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1

Describe this XRay?  15yo boy with painful mass.  Differential?  Top Diagnosis?  Prognosis and Prognostic factors?

EWING SARCOMA

  • Differential
    • ​ewing
    • osteosarcoma​​
    • less likely
      • leukemia
      • rhabdomyosarcoma​​
      • neuroblastoma (<5yo)
  • Ewing Sarcoma
    • 5-25 years of age
    • second most common bone tumor in children
    • ~50% are found in the diaphysis of long bones
  •  t(11:22) translocation 
    • found in all cases
    • leads to the formation of a fusion protein (EWS-FLI1)  
    • can be identified with PCR and useful to differentitate Ewing sarcoma from other round cell lesions
  • Prognosis
    • survival
      • 60-70% long term survival with isolated extremity disease at presentation and appropriate treatment/tumor response to chemotherapy
      • 40% long term survival with pelvis lesions
      • 15% long term survival if patient presents with metastatic disease
    • poor prognostic factors
      • spine and pelvic tumors
      • tumors greater than 100cm3
      • < 90% necrosis with chemotherapy
      • elevated lactic dehydrogenase levels
      • p53 mutation in addition to t(11:22) translocation

 

2

16yo female with painful thigh.  Top Diagnosis?  How will she present to you?  Radiographic findings typical of this?

 

Ewing Sarcoma

  • Presentation
    • pain often accompanied by fever
    • often mimics an infection
  • Physical exam
    • swelling and local tenderness
  • Radiographs
    • large destructive lesion in the diaphysis or metaphysis with a moth-eaten appearance 
    • Most common diaphyseal lesion
      • Common in pelvis
    • lesion may be purely lytic or have variable amounts of reactive new bone formation
    • periosteal reaction may give "onion skin" or "sunburst" appearance 
    • Codman's triangle
    • associated soft tissue mass
  • Bone scan
    • required as part of staging workup - will show very "hot" lesion
  • MRI
    • neccessary to identify soft-tissue extension and marrow involvement
    • often shows a large soft tissue component 
  • CT chest 
    • is required for appropriate staging to look for pulmonary metastasis

3

12 yo male comes in with fever, and painful left hip.  Below is his XR.  Diagnosis?  Managment?  Treatment?

Ewing Sarcoma

  • Differential
    • ​infection
    • leukemia
    • osteosarcoma
    • neuroblastoma (if young)
  • ​Radiology
    • permative, moth eatten appearance
    • ​diffuse, aggressive, lytic lesion with periosteal reaction
    • may be evidence of onion skinning or sunburst
    • ill defined margins, associated soft tissue mass
  • ​Further work-up
    • Labs - ESR, CRP, WBC, CBC and smear, LDH
      • ESR is elevated
      • WBC is elevated
      • anemia is common
      • lactic dehydrogenase (LDH)
    • Bone marrow biopsy 
      • required as part of workup for Ewing's to rule out metastasis to the marrow 
      • also assesses for translocation; translocation in normal marrow has increased risk of relapse
    • Molecular Analysis
      • ​CD99
      • PCR can confirm - 11, 22 translocation 
    • Bone scan - hot
      • assess mets
    • MRI - assess soft tissue mass
      • marrow involvement
    • CT chest - assess pulmonary mets
      • can help to plan radiation
  • ​Consult pediatric oncology and rad onc to discuss imaging
  • Treatment is multi-modal
    • preoperative chemotherapy given for 8-12 weeks (3 drugs)
      • doxyrubicin (cardiotoxicity)
      • vincristine
      • cyclophosphamide
      • isophosphamide
      • etoposide
    • Restage with MRI to assess response
      • ​>90% necrosis is good prognosis
    • Wide surgical resection with limb salvage or amputation 
      • Positive margins - re-resection and consider radiation
    • OR Radiation if difficult area, but this is falling out of favour due to long term complications
      • ​nonresectable tumor
      • wide metastatic disease
    • maintenance chemotherapy for 6-12 months  
    • Monitor for recurrent disease every three months for 2 years, then reduce intervals

4

Poor prognostic factors associated with Ewings

  • The most important factor is clincially evident metastatic disease
    • ​mets to lungs is better than distant boney mets
    • skip lesions better than distant boney mets
  • Location
    • spine and pelvic tumors (distal tumors have a better prognosis)
  • Size
    • tumors greater than 100cm3
    • or >8cm
  • Age >14 yo
  • Male
  • LDH >200IU
    • CRP/WBC elevation may be associted with mets and higher tumor burden
  • < 95% necrosis with chemotherapy
  • p53 mutation in addition to t(11:22) translocation
  • Relapse at < 2years

 

5

What is important for staging Ewings?

