Oncology - Orthobullets

  1. 5 carcinomas that commonly spread to bone
    • Breast
    • Lung
    • Thyroid
    • Renal
    • Prostate
  2. 3 most common sites of bone mets
    • Spine #1: most common thoracic
    • Proximal femur #2
    • Humerus #3
  3. Most common site of fracture secondary to mets
    Proximal femur
  4. Mechanism of action of osteolysis in bone mets
    Tumor induced activation of osteoclasts: Through RANKL pathway
  5. Metastatic hypercalcemia: 4 treatment options
    Hydration: volume expansion

    Loop Diuretics

    Bisphosphonates

    calcitonin: inhibit osteoclast and increase renal excretion of calcium
  6. Why is the axial skeleton the most common site of mets
    There is vascular spread through Batson's vertebral plexus: Valveless venous plexus of the spine that provide a route for mets from organs to axial structures
  7. Mechanism of bone sclerosis in bony mets
    Activation of WNT pathway: for example prostate cancer secretes endothelin 1
  8. 5 symptoms of metastatic hypercalcemia
    • COnfusion
    • Muscle weakness
    • Poluuria/polydipsia
    • Nausea/vomiting
    • dehydration
  9. 2 bony mets that are cold on bone scan
    Thyroid and myeloma: evaluate with skeletal survey
  10. If there is metastatic adenocarcinoma not identified by CT, what is the most likely source
    Small cell lung cancer
  11. Most likely primary: Cortical mets
    Lesions distal to elbow and knee
    • Lung cancer
    • Lung or renal
  12. 2 cancers that require pre op embolization
    • Thyroid
    • Kidney: RCC
  13. GCT: gender
    Age
    Location
    Where does it metastasize
    • Female > male
    • 30-50
    • Distal femur > proximal tibia > distal radius > sacral ala
    • Lung: usually benign
  14. GCT lesions that have greatest chance of metastasis
    Hand lesions
  15. eccentric lytic epiphyseal/metaphyseal lesion that often extends into the distal epiphysis and borders subchondral bone: Diagnosis
    GCT
  16. Medical management of GCT
    Bisphosphonates: osteoclast inhibitors...may decrease size of defect

    Denosumab: Rankl inhibitor > shows sclerosis and reconstitution of cortical bone
  17. GCT: malignant transformation to
    Location in the spine
    If suspecting, always rule out
    HIgh grade sarcoma: poor prognosis

    Anterior elements

    Teleangectatic osteosarcoma
  18. DDx benign aggressive tumors 5 + how to treat it
    • GCT
    • CHondroblastoma
    • Aneurysmal bone cyst
    • Osteoblastoma
    • Chondromyxoid fibroma

    Treatments is extended intralesional curetage and bone grafting
  19. 5 causes of secondary chondrosarcoma
    • Osteochondroma: <1% risk
    • Multiple hereditary exostosis: 1-10% transformation
    • Enchondromas: 1%
    • Ollier's disease: 25-40 risk malignant transformation
    • Maffucci's: 100% risk malignant transformation
  20. Chondrosarcoma: what correlates to rate of recurrence
    2 subtypes
    RT-PCR showing increased telomerase activity

    Clear cell chondrosarcoma: can look like low grade chondroblastoma

    Mesenchymal chondrosarcoma: needs neoadjuvant chemo followed by wide resection
  21. Treatment of: low grade chondrosarcoma
    High grade chondrosarcoma
    Mesenchymal chondrosarcoma
    • Intra-lesional curettage
    • WIde excision: no chemo or radiotherapy
    • Chemotherapy + wide excision
  22. A benign dysplasia that leads to unilateral varus of the tibia: Dx

    treatment
    Focal fibrocartilagenous dysplasia: classic consists of a lucency in the medial cortical border of the metaphysis

