9) Metabolic Bone Diseases and Bone Tumors Flashcards

1
Q

Tumor basics

A
  • Tumors are classified based on the origin cell type

- Growing tumors generally present with pain

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2
Q

Metastasis

A
  • Malignant lesions are called sarcomas

- Sarcomas metastasize primarily via blood vessels

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3
Q

Requirements for metastasis

A
  • Invasion of blood vessels
  • Detachment of cells
  • Cells transported to distant locations
  • Cells lodge and grow secondary tumor
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4
Q

Geographic bone destruction pattern

A
  • Least aggressive pattern
  • Consistent with slow growth
  • Well defined margins
  • Generally benign
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5
Q

Moth eaten bone destruction pattern

A
  • More aggressive
  • More rapid growth
  • Less defined margins – greater zone of transition
  • May be malignant
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6
Q

Permeative bone destruction pattern

A
  • Most aggressive
  • Rapid growth
  • Poorly demarcated
  • Often malignant
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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
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8
Q

Critical calcifications

A
  • Occur with cartilaginous tumors
  • Eccentric location
  • Flocculent appearing
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9
Q

Ossification

A
  • Occurs with bone tumors

- Trabecular patterns

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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
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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

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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
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13
Q

Technetium scan

A
  • Rapid uptake via osteoblastic absorption
  • Angiogram
  • Blood pool
  • Delayed image
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14
Q

Gallium-67

A
  • Binds to WBCs and plasma proteins
  • Renal excretion
  • 6 – 24 hours - infection
  • 24 – 72 hours - tumor
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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?
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16
Q

Alkaline phosphatase levels

A
  • Osteoblastic activity
  • Malignancies (Paget’s)
  • Osteitis deformans
  • Multiple myeloma
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17
Q

Calcium levels

A
  • Levels elevated with malignant tumors

- Symptoms (>12.5mg/dl): irritability, memory loss, muscle weakness

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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
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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
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20
Q

Excision techniques

A
  • Intralesional
  • Marginal excision
  • Wide excision
  • Radical excision
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21
Q

Intralesional excision

A
  • Removal of tumor from within capsule
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22
Q

Marginal excision

A
  • Removal of tumor and capsule from surrounding soft tissue
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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
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24
Q

Radical excision

A
  • Removal of the entire anatomical compartment

- Example: osteogenic sarcoma (they used to cut off entire leg for these)

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25
Q

Unicameral bone cyst

A
  • 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
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26
Q

Unicameral bone cyst incidence

A
  • 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
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27
Q

Unicameral bone cyst characteristics

A
  • 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
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28
Q

Unicameral bone cyst treatment

A
  • 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
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29
Q

Aneurysmal bone cyst

A
  • 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
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30
Q

Aneurysmal bone cyst incidence

A
  • Usually occurs in the first 2 decades, but can occur at any age
  • Male to female – 1:1
  • Eccentrically located
  • “likes” long tubular bones
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31
Q

Aneurysmal bone cyst radiographically

A
  • Generally solitary
  • Arises from within bone
  • Possesses delicate trabecular patterns
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32
Q

Aneurysmal bone cyst characteristics

A
  • Possesses sinusoidal cavities
  • Filled with blood
  • Sinusoidal cavities can be imaged with CT or MRI
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33
Q

Aneurysmal bone cyst treatment

A
  • Biopsy – rule out malignancy!

- Curettage and bone grafting

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34
Q

Cartilage forming tumors

A
  • Arise from bone preformed in cartilage
  • Possess speckled calcifications
  • Lesions may involve soft tissue
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35
Q

Endochondroma

A
  • Benign neoplasm
  • Generally asymptomatic
  • Pain may suggest pathologic stress fracture or malignant transformation into chondrosarcoma
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36
Q

Endochondroma incidence

A
  • Third to fourth decades
  • Male to female – 1:1
  • Arises in medullary areas of bone
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37
Q

Endochondroma radiographic findings

A
  • Well defined medullary lesion
  • May occur in cancellous bone
  • “likes” the phalangeal and metatarsal-phalangeal joint areas
  • May have lobulated contour
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38
Q