  • All present as stage IIb or III
  • plain radiographs and MRI of the primary site
  • CT chest - pulmonary mets
  • Bone Scan - skip lesions, boney mets
  • Bone marrow biopsy
  • ESR, CRP, LDH

6

Treatment of Ewings

  • preoperative chemotherapy given for 8-12 weeks (3 drugs)
    • doxyrubicin (cardiotoxicity)
    • vincristine
    • cyclophosphamide
    • isophosphamide
    • etoposide
  • Restage with MRI to assess response
  • Wide surgical resection with limb salvage or amputation 
    • Send to pathology to assess for margins and amount of necrosis
    • >90% necrosis good prognosis
    • positive margins = RT
  • OR Radiation if difficult area, but this is falling out of favour due to long term complications
    • ​nonresectable tumor
    • wide metastatic disease - should irradiate lungs even when improves with chemo
    • technique
      • 2-3 cm margin
      • 60 grey
  • maintenance chemotherapy for 6-12 months  
  • Monitor for recurrent disease every three months for 2 years, then reduce intervals
    • Relapse is common
    • treatment associated AML (5 years)
    • treatment associated myelosysplasia
    • sarcoma 2nd to radiation (10 years); 20%
    • carcinoma

7

Complications associated with radiotherapy in children

  • limb length discrepany
  • joint contracture
  • muscle atrophy
  • pathological fractures
  • secondary malignancy

8

Two class of criteria you can use to decide wether to prophylactically fix this?

Harington Critera

  • > 50% destruction of diaphyseal cortices
  • > 50-75% destruction of metaphysis (> 2.5 cm)
  • Permeative destruction of the subtrochanteric femoral region
  • Persistent pain following irradiation

Mirel's Criteria


 

9

What is the most common site of metastatic cancer?

thoracic spine

Always make sure to ask about neurological symptoms in any lesion that could be mets

10

A patient with metastatic disease present with confusion, lethary, polyuria, N/V and are severaly dehydrated.  What is the diagnosis?  What is the treatment?

Hypercalcemia 

  • Loop diuretics
  • fluid resussitation

11

Principles of tumor biopsy

  • Prerequisites
    • CBC, INR, PTT
    • Imaging
      • XR, CT
      • MRI - to look at most appropriate site of lesion
  • Indications
    • bone tumor
    • Where multiple sites need biospy (because of tumor heterogeneity)
  • Call your nearest tumor specialist to confirm you are dong appropriate biopsy, or ask if they would like referal
  • Incision
    • Keep the incision as small as possible
    • use longitudinal incision in the extremities
    • allows for extension of the incision for definitive management
  • Approach
    • do not expose neurovascular structures
    • all tissue exposed during the biopsy is considered contaminated with tumor
    • maintain meticulous hemostasis
    • release tourniquet prior to wound closure
  • Biopsy 
    • perform thru the involved compartment of the tumor, if possible threw one muscle
    • for bone lesions with a soft tissue mass, it is ok to perform the biopsy using the soft tissue mass
    • Make a circular or longitudinal hole, <10% bone diameter
    • Confirm with your pathologist that you have adequate specimen
  • Closure
    • if using a drain, bring drain out of the skin in line with surgical incision
    • layered tension-free closure

12

Prognosis and characteristics of common metastatic cancer?

Thyroid (lytic) - 48 months
Prostate (blastic) - 40
Breast (mixed) - 24
Kidney (lytic) - variable
Lung (lytic) - 6 months