    Observation: varus will correct with growth
  23. medullary lesion of sheet-like central lucency surrounded by sclerosis with a serpiginous border : diagnosis
    Bone infarct: also looks like smoke up chimney
  24. Angiosarcoma: Prognosis
    Treatment
    Tumor marker
    • Poor: very aggresive
    • Wide excision: does not respond to chemo or radtx
    • CD31/CD34
  25. Most common solid tumor in children
    Neuroblastoma
  26. Neuroblastoma: Location
    Age
    treatment
    • CLose to adrenals or spinal cord: from sympathetic neural tissue
    • Children < 2
    • Neoadjuvant chemo, stem cell transplant and surgical excision
  27. 5 small round blue cell tumors
    • WIlms
    • Rhabdomyosarcoma
    • Neuroblastoma
    • Non hodgkin lymphoma
    • Ewing sarcoma
  28. Lederhosen disease: what is it
    Plantar fibromatosis similar to dupuytrens
  29. Undifferentiated pleomorphic sarcoma: histology
    Treatment
    Spindle cells in cartwheel pattern

    Wide local resection and adjuvant radiation: if tumor >5cm add radiation
  30. Teleangectatic osteosarcoma: looks like
    Behaves like
    Treatment
    Good prognostic sign
    • ABC
    • Regular osteosarcoma
    • Neoadjuvant chemo and limb salvage resection: chemo is doxy/cisplatin/methotraxate/fosamide
    • 98% necrosis in biopsy: good prognosis
  31. Melanoma 5 poor prognostic factord + most important
    • Depth: most important..if <0.7 mm 96%..if >4mm 47% (breslow classification)
    • Male sex
    • lesion on neck or scalp
    • Positive lymph nodes or mets
    • ulcerations
  32. Melanoma:Cell origin
    Neural crest cells
  33. Melanoma treatment if: <1mm thick
    1-2mm thick
    Subungual melanoma
    • Local resection with 1 cm margin
    • Local resection with 1-2 cm margin and sentinel node biopsy
    • Amputation
  34. DDx of an epiphyseal/subchondral lesion 4
    • CHondroblastoma
    • Infection
    • GCT
    • Clear cell chondrosarcoma
  35. DDx of sacral lesion on young patient 5
    • GCT
    • ABC
    • Ewings
    • CHordoma
    • Osteosarcoma
  36. 4 primary bone tumors treated with wide resection alone
    • Chondrosarcoma
    • Paraosteal osteosarcoma: low grade only
    • Chordoma
    • Adamantinoma
  37. dripping candle wax” appearance with dense hyperostosis that flows along the cortex of the bone: diagnosis
    Melorheostosis
  38. Melorheostosis: Typical appearance
    Prognosis
    Treatment
    • Dripping candle wax
    • Benign
    • Symptomatic: treat if severe contractures,limited mobility, pain
  39. Leiomyosarcoma: cell origin

    Treatment
    Smooth muscle lining blood vessels

    Early wide resection +/- chemo
  40. Mazabraud syndrome: what is the pathology
    Multiple intramuscular myxomas associated with fibrous dysplasia
  41. Intramuscular fibromas: are

    Differentiate from myxoid liposarcoma

    Treatment
    Benign soft tissue mass confined to skeletal muscle: intramuscular

    Liposarcomas are intermuscular

    Marginal excision: if symptomatic
  42. Marjolin ulcer: is
    SCC resulting from chronic draining wounds or burn scars

    Rate of transformation is 1%
  43. GLomus tumor: location
    symptoms 3
    3 physical exam findings

    treatment
    Subungual region

    • Paroxysmal pain
    • Exquisite tenderness to touch
    • Cold intolerance

    • Small bluish nodule: difficult to see if subungual
    • Love test: pressure with pinhead leads to exquisite tenderness
    • Hildreth test: Tourniquet inflation reduces pain and tenderness caused by love test

    Marginal excision is curative
  44. Soft tissue mass with phlepboliths: dx
    Need to rule out

    Treatment options 3
    • Soft tissue hemangioma
    • Angiosarcoma: use MRI

    • Non-op: NSAID's stockings, activity modification
    • Sclerotherapy or embolization
    • Surgical excision
  45. Tumoral calcinosis: pathophysiology
    What is a secondary cause of it
    Treatment
    Metabolic dysfunction of phosphate regulation: leads to periaricular calcinosis in extracapsular soft tissues

    Renal failure

    Treat like HO: Remove once mature
  46. Difference btw neurofibroma and schwanoma
    Neurofibroma: from non-myelating schwann cells as well as fibroblast (predominant)