Endochondroma characteristics

A
  • Bluish-white hyaline cartilage
  • May be mixed with yellow cartilage
  • May have calcifications
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39
Q

Endochondroma treatment

A
  • Curettage and packing

- Lesions may recur

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40
Q

Endochondroma associated syndromes

A
  • Ollier disease: multiple enchondromatoses

- Marfucci syndrome: multiple enchondromatoses with hemangiotosis

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41
Q

Chondroblastoma (Codman’s tumor)

A
  • Uncommon benign lesion
  • 1% of all bone tumors
  • Made up of immature chondroblasts
  • Eccentric epiphyseal location
  • Usually occurs when growth plate is open
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42
Q

Chondroblastoma incidence

A
  • Second decade

- Male to female – 2:1

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43
Q

Chondroblastoma clinical findings

A
  • Painful limited ROM

- Juxta-articular swelling

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44
Q

Chondroblastoma radiographic findings

A
  • Oval to round lytic lesion
  • Two to six cm in diameter
  • Eccentric epiphyseal location
  • “fuzzily rarefied and mottled”
  • Curettage and packing
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45
Q

Chondromyxoid fibroma

A
  • Painful benign tumor
  • Arises from epiphyseal region and occupies metaphyseal bone
  • May undergo malignant transformation
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46
Q

Chondromyxoid fibroma incidence

A
  • Can affect any age group but third decade common
  • Male : female – 3:2
  • “likes” proximal tibia, metatarsals and phalanges – tubular bones
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47
Q

Chondromyxoid fibroma characteristics

A
  • 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
Q

Chondromyxoid fibroma treatment

A
  • Curettage with packing

- Recurrence rate – 25%

49
Q

Osteochondroma

A
  • 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
Q

Osteochondroma incidence

A
  • Second to third decade
  • Male to female – 2:1
  • Metatarsal shafts most commonly involved in foot
51
Q

Osteochondroma radiographic findings

A
  • Slant away from adjacent joint
  • Cortex and trabecular pattern continuous with “host” bone
  • Hyaline cartilaginous cap
  • Does not invade soft tissue
52
Q

Osteochondroma treatment

A
  • En bloc excision with bone grafting
53
Q

Subungual exostosis

A
  • Among the most frequent bony lesions in foot
  • Possesses a fibrocartilaginous cap
  • Possible trauma etiology
54
Q

Subungual exostosis incidence

A
  • Most common on hallux
  • Distal tuft of terminal phalanx
  • Subungual location
  • Frequent in 2nd-3rd decades of life
55
Q

Subungual exostosis radiographically

A
  • Cortical margins continuous with host bone

- May appear smaller due to cartilaginous cap

56
Q

Subungual exostosis clinical presentation

A
  • “pincer” nail plate

- Pain on dorsal compression

57
Q

Subungual exostosis treatment

A
  • En bloc excision and curettage
58
Q

Chondorsarcoma

A
  • Malignant tumor arising from within bone
  • Six subgroups
  • Undergoes rapid growth
  • Metastasizes most often to lungs
59
Q

6 subgroups of chondrosarcoma

A
  • Central – from medullary bone
  • Peripheral – from cortical surface
  • Juxtacortical – periosteum with soft tissue involvement
  • Extraosseous – somatic soft tissue
  • Synovial – intra-articular
  • Tenosynovial
60
Q

Chondrosarcoma incidence

A
  • Predilection for long tubular bones
  • Third to sixth decade
  • Slightly more common in males
61
Q

Chondrosarcoma characteristics

A
  • Thick radiolucent defect
  • Extensive size – may lead to pathological fracture
  • They grow large and rapidly
  • May possess speckled calcifications
  • May involve soft tissue
62
Q

Chondrosarcoma treatment

A
  • 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
Q

Bone forming tumors general trends

A
  • Elevation of serum alkaline phosphatase

- Anemia of chronic disease

64
Q

Bone forming tumors systemic symptoms

A
  • Malaise
  • Fever
  • Leukocytosis
65
Q

Osteoid osteoma

A
  • Unique osteoblastic tumor

- Painful lesion with focal tenderness

66
Q

Osteoid osteoma incidence

A
  • Occurs in second decade
  • Male to female – 2:1
  • Affects long bones
  • In foot common in talus and calcaneus
67
Q