myeloma (lytic) - 2-5 years

13

Work-up of this patient

  • History
    • Pain, qualify the pain, other sites of pain
      • This is the most common presenting symptoms
    • Constitutional symptoms or specific symptoms to any carcinoma
    • Chest pain, abdominal bloating, intolerance to hot and cold
    • Neurological symptoms
      • Myelopathy, weankness, walking aids, bowel/bladder
    • Risk factors - smoking, family history
    • Last pap smear, mammogram, colonoscope
  • Physical
    • Look for evidence of primary
      • Breast exam, thyroid mass, prostate (rectal)
      • Costovertebral tenderness = renal call
    • Look for evidence of mets
      • Ascultate the lungs, palpate the abdomen
      • Abdomenal exam
    • Full neurological exam including gait, ASIA
  • Workup for older patient with unknown primary
    • Plain radiographs
      • Full length films of lesion, chest
    • CT chest/abdo/pelvis
    • Can consider head
    • Technetium bone scan - rule out other lesions
    • Skeletal survery - Myeloma and thyroid cancer - cold bone scan
  • Blood
    • CBC and differential, ESR/CRP, INR, PTT
    • LFT, Cr/Urea
    • Ca, Phos, Alk Pho, LDH
      • ***Hypercalcemia can be fatal to patient
      • Helps to rule out metabolic bone disease (hyperparathyroid)
    • Immunoelectrophoresis - SPEP, UPEP
    • PSA, TSH
  • Imaging
    • Radiographs - full length films of affected area
    • Blastic - Prostate > breast > lung
    • Lytic - renal, lung, thyroid, uterine, adrenal, melanoma, GI
      • Not responsive to rads/chemo
      • Usually need to treat operatively
    • Elbow or knee = lung or renal
    • Cortical = lung
  • Biopsy
    • Indications to rule out primary
      • No history of cancer
      • History of cancer with no evidence of mets
      • When there is history of cancer and evidence of mets, ok to send reamings for path
    • See the principles of biopsy section
      • Use the easiest site to biopsy
      • Can do a frozen section intra-op and if the pathologist confirms carcinoma you can proceed with fixation
  • Histology
    • Mets - epithelial cells in clumps or glands in a fibrous stroma
    • Immunohistochemical stains positive
      • Keratin
      • CK7 - breast, lung
      • TTF1 - lung
  • Once you have the primary
    • Med Onc - chemo
    • Rad Onc - post-op radiation
    • Radiology - if you want them to be embolized pre-op

14

Treatment prinicples of metastatic lesion

  • Non-op
    • Indications
      • Small lesions with little disability
      • Upper extremity
      • Sensitive tumors
      • Blastic lesions
      • Short expected survival or short condition
    •  Bisphosphanate - have been shown to decrease secondary skeletal events (SSE) - funded fr breast, myeloma and prostate
    • Radiation
      • All require post-op unless death is imminent - otherwise you risk reoperation
      • Begin radiation after surgery - 3 weeks
      • Make sure you close your wounds well and can leave in the sutures
      • Radiation needs to include the entire fixation device
    • Chemo
      • Lymphoma/leukemia are very sensitive to rads/chemo so you usually don't need to do sx
  • Operative
    • Get imaging of the full bone including joint above and below
      • CT may be necessary
    • Embolization - renal cell, thyroid
    • Post-op RADS - helps to minimize recurrence and for pain control
    • Indications
      • Femur fractures
      • >2.5cm
      • >50% cortical lesion
      • Pain refractory to rads
      • Mirel's criteria
    • Hemiarthroplasy or tumor prosthesis
      • Required when there in an intra-articular, uncontained lesion
      • Can also be done for a proximal femoral neck fracture
      • Cemented
      • Considerations
        • Soft tissue deficient and instability
          • Adequate closure of capsule, abductors and ER's
        • High risk of infection
    • Prophylactic nailing 
      • Statically locked
      • Recon nail has a higher failure rate for both intertroch and subtroch
      • Can augment with PMMA
      • Don't forget to vent the femur if there is no fracture when reaming
      • *Doing more than one intramedullary device for multiple lesions can put a lot of stress on the pulmonary system - might be better to stage multiple lesions and allow the patient to recover from each nail
    • ORIF with PMMA
      • Indications
        • Humerus diaphyseal or metaphyseal with uncontained defect
        • Tibia - metaphyseal defect that is extra-articular
        • Impending and complete fractures
      • Curettage, cement, screw into cement
  • Spinal neurologic decompression, surgical stabilization, post-op radiation therapy
    • Met lesions to spine with compression of neuro elements

15

Treatment of humeral lesions

16

Treatment of tibial lesions

17

Tumors that require chemo

  • Indications
    • osteosarcoma (intramedullary and periosteal)
    • Ewing's sarcoma/primative neuroectodermal tumor
    • malignant fibrous histiocytoma
    • mesenchymal chondrosarcoma
    • Rhabdomyosarcoma
    • Synovical cell
  • Timing
    • ​pre-op 8-12 weeks
    • post-op 6-12 months
  • ​Common Drug
    • Doxorubicin
      • anthracycline antibiotic 
      • functions as a cytostatic agent
      • side effects
        • cardiac toxicity 

18

Describe the lesion.  Differential?  Diagnosis?  Associated conditions?

Non-ossifying fibroma

  • Differential for larger NOF
    • ABC
    • Chondromyxoid fibroma
    • Fibrous dysplasia
    • Desmoplastic fibroma

  • Associated conditions
    • Jaffe-Campanacci syndrome
      • congenital syndrome of multiple non-ossifying fibromas and
      • cafe au lait pigmentation
      • mental retardation
      • heart, eyes, gonads involved
    • neurofibromatosis
    • familial multifocal NOF
    • ABC

19

Describe this XR.  Differential?  Diagnosis?