    Schwannoma: Schwann cells alone

    • ON MRI
    • Schwanoma: eccentric to nerve
    • Neurofibroma: Central to nerve fibers
  47. Plexiform neurofibroma: dx
    Neurofibromatosis
  48. 7 diagnostic criteria for NF
    • 2 or more neurofibromas or one plexiform neurofibroma
    • Axillary/groin freckling
    • 6 or more cafe au lait spots: 5mm if prepubertal or 15mm in postpubertal
    • Sphenoid wing displasia or CPT
    • 2 or more lisch nodules
    • Optic glioma
    • 1st degree relative with NF1
  49. Neurofibroma: can transform to
    Rate malignant transformation
    • MPNST
    • 5% of patients with NF
  50. MPNST: arise from
    treatment
    Stains positive for
    • Neurofibromas
    • Wide surgical resection +radiation
    • S100
  51. Ostoblastoma: location
    Size
    Associated with
    NSAIDs
    Management
    • Most common in posterior elements spine
    • >2cm osteoid osteoma: has a nidus
    • Secondary ABC
    • Does not respond to NSAID's

    • Surgery: does not respond to non op
    • Curetage or marginal excision with bone grafting
  52. Difference btw pre and post op RT:
    Wound complications
    Edema/stiffness/fibrosis
    Radiation field
    Pseudocapsule
    Tumor shrinkage
    Local recurrence
    Image Upload 1
  53. Radiotherapy sideeffects: 3 early
    3 late
    • Delayed wound healing
    • Infection
    • Desquamation

    • Stiffnes
    • Secondary sarcoma
    • Fractures
  54. 6 risk factors for post radiation fracture
    • Radiation dose >59 Gy
    • Weight bearing bones: femur
    • Female
    • Anterior femoral compartment excision
    • Periosteal stripping
    • Osteoporosis
  55. Most common primary spinal tumor in adults
    Chordoma
  56. Chordoma: most common location
    Metastasis to
    Physical exam
    Treatment
    • Sacrum and coccyx in midline
    • Lung: 30-50%
    • Palpable on rectal
    • Wide resection including nerve roots: radiation if negative margin not achieved
  57. Chordoma: how to preserve bowel/bladder function
    • Bilateral S2 nerve roots
    • Unilateral S2-4 nerve roots
  58. Malignant Fibrous  Histiocytoma (MFH): AKA
    Presentation
    Location
    Risk factors
    Mutation
    Good prognosis
    Treatment
    • Undifferentiated pleomorphic sarcoma
    • Similar to osteosarcoma: but lacks osteoid formation
    • Most common metaphysis of long bones
    • 25% arise from bone infarcts, pagets, radiation
    • P53
    • >90% necrosis to neoadjuvant chemo

    Neoadjuvant chemo + wide resection + post op chemo +/- radiation(if questionable margins)
  59. Chondrommyxoid fibroma: location
    Mutation
    Negative prognostic factors 2
    Treatment
    • Long bone metaphyseal eccentric: lytic
    • Chromosome 6 (q13)
    • Children + lobulated tumor with abundant myxoid material
    • Extended intralesional curetage and bone grafting
  60. Primary bone lymphoma: most common type
    Most common sites
    Prognosis
    Appearance x ray
    Histology
    Treatment
    • Non hodgkin b cell lymphoma
    • Pelvis, spine, ribs
    • Better than secondary lymphoma of bone
    • Large ill defined diffuse lytic lesion with mottled appearance
    • Small round blue cells
    • Multiagent chemo +/- radiation (in persistant disease)
  61. Jail bar vertebrae: dx
    Hemangioma
  62. Hemangioma:2 interventions for symptomatic patients
    • Curetage and bone grafting
    • Low dose radiation: 25-40Gy)>>if lesion is not accesible
  63. 3 advantages of prophylactic fixation over pathological fracture fixaton
    • Shorter operative time
    • Decreased morbidity
    • Quicker recovery
  64. What is mirel criteria
    Image Upload 2
  65. 4 factors that affect prognosis of bone tumors in general
    • Metastasis
    • Skip lesions
    • Histologic grade
    • Tumor size
  66. Describe the ennekin classification of benign bone tumors
    • Latent: NOF, enchondroma
    • Active: ABC, UBC, Chondromyxoid fibroma, chondroblastoma
    • Aggresive: GCT
  67. Describe the 3 variables in the ennekin classification of malignant tumors
    • Grade: low or high
    • SIte: Intracompartmental (within bone cortex) or extracompartmental (outside bone cortex)
    • Mets: yes or no
  68. Schwannoma: common location
    Mutation
    MRI finding
    Histology pathogomonic finding
    Treatment
    • Flexor surface of extremities and head and neck
    • NF-2
    • String sign
    • Verocay bodies
    • Excision if symptomatic
  69. Synovial chondromatosis: Pathophysiology
    most common location
    Problem
    Treatment
    MRI finding
    • Metaplasia: synovial transformation to chondroid tissue
    • Knee
    • Multiple intra articular loose bodies
    • Symptomatic
    • Cartilage nodules only visible on MRI initially
  70. Most common sarcoma in children
    Rhabdomyosarcoma
  71. Rhabdomyosarcoma: 4 subtypes

