- Painful limited ROM
| - Juxta-articular swelling
44
Chondroblastoma radiographic findings
- Oval to round lytic lesion
- Two to six cm in diameter
- Eccentric epiphyseal location
- “fuzzily rarefied and mottled”
- Curettage and packing
45
Chondromyxoid fibroma
- Painful benign tumor
- Arises from epiphyseal region and occupies metaphyseal bone
- *May undergo malignant transformation*
46
Chondromyxoid fibroma incidence
- Can affect any age group but third decade common
- Male : female – 3:2
- “likes” proximal tibia, metatarsals and phalanges – tubular bones
47
Chondromyxoid fibroma characteristics
- Round to oval lesion
- Sharply defined sclerotic margin
- May possess scalloped margin
- Presence of coarse trabecular patterns
- Has lobulated areas of spindle shaped cells
48
Chondromyxoid fibroma treatment
- Curettage with packing
| - Recurrence rate – 25%
49
Osteochondroma
- **The most common bone tumor:
20 to 50% of all benign osseous tumors**
- Benign bony projections away from joints
- Arise from/continuous with cortical bone
- No pain unless pressing on something else
- Possess smooth hyaline cartilaginous cap
- Accounts for 50% of all bone tumors
- May correlate with trauma
50
Osteochondroma incidence
- Second to third decade
- Male to female – 2:1
- Metatarsal shafts most commonly involved in foot
51
Osteochondroma radiographic findings
- Slant away from adjacent joint
- Cortex and trabecular pattern continuous with “host” bone
- Hyaline cartilaginous cap
- Does not invade soft tissue
52
Osteochondroma treatment
- En bloc excision with bone grafting
53
Subungual exostosis
- Among the most frequent bony lesions in foot
- Possesses a fibrocartilaginous cap
- Possible trauma etiology
54
Subungual exostosis incidence
- Most common on hallux
- Distal tuft of terminal phalanx
- Subungual location
- Frequent in 2nd-3rd decades of life
55
Subungual exostosis radiographically
- Cortical margins continuous with host bone
| - May appear smaller due to cartilaginous cap
56
Subungual exostosis clinical presentation
- “pincer” nail plate
| - Pain on dorsal compression
57
Subungual exostosis treatment
- En bloc excision and curettage
58
Chondorsarcoma
- Malignant tumor arising from within bone
- Six subgroups
- Undergoes rapid growth
- Metastasizes most often to lungs
59
6 subgroups of chondrosarcoma
- Central – from medullary bone
- Peripheral – from cortical surface
- Juxtacortical – periosteum with soft tissue involvement
- Extraosseous – somatic soft tissue
- Synovial – intra-articular
- Tenosynovial
60
Chondrosarcoma incidence
- Predilection for long tubular bones
- Third to sixth decade
- Slightly more common in males
61
Chondrosarcoma characteristics
- Thick radiolucent defect
- Extensive size – may lead to pathological fracture
- They grow large and rapidly
- May possess speckled calcifications
- May involve soft tissue
62
Chondrosarcoma treatment
- Radical excision with removal of surrounding uninvolved osseous margins and soft tissue
- Monitor for metastasis – generally pulmonary
- May also involve liver, kidney and brain
63
Bone forming tumors general trends
- Elevation of serum alkaline phosphatase
| - Anemia of chronic disease
64
Bone forming tumors systemic symptoms
- Malaise
- Fever
- Leukocytosis
65
Osteoid osteoma
- Unique osteoblastic tumor
| - Painful lesion with focal tenderness
66
Osteoid osteoma incidence
- Occurs in second decade
- Male to female – 2:1
- Affects long bones
- In foot common in talus and calcaneus
67
Osteoid osteoma clinical presentation
- Localized pain with edema
- Begins as a dull ache
- Typically worse at night
- Classically relieved by aspirin or NSAID
68
Osteoid osteoma composition
- Osteoid and woven bone
| - Smaller than 1.5 cm
69
Osteoid osteoma radiographic findings
- Oval lytic lesion
- Sclerotic rim with central nidus
