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AD Orthobullets Study Plan > Tumour > Flashcards

Tumour Flashcards

1
Q

Lifetime risk of malignant transformation for solitary osteochondroma?

A

1%

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2
Q

Two important differences between Maffucis and Olliers?

A

Maffucis has hemangiomas

Maffucis has higher risk of secondary malignant transformation (100% vs. 30%)

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3
Q

Which type of liposarcoma does not require adjuvant radiotherapy?

A

Well differentiated liposarcoma

Operative

  • marginal resection without radiotherapy
    • indications
      • well-differentiated liposarcoma
    • outcomes
      • low risk of local recurrence, metastasis extremely rare
      • dedifferentiation risk of 2% in extremities and 20% in retroperitoneal lesions
  • wide surgical resection with adjuvant radiotherapy
    • indications
      • intermediate grade liposarcomas
      • high grade liposarcomas
    • outcomes
      • radiation decreases local recurrence
      • chemotherapy may be beneficial in selected patients
      • myxoid liposarcomas with >10% round cells have high likelihood of metastasis
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4
Q

Can you do limb salvage in osteosarcoma in a patient with a pathologic fracture?

A

Yes

However increased rates of local recurrence and decreased survival overall in these patients

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5
Q

Adult Patient with osteochondrama has acute onset of pain at the location of lesion. What do you suspect?

A

Sarcomatous transformation

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6
Q

Treamtent for osteosclerotic myeloma

A

Chemo + radio+ plasmapheresis

Generally no surgery needed

(This is a form of MM associated with POEMS)

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7
Q

What is the multidrug resistance gene (MDR)

A

Pumps chemo out of cancer cells

Present in 25% of primaries

50% of mets

Very poor prognostic indicator

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8
Q

What primary is very vascular, requiring embolization of lesions primary to ORIF?

A

RCC

Thyroid

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9
Q

What does GCT look like on MRI?

A

Dark on T1 and T2

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10
Q

Ddx for lytic lesion in greater than 40 year old

A
  1. Mets
  2. MM
  3. Lymphoma
  4. Metabolic (browns Tumor)
  5. Sarcoma
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11
Q

Diagnosis?

A

Melorheostosis

Periosteal formation of new bone

Looks like dripping candle wax

Benign but painful

Symptomatic treatment ± excision

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12
Q

Name these translocations!!

  1. t(2;13)
  2. t(11;22)
  3. t(X;18)
  4. t(12:16)
A

  1. Alveolar rhabdomyosarcoma; t(2;13)
  2. Ewings Sarcoma; t(11;22)
  3. Synovial sarcoma; t(X;18)
  4. Myxoid liposarcoma; t(12:16)
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13
Q

Who gets Ewing’s sarcoma?

A

White males aged 10-20

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14
Q

What blood markers can be used to measure bone turnover?

A

ALP, LDH

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15
Q

What is stone man’s disease?

A

Fibrodysplasia ossificans progressiva

Hallmarks:

  • Progressive and EXTENSIVE heterotopic ossification (muscles, fascia, tendons, ligaments, joint capsules)
  • Congenital malformation of the great toe

Mutation in ACVR1 gene (activin A type I receptor)

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16
Q

Enchondromatosis is characterized by: (3 things)

A

A) multiple enchondromas, hemangiomas, lymphangiomas

B) multiple enchondromas in a unilateral distribution

C) auto-dominant transmission, multiple enchondromas and osteochondromas

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17
Q

Poor prognostic indicators in Multiple myeloma (9)

A

Renal failure (worst)

hypercalcemia

Stage

Type (plasmacytoma has best prognosis)

chromosome 13 deletion or translocation (t4;14), 4(14;16)

circulating plasma cells

increased beta 2 microgloblulin (indicates elevated tumor burden)

decreased serum albumin

increased marrow microvessels

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18
Q

Surveillance of soft-tissue & osteosarcoma?

(They are the same)

A

Physical exam, CXR, CT Chest

1-2 years: 3 months

3-5 years: 6 months

5-10 years: yearly

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19
Q

Common locations of ABC?

A

Femur > tibia > spine

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20
Q

What part of an osteochondroma is usually the site for secondary malignant transformation?