    Best survival
    Fatal
    Embryonal: infants

    Alveolar: Adolescents

    Botryoid: infants..in vagina

    Pleomorphic: older patients

    • Embryonal has best survival
    • Botryoid is fatal
  72. Rhabdomyosarcoma: Histology 
    Treatment (old vs young)
    • t2:13 translocation
    • Small round blue cells

    In young: Wide excision and chemo

    In Old:Wide excision and radiotherapy
  73. Most common soft tissue sarcoma in the foot
    Synovial sarcoma
  74. extra abdominal desmoid tumor: 2 associated syndromes
    Most common location
    Lab test that is high
    metastasis
    Medical management
    Surgical mgmt
    • Gardner and familial adenomatous polyposis
    • Shoulder>chest wall/back>thigh
    • Beta catenin
    • No risk
    • Low dose chemo or tamoxifen: since it has 100% estrogen receptor
    • Wide excision with radiotherapy
  75. Principles of tumor biopsy 5
    • Longitudinal incision in line with extensile approach
    • Avoid neurovascular structures
    • Maintain hemostasis
    • Through one compartment only
    • Drain in line with incision
  76. Most common soft tissue sarcoma in the hand and wrist
    Epithelioid sarcoma
  77. Epithelioid sarcoma: age
    Presentation
    Treatment
    • Adolescent and young adults
    • Painless growing mass

    Wid excision +adjuvant radiotherapy + sentinel LN biopsu
  78. Most common: Site of fracture secondary to mets
    Solid tumor in children
    Primary spinal tumor in adults
    Sarcoma in children
    Soft tissue sarcoma of the hand
    Soft tissue sarcoma of the foot
    Benign bone tumor
    • Proximal femur
    • Neuroblastoma
    • Chordoma
    • Rhabdomyosarcoma
    • Epithelioid sarcoma
    • Synovial sarcoma
    • Osteochondroma
  79. Adamantinoma: Is
    Location
    Age
    Mets
    Classic imaging
    Treatment
    • A low grade malignant tumor
    • Mid tibia: anterior cortex
    • Yong adults: 20-40
    • Can go to lung: 25%
    • Soap bubble appearance: multiple sharply circumscribed lucent lesions with interspersed sclerotic bone in the mid tibia
    • Wide surgical excision:often requires intercallart resection
  80. 3 types of langerhans cel histiocitosis
    • EG: single self limiting lesion in younger patients
    • Hand Schuller Chirstian: Disseminated LCH with bone and visceral involvement
    • Letterer-siwe disease: fatal...occurs in young children
  81. Hand schuller christian: what is it

    classic triad

    Treatment
    Type of langerhans cell histiocytosis

    • Lytic skull lesions
    • Diabetes insipidus
    • exophthalmos

    Chemotherapy and referal to pediatrics for assessment of systemic disease
  82. EG: histology
    Mgmt
    Coffee bean idented nuclei

    COnservative: usually a self limiting entity
  83. Paraosteal osteosarcoma: location
    Prognosis
    x rays
    treatment
    • Metaphysis of long bones: most common posterior distal femur
    • 95% long term survival if local control achieved
    • Heavily ossified lobulated mass arising from cortex: appears stuck on cortex