- Less than 1.5 cm in size
70
Osteoid osteoma treatment
- Transcutaneous thermocoagulation
- Percutaneous radiofrequency ablation
- Excision and curettage
71
Osteoblastoma
- Uncommon benign tumor
- May be related to trauma
- Expansile osteolytic lesion
- Pain is most common presenting symptom
72
Osteoblastoma characteristics
- Concomitant soft tissue mass
- Rich osteoblastic activity
- Locally very aggressive
- May resemble osteoid osteoma
73
Osteoblastoma incidence
- “Likes” long tubular bones and neural arches
- 1% of all primary bone neoplasms
- Male to female – 2:1
74
Osteoblastoma physical appearance
- Highly vascularized connective tissue stroma
- Active production of osteoid and primitive woven bone
- High Alkaline phosphatase
75
Osteoblastoma radiographic findings
- May not be specific
- Expansile: well circumscribed, partially calcified
- Osteolytic and sclerotic areas: “patchy” granular radiopacity, thin periosteal shell
76
Osteosarcoma
- Primary malignant tumor of bone
- May have adjacent soft tissue mass
- Often metaphyseal
- Occurs around knee
77
Osteosarcoma incidence
- Femur: 40%
- Tibia: 16%
- 3rd most common pediatric malignancy
- Peak incidence – bi-modal
- 10 – 20 years of age
- 50 – 70 years of age
78
Osteosarcoma classic radiographic findings
- Sunburst periosteal reaction
- Codman's triangle (periosteal elevation)
- Metaphyseal tubular bones
- Cortical bone destruction
- Motheaten bone destruction
- May see dense sclerosis in the center
- Extraosseous mass may be present
79
Osteosarcoma treatment
```
- Preoperative chemotherapy
to shrink it before surgery
- Radical excision
- Rotationplasty
- Below the knee
- Symes
```
80
Osteosarcoma prognosis
- SEER stage 5-year relative survival rate
- Localized: 74%
- Regional: 66%
- Distant: 27%
- All SEER stages combined: 60%
81
Ewing sarcoma
- Primary malignant tumor of bone
- Highly metastatic
- Peripheral neuroectodermal origin
- Derived from red marrow
- Genetic chromosomal translocation parts of chromosome 11 → 22
- Dramatic constitutional symptoms
82
Ewing sarcoma constitutional symptoms
- Anemia of chronic disease
- Fever and leukocytosis
- Marked pain with palpable swelling
- Highly metastatic
83
Ewing sarcoma incidence
- Peak: 5 to 15 years of age
- Male to female – 3:2
- Affects medullary cavities with diaphyseal location
84
Ewing sarcoma most common in
- Femur
- Tibia
- Humerus
- White people more than others
85
Ewing sacroma radiographic findings
- Classic “onion skin” periosteal reaction
- Motheaten or mottled
- May show marked reactive bone formation
- May have endosteal scalloping
- May spread into soft tissue
86
Ewing sarcoma histochemical markers
- Glycogen positive
| - Reticulin negative
87
Ewing sarcoma treatment
- Radiation and chemotherapy preoperatively
- Radical amputation
- Additional chemotherapy following resection
88
Poorer prognosis in Ewing sarcoma associated with
- ↑ ESR at presentation
| - Leukocytosis at presentation
89
Ewing sarcoma survival rate
- 5 year without metastasis: 56-80%
- 5 year with metastasis: < 30%
- Metastasis to lungs, bone and bone marrow
90
Multiple myeloma
- Unregulated growth and proliferation of plasma cells
- Diffuse plasma cell infiltration into bone marrow
- Overproduction of monoclonal immunoglobulins or light chains only
- Possibly most common malignant tumor of bone
91
Multiple myeloma constitutional symptoms
- Skeletal destruction and bone pain
| - Bone resorption due to elaboration of osteoclast-activating factor
92
Multiple myeloma incidence
- Middle and old age
- More common in people of color
- Slightly more common in males
93
Multiple myeloma staging based on
- Hemoglobin levels (low)
- Serum calcium levels (high)
- Number of bone lesions
- Immunoglobulin levels
- Serum creatinine
94
Multiple myeloma radiographic findings
- Generalized osteoporosis