A

Cartilage cap

risk of malignant transformation is

  • <1% with solitary osteochondroma
  • ~5-10% with MHE develop secondary chondrosarcoma
  • cartilage cap >2cm is associated with increased chance of malignancy
  • mean age of diagnosis, 31yrs
  • seldom in 1st decade or after 5th decade of life
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21
Q

Name the tumours associated with NF1

A

Wilm’s tumour

Optic glioma - part of dx criteria

Neurofibroma
Neuofibrosarcoma

Astrocytoma

meningioma

melanoma

leukemia

rhabdoyosarcoma

pheochromocytoma

carcinoma

pancreatic endocrine tumors

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22
Q

Describe FU for solitary enchondroma with no concerning features:

A

serial x-rays

q3-6 months for 1-2 years, then annually

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23
Q

What is the incidence of post radiation sarcoma in an area previously radiated to treat malignancy?

A

13%

(more common if also had chemo)

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24
Q

Episyphyseal lesions?

A

Chondroblastoma, infection, GCT

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25
Q

What is the main dDx for intramuscular myxoma that you must rule out?

A

myxoid liposarcoma

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26
Q

Name some negative prognostic characteristics of osteosarcoma.

A
  1. Poor response to Neo adjuvant chemo (% necrosis)
  2. Tumour size (> 8 cm is bad)
  3. Patient age (old is bad)
  4. Higher stage of Tumor
  5. Anatomical site (central is bad)
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27
Q

Chrondroblastomas are located where?

A

Epiphysis

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28
Q

Lifetime risk of malignant transformation for olliers?

A

30%

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29
Q

Describe the Enneking system for classifying malignant tumours.

A

Three factors: Grade, site and metasteses

Grade is high or low

Site is intracompartmental (enclosed by natural barriers) or extracompartmental

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30
Q

Parosteal osteosarcoma with invasion into medullary cavity - what is the affect on prognosis?

A

No effect - still good prognosis

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31
Q

Name 2 scoring systems that may help you decide whether to operate on a pathologic spine tumour

A

Spinal instability neoplastic score

based on: location, pain, type of lesion, spinal alignment, vertebra body collapse, involvement of posterior elements

Score:

0-6: stable

7-12: impending instability

13-18: unstable

Modified Tokohashi Scoring system

based on: Karnofsky performance status, # of extraspinal bony mets, # of vertebral body mets, Mets to major organs, primary site of cancer, palsy

Score

0-8: <6 months life expecancy: conservative + palliative

9-11: life expectancy > 6 months: palliative surgery

12-15: life expectancy > 1 year. Excisional surgery

I think the principles of these scoring systems are imporant

JAAOS 2015

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32
Q

Presentation of glomus Tumour

A

Triad:

  • cold sensitivity
  • point tenderness
  • intermittent severe pain

Also may have a bluish-reddish hue

Occur in subungal region

X-rays will show a scalloped, osteolytic defect

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33
Q

Name the 5 subtypes of liposarcoma.

A

Well differentiated liposarcoma

Myxoid liposarcoma

Pleomorphic liposarcoma

Dedifferentiated liposarcoma

Mixed-type liposarcoma

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34
Q

What is the most common sarcoma in the foot?

A

Synovial sarcoma

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35
Q

Soft tissue tumours that mets via LN & to places other than lungs

A

SCREAM

Synovial sarcoma

Clear cell sarcoma

Rhabdomyosarcoma

Epitheliod sarcoma

Angiosarcoma

Myxoid liposarcoma

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36
Q

Name 2 sarcomas for which excisional bx can be performed

A

Parosteal osteosarcoma

low grade chondrosarcoma

*not sure I would say this on an exam

It’s from AAOS CORE2

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37
Q

What is a defining feature on histo for extra-articular desmoid tumour?

A

100% positive for estrogen receptor-beta

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38
Q

4 Options for treating PVNS (think techniques)

A

Arthroscopic synovectomy: best for focal disease

arthroscopic + open synovectomy (ie arthroscopic + open posterior knee approach)

Total joint arthroplasty + synovectomy

Total synovectomy + arthrodesis

± radiation - addition of radiation combined with total synovetomy reduces recurrence to 10-20%

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39
Q

2 negative prognostic indicators of CMF

A

children

lobulated with abundant myxoid material

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40
Q

Football player suffered injury after helmet to groin hit. Residual non-tender mass.

Diagnosis?

Treatment?

A

Myositis ossificans

Non-operative - most resolve in a year or so

If recalcitrant, excise, but not before it matures (> 6 months)

Note x-ray: it calcifies from outside in, vs. tumour, which goes inside out

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41
Q

Olliers is characterized by:

A) multiple enchondromas, hemangiomas, lymphangiomas

B) multiple enchondromas in a unilateral distribution

C) auto-dominant transmission, multiple enchondromas and osteochondromas

A

B

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42
Q

What findings differentiate chondrosarcoma from a benign lesion?