    Wide local excision: no need for chemo unless high grade component on histology
  84. Osteofibrous dysplasia: location
    deformity
    x ray
    problem with this pathology
    • Anterior tibia cortex
    • Anterior or anterolateral bowing of tibia
    • Anterior eccentric lytic lesion in tibia
    • Pseudoarthrosis: 10-30%
  85. Myositis ossificans:caused by 2
    2 most common locations
    Natural history
    Non op mgmt
    • Direct trauma/intramuscular hematoma
    • Quads/brachialis
    • Mass begins to decrease after one year
    • Rest and activity modification: avoid passive stretching
  86. 4 indications for chemotherapy in orthopaedic oncology
    • Osteosarcoma
    • Dedifferentiated chondromsarcoma
    • Malignant fibrous histiocytoma
    • Ewing sarcoma
  87. Side effect: Doxyrubicin
    bleomycin
    CYclophosphamide
    • Cardiotoxicity: leads to CHF
    • Pulmonary fibrosis
    • Myelosupression
  88. multiple myeloma: types of proteins
    Heavy chains (IgG and light chain (kappa or lambda)
  89. MOst common primary bone malignancy
    Multiple myeloma
  90. 5 poor prognostic factures in multiple myeloma
    • CHromosome 13 deletion or translocation
    • CIrculating plasma cells
    • increased beta 2 microglobulin
    • Decreased serum albumin
    • INcreased marrow microvessels
  91. Multiple myeloma mnemonic for end organ damage
    CRAB

    • Hypercalcemia
    • renal insufficiency
    • Anemia
    • BOne lesions
  92. Multiple myeloma: labs to order 4
    • SPEP/UPER
    • CALCIUM
    • HEMOGLOBIN
    • CREAtinine
  93. Multiple myeloma: medical management 3
    • Chemotherapy
    • Stem cell transplant
    • Bisphophonates: reduce skeletal events
  94. Multiple myeloma: cell type involved
    type ofimmunoglobulin
    If only one lesion
    • Plasma cell
    • Light chains
    • Solitary plasmocytoma
  95. Heterotrophic ossification: most common location
    Pathology associated with elbow HO
    • Between muscle and joint capsule: hip>knee>elbow>shoulder
    • Brain trauma
  96. Heterotrophic ossification risk factors

    In acetabular #
    In THA
    in TKA
    • HIgh ISS
    • TBI: in spastic limb
    • SCI: compete >incomplete
    • Prolonged coma
    • Decubitus ulcers
    • DISH/ank spond
    • Femoral nail: worse if piriformis entry
    • Surgical approach: iliofemoral > kocher langenbech > ilioinhuinal
    • THA approach: Anterior > lateral > posterior
    • TKA if notching or periosteal stripping
  97. Best test for early diagnosis of HO
    Triphasic bone scan
  98. Heterotrophic ossification abnormal labs (4)
    • ALP: increased > removes inhibitors of mineraization
    • CRP: Elevated >>once normalized may be sign of maturity
    • ESR: elevated 12 weeks  after THA is predictor for HO
    • CK: correlates with muscle involvement
  99. Heterotrophic prophylaxis 2 types
    INdomethacin 75mg/day for 10 days to 6 weeks: literature seems to show no great benefit

    Radiotherapy: single dose of 700cGy within 72 hrs of surgery
  100. Most common benign bone tumor
    Osteochondroma
  101. Osteochondroma: Pathophysiology
    ages
    Inheritance
    Gene
    Malignant transformation to
    • Derived from aberrant cartilage from the perichondral ring
    • 10-20 years old
    • Autosomal Dominant
    • EXT gene: loss of regulation of indian hedgehog
    • Low grade chondrosarcoma
  102. 3 causes of solitary osteochondromas
    • SH fracture
    • Radiation
    • Surgery
  103. Multiple hereditary exostosis: Inheritance
    Gene
    Which gene is worse
    malignant transformation %
    • Autosomal dominant
    • EXT1-3
    • EXT 1
    • 5-10%: proximal lesions more likely than distal lesions
  104. osteochondroma: xray description
    Cortex of lesion in continuity with cortex of native bone...medullary cavity also in continuity with native bone
  105. 4 associated deformities caused by MHE
    Osteochondromas lead to