- “punched-out” lesions
- Lesions do not stimulate osteoblastic activity
- Hypercalcemia may occur secondary to bone destruction
95
Multiple myeloma treatment
- Radiation and chemotherapy
- Melphalan and prednisone
- Bisphosphonates
96
Giant cell tumors
- Very rare tumors
- Arise from mesenchymal and connective tissue of bone
- Present with pain and swelling
97
Gian cell tumors incidence
- Generally metaphyseal
- “likes” long tubular bones
- 3rd to 5th decades
98
Giant cell tumor radiographic findings
- Large lytic lesion
- May extend from metaphysis to subarticular bone
- *“Soap Bubble”* Appearance
99
Giant cell tumor treatment
- Excision, curettage with bone substitute filler
- Radiation therapy
- Generally benign
100
Giant cell tumor of the tendon sheath
- Arise from synovial linings of tendon sheaths
- Causes the affected anatomy to thicken and overgrow
- Involves the synovium, bursae and tendon sheath
101
Giant cell tumor of the tendon sheath clinical presentation
- Lesions are almost always benign
- Can be aggressive and destroy the surrounding bone and tissue
- Painless slow growing mass
- Location generally suggests benign nature
102
GCT of the tendon sheath treatment
- Prompt diagnosis and treatment essential to avoiding disability
- Surgery remains the cornerstone of treatment
- Medical therapy
103
Pigmented villonodular synovitis
- May arise from synovial tissue
- Most commonly occur around joints
- *Highly infiltrative*
104
Pigmented villonodular synovitis clinical presentation
- May occur locally (within a joint) or diffusely
- Anterior knee is the most common (80%)
- Acute synovitis
- Aspiration reveals brownish, bloody fluid
- May involve adjacent bone
105
Pigmented villonodular synovitis incidence
- Adults aged 30-40 (but can occur at any age)
| - Men = Women
106
Pigmented villonodular synovitis treatment
- Partial or Total Synovectomy
- Recurrence is the most frequent complication for both intra-articular and extra-articular disease
- 30%-50% recurrence rate despite complete synovectomy
- Radiation therapy may be beneficial
- CSF-1 receptor antagonist (pexidartinib)
107
Synovial sarcoma
- Rare
- Lesions initially grow quite slowly
- Painful when on plantar surface of foot
- Needle aspiration fails to produce fluid
- Rapid spread into surrounding structures
- Encapsulated
108
Synovial sarcoma diagnosis
- Radiographs can be unremarkable
- CT and MRI to evaluate extent of tumor invasion
- Triple sign: mixture of high, intermediate and low signal regions within the mass seen on T2 weighted images
- Bowl of grapes: appearance of multiple fluid-fluid levels in the mass
- Angiogram may be useful
- Triphasic technetium scan
109
Synovial sarcoma treatment
- Surgery is the mainstay of treatment for synovial sarcoma.
- Radical excision
- Radiation therapy or chemotherapy
110
Non-ossifying fibroma
- Benign common self healing lesion
- Geographic, multi-lobulated, lytic lesions
- Migrates away from the epiphysis
111
Non-ossifying fibroma incidence
- Generally, between 5-20 years of age
- Males slightly more than females
- Incidental radiographic finding
- Metaphyseal or metaphyseal-epiphyseal junction location
- “Likes” tubular bones
112
Non-ossifying fibroma radiographic appearance
- Eccentric, round or ovoid
- Scalloped contour
- Sharply defined sclerotic border
- Begins at the epiphyseal-metaphyseal junction
- Grows into the metaphysis
- Epiphysis never affected
113
Non-ossifying fibroma treatment
- Generally, none
- Develop into fibroxanthomas
- Curettage with bone packing if > 50% diameter involved
114
Metastatic tumors of bone
- Can mimic any type of tumor
| - Biopsy only can confirm etiologic cell type
115
Metastasis of bone tumors in adults
- Pulmonary
- Breast
- Prostate
116
Metastasis of bone tumors in kids
- Neuroblastoma
| - Nephroblastoma (Wilm's Tumor)