Osteoid osteoma clinical presentation

A
  • Localized pain with edema
  • Begins as a dull ache
  • Typically worse at night
  • Classically relieved by aspirin or NSAID
68
Q

Osteoid osteoma composition

A
  • Osteoid and woven bone

- Smaller than 1.5 cm

69
Q

Osteoid osteoma radiographic findings

A
  • Oval lytic lesion
  • Sclerotic rim with central nidus
  • Less than 1.5 cm in size
70
Q

Osteoid osteoma treatment

A
  • Transcutaneous thermocoagulation
  • Percutaneous radiofrequency ablation
  • Excision and curettage
71
Q

Osteoblastoma

A
  • Uncommon benign tumor
  • May be related to trauma
  • Expansile osteolytic lesion
  • Pain is most common presenting symptom
72
Q

Osteoblastoma characteristics

A
  • Concomitant soft tissue mass
  • Rich osteoblastic activity
  • Locally very aggressive
  • May resemble osteoid osteoma
73
Q

Osteoblastoma incidence

A
  • “Likes” long tubular bones and neural arches
  • 1% of all primary bone neoplasms
  • Male to female – 2:1
74
Q

Osteoblastoma physical appearance

A
  • Highly vascularized connective tissue stroma
  • Active production of osteoid and primitive woven bone
  • High Alkaline phosphatase
75
Q

Osteoblastoma radiographic findings

A
  • May not be specific
  • Expansile: well circumscribed, partially calcified
  • Osteolytic and sclerotic areas: “patchy” granular radiopacity, thin periosteal shell
76
Q

Osteosarcoma

A
  • Primary malignant tumor of bone
  • May have adjacent soft tissue mass
  • Often metaphyseal
  • Occurs around knee
77
Q

Osteosarcoma incidence

A
  • Femur: 40%
  • Tibia: 16%
  • 3rd most common pediatric malignancy
  • Peak incidence – bi-modal
  • 10 – 20 years of age
  • 50 – 70 years of age
78
Q

Osteosarcoma classic radiographic findings

A
  • 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
Q

Osteosarcoma treatment

A
- Preoperative chemotherapy
 to shrink it before surgery
- Radical excision
- Rotationplasty
- Below the knee
- Symes
80
Q

Osteosarcoma prognosis

A
  • SEER stage 5-year relative survival rate
  • Localized: 74%
  • Regional: 66%
  • Distant: 27%
  • All SEER stages combined: 60%
81
Q

Ewing sarcoma

A
  • 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
Q

Ewing sarcoma constitutional symptoms

A
  • Anemia of chronic disease
  • Fever and leukocytosis
  • Marked pain with palpable swelling
  • Highly metastatic
83
Q

Ewing sarcoma incidence

A
  • Peak: 5 to 15 years of age
  • Male to female – 3:2
  • Affects medullary cavities with diaphyseal location
84
Q

Ewing sarcoma most common in

A
  • Femur
  • Tibia
  • Humerus
  • White people more than others
85
Q

Ewing sacroma radiographic findings

A
  • Classic “onion skin” periosteal reaction
  • Motheaten or mottled
  • May show marked reactive bone formation
  • May have endosteal scalloping
  • May spread into soft tissue
86
Q

Ewing sarcoma histochemical markers

A
  • Glycogen positive

- Reticulin negative

87
Q

Ewing sarcoma treatment

A
  • Radiation and chemotherapy preoperatively
  • Radical amputation
  • Additional chemotherapy following resection
88
Q

Poorer prognosis in Ewing sarcoma associated with

A
  • ↑ ESR at presentation

- Leukocytosis at presentation

89
Q

Ewing sarcoma survival rate

A
  • 5 year without metastasis: 56-80%
  • 5 year with metastasis: < 30%
  • Metastasis to lungs, bone and bone marrow
90
Q