    • Subluxation radiocapitellar joint
    • LLD
    • Valgus deformity knee
    • Ulnar deviation of wrist
  106. Most common primary sarcoma of bone
    Intramedullary osteosarcoma
  107. Intramedullary osteosarcoma: IN elderly associated with
    Most common location
    Location of mets
    Genetic mutation
    • PAgets disease
    • Distal femur/proximal tibia
    • Lung
    • Mytation in Rb gene
  108. 6 poor prognostic factors in intramedulalry osteosarcoma
    • Advanced stage
    • Response to chemo: tumor necrosis from sample
    • Tumor size
    • High serum alk phos
    • High LDH
    • +ve margins
  109. Intramedullary osteosarcoma: classic x ray findings 3
    • Sunburst periosteal reaction
    • Codman triangle
    • Blastic destructive lesion
  110. Intramedullary osteosarcoma: treatment

    good prognostic factor
    Multi agent chemotherapy and wide resection

    • Pre op chemo: 8-12 weeks
    • Post op chemo: 6-12 months

    Tumor necrosis >98%: good prognosis
  111. Ewing sarcoma: age
    Location
    Genetics mutation
    Abnormal protein
    histology
    • 5-25 years
    • 50% in diaphysis of long bonesTranslocation T11:22
    • EWS-FLI1
    • Small round blue cells
  112. Ewing sarcoma 8 poor prognostic factors
    • Tumor size >  8cm or 100cm3 
    • Spine and axial worse than proximal extremity worse than distal extremities
    • Age >14
    • Male
    • Elevated LDH
    • p53 mutation
    • Metastases: MOST IMPOSTANT PROGNOSTIC FACTOR
    • Chemo responce: <90% necrosis
  113. Ewing sarcoma 2 classic x ray findings
    • onion skinning
    • sunburst priosteal reaction
  114. Ewing sarcoma treatment
    Neoadjuvant chemo + wide resection + adjuvant chemo +/- adjuvant radiation

    Radiation of: positive margins, metastasis, poor chemo response
  115. 3 complications of medical management of ewing sarcoma
    • Bone sarcoma: secondary to radiotherapy ...risk 10-20% at 20 years
    • Treatment related acute myeloid leukemia/myelodysplasia
    • Metastases
  116. myxoid liposarcoma: translocation
    t12:16
  117. Liposarcoma and risk of mets: 3 categories
    • Low grade (well differentiated(: < 1%
    • Myxoid (intermediate grade): 10-30%
    • High grade: >50%
  118. Atypical lipomatous tumor: stain for
    MDM2/CDk14
  119. Treatment of liposarcoma
    Low grade (ALT): wide resection>>>low risk of mets/dedifferentiation

    Intermediate or high grade

    Wide surgical excision and adjuvant radiotherapy
  120. Pre chemotherapy work up
    • Fertility consult
    • Echocardiogram
    • CBC
    • 24hr urine
  121. SOft tissue sarcoma:site of mets
    Lng
  122. Soft tissue sarcoma: 3 negative prognostic factors
    • High grade
    • size >5cm
    • Tumor deep to the fascia
  123. Soft tissue sarcoma: treatment
    Radiation and wide surgical excision

    50-60 Gy
  124. Soft tissue sarcoma: radiation dose
    If given pre
    If given post
    • 30% wound complication
    • Greater radiation induced morbidity and chance of radiation induced sarcoma: need bigger fields
  125. Radiation dose for: adjuvant soft tissue sarcoma
    Mets
    POst op HO prophylaxis
    PVNS
    • 50-60 Gy
    • 30 Gy
    • 6 Gy
    • 30-35 Gy in 15 fractions or 50Gy in 25 fractions
  126. 3 cancer lets tthat are purely lytic
    Renal, thyroid, lung
  127. Spine metastasis: how to determine life expectancy
    2 options in management
    Tokahashi score: >8 means  >6 months to live

    palliative: pain control and radiotherapy

    SUrgery: decompression stabilization and post op radiation
  128. Chondroblastoma: age
    Location
    Matrix
    Mets
    x ray finding
    HIstology
    Treatment
    • 80% age <25
    • Epiphyseal: distal femur and proximal tibia most common
    • CHondroid on histology
    • To lung but are benign: similar to GCT
    • Well circumscribed epiphyseal lytic lesion with rim of sclerotic done: can have cortical expansion, punctate calcification not always present
    • Cobblestone or chickenwire pattern
    • Extended intralesional curetage and bone grafting
  129. Pagets disease: pathophysiology
    Casued by
    Inheritance
    Medical problem
    Malignant transformation to 3 things
    • Abnormal bone remodelling: excessive bne resorption and abnormal bone formation
    • Paramyxovirus or RSV
    • Mostly spontaneus: if inherited its autosomal dominant
    • Hiho output cardiac failure