Differential for metaphyseal fibrous defect

Osteoid osteoma
Intracortical abcess
Stress fracture
Intracortical osteosarcoma

20

Describe this XR?  Differential? Diagnosis?  Treatment?

Non-ossifying Fibroma

  • Radiographs
    • diagnostic
    • metaphyseal, eccentric, "bubbly" lytic lesion surrounded by sclerotic rim  
    • cortex expansion and thinning
    • as bone grows
      • migrates to diaphysis
      • lesions enlarge (1-7cm)
      • lesions become sclerotic as patient approaches skeletal maturity
      • avulsion of adductor magnus insertion in the posteromedial aspect of the distal femur may produce a similar looking lesion. 
  • CT
    • quantitative CT shown to be useful in predicting fracture risk
  • observation
    • first line of treatment
    • most lesions resolve spontaneously and observation alone is the treatment for most cases   
  • casting
    • pathologic fracture
    • can be treated as per the fracture alone (long leg casting for distal femur pathologic fx)
    • Once fracture heals treat with curettage and bone grafting
  • intralesional curettage and bone grafting
    • symptomatic and large lesion
    • increased risk of fracture shown on quantitative CT

 

21

Describe the XR.  Differential?  Managment?

Malignant Fibrous Histiocytoma

  • 20-30% arise from infection or implant
  • usually >50yo
  • Presentation
    • pain, swelling, pathological fracture
  • Imaging
    • ​lytic
      • ​no evidence of cortical reaction; too fast for bone to respond
    • destructive
    • metaphyseal
  • Mangement
    • ​requires a complete WU of solitary bone lesion
    • ensure no hypercalcemia
    • MRI to look at soft tissue invovlement
    • biopsy to confirm
  • ​Treatment
    • multi-agent chemotherapy and limb salvage resection
      • chemotherapy
        • preoperative 8-12 weeks
        • maintenance 6-12 months 
      • surgical resection
        • trend towards limb salvage whenever possible

22

Differential for multiple lesions in a young adult

EG

Fibrous dysplasia

Leukemia

Lymphoma

Hemangioendothelioma

Enchondroma / Olliers / Marfucci's

Osteochondroma / MHE

NOF / Jaffe-Campanacci syndrome

23

Differential for multiple lesions in an older adult

Paget's

Metastatic bone disease

Multiple myeloma

Lymphoma 

Hyperparathyroidism

Bone infarcts

24

Lesions that have a cold bone scan

multiple myeloma

melanoma

25

Sacral lesions < 40

Giant cell tumor

ABC

Ewing's

Osteosarcoma

26

Sacral lesions 40-80

Chordoma

Metastasis

Myeloma

Lymphoma

Chondrosarcoma

MFH

27

How to assess an XR for a tumour

 

  • Age
  • Location
    • Epiphyseal vs metaphyseal vs disphyseal
    • Medullary vs cortical
  • Geographic vs Permative - geographic will stay with-in the lesion​
  • What is the tumor doing to the bone?
    • Lytic vs blastic
    • infiltrative - moth eatten, permeative
  • What is the bone doing to the tumour?
    • Cortical reaction - expansile, sclerotic rimming, lysis
    • Periosteal reaction
    • Onion skinning, sunburst, spiculated
  • Mineralization/Matrix
    • Ground glass
    • calcification
  • Size and number
  • Associated soft tissue mass

28

Differential diagnosis by location

  • Surface lesions
    • Osteochondroma
    • Periosteal chondroma
    • Surface ABC
    • Parosteal osteosarcoma
    • Surface chondrosarcoma
  • Epiphyseal
    • chondroblastoma
    • Chondrosarcoma
    • OM
  • Epiphyseal-Metaphyseal
    • GCT
    • ABC
  • Metaphyseal
    • Unicameral bone cyst
    • Osteosarcoma
    • Enchondroma
    • Chondrosarcoma
    • Chondromyxoid fibroma
    • Non-ossifying fibroma
  • Diaphyseal
    • Osteoid osteoma
    • Osteoblastoma
    • Stress fracture
    • OM
    • Fibrous dysplasia
    • Adamatinoma
    • Ewings
  • Periarticular
    • Synovial chonromatosis
    • PVNS
    • NOT synovial sarcoma

29

Most common tumour of the sacrum?

chordoma

30

What is the most common malignant spinal tumour in adults?

chordoma