Multiple myeloma

A
  • 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
Q

Multiple myeloma constitutional symptoms

A
  • Skeletal destruction and bone pain

- Bone resorption due to elaboration of osteoclast-activating factor

92
Q

Multiple myeloma incidence

A
  • Middle and old age
  • More common in people of color
  • Slightly more common in males
93
Q

Multiple myeloma staging based on

A
  • Hemoglobin levels (low)
  • Serum calcium levels (high)
  • Number of bone lesions
  • Immunoglobulin levels
  • Serum creatinine
94
Q

Multiple myeloma radiographic findings

A
  • Generalized osteoporosis
  • “punched-out” lesions
  • Lesions do not stimulate osteoblastic activity
  • Hypercalcemia may occur secondary to bone destruction
95
Q

Multiple myeloma treatment

A
  • Radiation and chemotherapy
  • Melphalan and prednisone
  • Bisphosphonates
96
Q

Giant cell tumors

A
  • Very rare tumors
  • Arise from mesenchymal and connective tissue of bone
  • Present with pain and swelling
97
Q

Gian cell tumors incidence

A
  • Generally metaphyseal
  • “likes” long tubular bones
  • 3rd to 5th decades
98
Q

Giant cell tumor radiographic findings

A
  • Large lytic lesion
  • May extend from metaphysis to subarticular bone
  • “Soap Bubble” Appearance
99
Q

Giant cell tumor treatment

A
  • Excision, curettage with bone substitute filler
  • Radiation therapy
  • Generally benign
100
Q

Giant cell tumor of the tendon sheath

A
  • Arise from synovial linings of tendon sheaths
  • Causes the affected anatomy to thicken and overgrow
  • Involves the synovium, bursae and tendon sheath
101
Q

Giant cell tumor of the tendon sheath clinical presentation

A
  • 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
Q

GCT of the tendon sheath treatment

A
  • Prompt diagnosis and treatment essential to avoiding disability
  • Surgery remains the cornerstone of treatment
  • Medical therapy
103
Q

Pigmented villonodular synovitis

A
  • May arise from synovial tissue
  • Most commonly occur around joints
  • Highly infiltrative
104
Q

Pigmented villonodular synovitis clinical presentation

A
  • May occur locally (within a joint) or diffusely
  • Anterior kneeis the most common (80%)
  • Acute synovitis
  • Aspiration reveals brownish, bloody fluid
  • May involve adjacent bone
105
Q

Pigmented villonodular synovitis incidence

A
  • Adults aged 30-40(but can occur at any age)

- Men = Women

106
Q

Pigmented villonodular synovitis treatment

A
  • Partial or Total Synovectomy
  • Recurrence is themost frequent complicationfor 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
Q

Synovial sarcoma

A
  • 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
Q

Synovial sarcoma diagnosis

A
  • 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
Q

Synovial sarcoma treatment

A
  • Surgery is the mainstay of treatment for synovial sarcoma.
  • Radical excision
  • Radiation therapy or chemotherapy
110
Q

Non-ossifying fibroma

A
  • Benign common self healing lesion
  • Geographic, multi-lobulated, lytic lesions
  • Migrates away from the epiphysis
111
Q

Non-ossifying fibroma incidence

A
  • 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
Q

Non-ossifying fibroma radiographic appearance

A
  • Eccentric, round or ovoid
  • Scalloped contour
  • Sharply defined sclerotic border
  • Begins at the epiphyseal-metaphyseal junction
  • Grows into the metaphysis
  • Epiphysis never affected
113
Q

Non-ossifying fibroma treatment

A
  • Generally, none
  • Develop into fibroxanthomas
  • Curettage with bone packing if > 50% diameter involved
114
Q

Metastatic tumors of bone

A
  • Can mimic any type of tumor

- Biopsy only can confirm etiologic cell type

115
Q

Metastasis of bone tumors in adults

A
  • Pulmonary
  • Breast
  • Prostate
116
Q

Metastasis of bone tumors in kids

A
  • Neuroblastoma

- Nephroblastoma (Wilm’s Tumor)