    Osteosarcoma, fibrosarcoma and chondrosarcoma
  130. Pagets disease 5 ortho manifestations
    • Bone pain
    • Long bone bowing
    • Fractures: tend to be transverse
    • Large joint arthritis
    • Secondary sarcoma
  131. COtton wool exudates in skull: dx
    Pagets disease
  132. Pagets disease lab values: ALP
    calcium
    • Elevated
    • Normal
  133. Pagets symptomatic  medical management
    Aim for osteoclast inhibition

    • Bisphosphonates
    • Calcitonin: decrease size and resorptive capacity of osteoclasts

    Teriparatide is contraindicated bc of risk of secondary sarcoma
  134. Periosteal osteosarcoma: location
    Mutation
    Good prognosis
    Poor prognosis
    x ray
    Histology
    Treatment
    • diaphysis of long bones
    • p53
    • 98% tumor necrosus
    • Expression of MDR gene: pumps chemo out of cell
    • Periostal sunburst or hair on end reaction
    • Osteoid and chondrobalstic matrix

    Neoadjuvant chemo >restage > wide resection > post op chemo
  135. PVNS:histology
    location
    If in hand
    Genes affected
    x ray findings
    MRI
    Arthroscopic appearance
    • Proliferation of synovial villi and nodules: hemosiderin stained multinucleated giant cells
    • Intra articular: Knee > hip > ankle
    • Giant cell tumor of nerve sheeth
    • 5q33: increased CSF1 expression
    • Csystic erosion with sclerotic margins on both sides of joint
    • Blooming artifact: becasue of iron in hemosiderin deposits
    • Brownish/red inflammaed synovium
  136. PVNS treatment
    COmbined open/arthroscopic synovectomy: marginal excison

    In giant cell tumor of nerve sheeth: marginal excison is ok but recurrence is high

    External beam radiotherapy: reduces rate of recurrence to 10-20%
  137. UBC: caused by
    Age
    Location
    What happens at skeletal maturity
    • Temporary failure of medullary bone formation near physis<20
    • Metaphysis adjacent to physis: proximal humerus most common
    • UBC decrease in size and may heal
  138. UBC classification
    Latent: if normal bone separates UBC from physis

    Active: Cyst adjacent to physis
  139. UBC  xray findings 3
    • Central, lytic, well demarcated metaphyseal lesion
    • Cystic expansion with symmetric thinning or cortices: not wider than physis
    • Fallen leaf sign: path fracture with fragment at the base of empty cyst
  140. UBC treatment
    Immobilization after path fracture: 15% heal after farcture

    Aspiration and methylprednisone injection: for active cysts...may require multiple injections

    Curetagge, bone grafting and internal fixation: avoid in active UBC bc communication with physis may lead to growth arrest
  141. ABC: age
    Location in spine
    x ray description
    MRI finding
    • <20
    • Posterior elements
    • Bubbly appearance: expansile, eccentric lytic lesion with bony septae
    • FLuid/fluid levels
  142. ABC associated with 5 other tumors
    • GCT
    • CHondroblastoma
    • Fibrous dysplasia
    • Chondromyxoid fibroma
    • NOF
  143. ABC always think to rule out
    Teleangectatic osteosarcoma
  144. ABC treatment: if fracture

    If no fracture and symptomatic
    Non op until fracture heals

    Agressive curetage and bone grafting +/- adjuvants

    • Phenol
    • Argon beam
    • Liquid nutrogen
  145. Synovial sarcoma: location
    Gene mutation
    Metastasis
    Most important positive prognostic factor
    Treatment
    • Arises near joints but rarely within joint: most common sarcoma of the foot
    • Translocation t(x:18): forms SYT-SSX1/2
    • Common (30%-60%): goes to lung
    • SYT:SSX2: better prognosis
    • Wide resection and adjuvant radiotherapy: chemo may be beneficial
  146. Sarcomas that can metastatize to LN 5
    • Synovial
    • Epithelioid
    • Angiosarcoma
    • Rhabdomyosarcoma
    • Clear cell
  147. Osteoid osteoma location: lower extremity
    Spine
    Hand
    Foot
    • Proximal femur > tibial diaphysis
    • Posterior elements: Thoracic and lumbar > cervical in concavity of scoliosis
    • Scaphoid and proximal phalanx
    • Talar neck
  148. Osteoid osteoma: histology
    Symptoms
    ETOH
    Size
    Resolution of scoliosis if
    • Nidus containing osteobalsts surrounded by sclerotic zone (reactive zone)
    • pain worse at night and relieved by NSAID's
    • Makes symptoms worse
    • <2cm
    • Early resection within 18 months in kids <11
  149. Osteoid osteoma vs OSteoblastoma:
    Incidence
    Size
    Site
    Location
    Natural historrt
    Symptoms
    Management of spine lesions
    Image Upload 3
  150. Osteoid osteoma: non op mgmt
    Operative
    NSAID's until it resolvea

    RFA: 90% succesfully treated with 1-2 sessions of rfa

    Surgical resection and curetage: digital lesions (RFA would burn skin) or lesions close to nerves/vessels (in spine)
  151. MOst common benign tumor in childhood
    NOF
  152. NOF: incidence
    Location
    associated syndrome
    x ray finding
    Treatment
    • 30% of children with open physis have NOF
    • Metaphysis of long bones
    • Jaffe-campanacci syndrome: multiple NOF, cafe au lait, mental retardation, heart eyes and gonads involved

    Metaphyseal eccenttic buccly lesion surrounded by sclerotic rim: as child grows lesion migrates towards diaphysis

    Observation and follow up: curetage and bone grafting in the case of pathologic lesion
  153. Enchondroma: matrix composition
    Most common location
    Pathophysiology
    X ray appearance
    Treatment
    • Hyaline cartilage
    • Hand (60%) > feet
    • Chondroblasts escape from the physis into medullary canal and begin proliferating there
    • Well defined central lucent medullary lesions that calcify over time (pop corn)

    Serial x rays for n patients and long term follow up in mafucci or olliers disease
  154. Most common bone tumor in the hand
    ENchondroma
  155. Enchondroma 2 associated conditions
    Ollier's disease: Multiple enchondromatosis >>risk malignant tranformation < 30%

    Maffucci syndrome: Multiple enchondromas and sost tissue angiomas.....enchondromas expand cortices and angiomas seen as phleboliths >>>malignant tranformation 50-100%..can also have visceral malignancies
  156. Genetic inheritance: olliers disease
    maffuccii syndrome
    • None
    • None
  157. DIfference btw enchondroma and chondrosarcoma
    • ENchondroma erosion cortex <50% thickness
    • Chondrosarc erosion cortex >50% thickness (scalloping)
    • Chondrosarc leads to cortical thickenning and expansion...enchondroma lead to cortical thinning and expansion
    • Chondrosarc are larger: >5cm
  158. Fibrous dysplasia: pathophysiology
    Most common site
    Gene mutation
    • Failure of normal production of lamellar bone
    • Proximal femur
    • GS alpha protein:increased cAMP production
  159. Fibrous dysplasia 3 associated syndromes
    • McCune albright: cafe au lait, endocrinopathy, unilateral polyostotic FD
    • Mazabraud syndrome: polyostotic FD and soft tissue intramuscular fibromas

    Osteofribrous dysplasia: Location anterior tibial diaphysis > bowing
  160. Fibrous dysplasia: malignant transfomation to 3
    MFH, fbrosarcoma, osteosarcoma: 1%
  161. Fibrous dysplasia x ray findings
    • Central lytic lesions in medullary canal: may have cortical thinning and be expansile
    • Ground glass appearance
    • Sheppard crook deformity
  162. Fibrous dysplasia: non op mgmt

    Op magmt
    Observation if asymptomatic

    Bisphosphonates: in symptomatic polyostotic FD >>decreases pain and bone turnover

    ORIF with bone grafting, never use autograft, best choice is cortical allograft...Use nail if possible
Author
egusnowski
ID
345821
Card Set
Oncology - Orthobullets
Description
Oncologynorthobullets
Updated