A

1) myxoid matrix - may still have chondroid matrix, but generally the more myxoid the higher the grade
2) cortical thinning or thickening
3) soft tissue mass
4) invasion of haversian system

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43
Q

5 tumors that can have an associated ABC?

A
  1. giant cell tumor
  2. chondroblastoma
  3. fibrous dysplasia
  4. chondromyxoid fibroma
  5. NOF
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44
Q

Fallen leaf sign

what is it

what is it pathognomonic for?

A

Cortical fragment in the bottom of an empty cyst

UBC

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45
Q

4 associated conditions of NOF

A

Jaffe-Campanacci syndrome

Neurofibromatosis

Familial multifocal NOF

ABC

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46
Q

What is the treamtnet of low-grade intramedullary osteosarcoma?

A

Surgial resection only

This is the exception of the usual rule of chemo-sx-chemo for sarcoma

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47
Q

Components of local staging?

A

MRI to characterize lesion (of entire bone)

Biopsy (can do soft tissue mass)

Send for pathology and cultures

Adequate hemostasis

Good lesional tissue

Use pathology to prognosticate

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48
Q

Physical exam for metastasis with unknown origin must include?

A

Thyroid exam

Lung exam

Abdomen exam

Breast/prostate exam

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49
Q

Describe the bones in enchondromatosis?

A

Dysplastic

They are not normal and can have deformities

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50
Q

Young, thirsty kid with these findings.

Diagnosis?

A

Hans-Schuller-Christian disease (disseminated eosinophilic granuloma)

  • Classic triad:
  • Exophthalmos
  • Diabetes insipidus
  • Lytic skull lesions

chronic, disseminated form with bone and visceral lesions

also known as Langerhans cell histiocytosis with visceral involvement

Langerhan’s cells

  • mononuclear histiocyte-like cells with oval nuclei with well-defined round or oval cytoplasm.
  • a prominent nuclear groove (coffee bean nuclei) can be seen in most of the nuclei
  • eosinophilic cytoplasm (pink generally)
  • stain with CD1A
  • electronmicroscopy
  • birbeck granules seen inside Langerhan’s cells

Brace the scoliosis, chemo for HSC (diffuse involvement)

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51
Q

What malignant tumour can secondarily arise from a bone infarct, Paget’s disease or prior radiation?

A

Malignant fibrous histiocytoma

(aka Undifferentiated pleomorphic sarcoma)
risk factors:

25% arise as secondary lesion from bone infarct, Paget disease, or prior radiation

Histology

  • pleomorphic spindle cells and histiocytic cells in storiform pattern
  • malignant multinucleated giant cells with grooved or indented nuclei
  • areas of chronic inflammatory cells
  • variable collagen production
  • fibrous fascicles radiate from focal hypocellular areas
  • hemorrhagic and necrotic regions not infrequent; suggest high-grade lesion

Treatment Same as Osteosarcoma

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52
Q

What do you do following unplanned excision of soft tissue sarcoma?

A

Work up as normal

Local staging to look for extent of tumour bed and contamination

Systemic staging to look for mets

Definitive is re-resection of tumour bed and all associated contaminated compartments

Radiotherapy (controversial whether pre or post-op)

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53
Q

Tumour association with poly-vinyl chloride

A

Angiosarcoma

very aggressive vascular tumour

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54
Q

Overall prognosis for multiple myeloma?

What confers the worst prognosis?

A

Poor

Median survival 3 years from diagnosis

5 year survival: 30%

10 years survival: 10%

Shortest survival in patients with renal failure

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55
Q

Name 3 syndromes assocaited with Fibrous Dysplasia

A
  1. McCune Albright [condition defined by the presence of

skin abnormalities (cafe au lait spots in coast of Maine pattern)

endocrine abnormalities (precocious puberty)\

renal phosphate wasting due to FGF-23 (oncogenic osteomalacia)

unilateral polyostotic fibrous dysplasia

obtain AP spine radiographs to look for scoliosis]

  1. Mazabraud -

[polyostotic fibrous dysplasia

soft-tissue intramuscular myxomas]

  1. Osteofibrous dysplasia

“alphabet soup” or “chinese letters”

1% conversion to sarcoma risk

Bisphosphonate therapy indications symptomatic polyostotic fibrous dysplasia effective in decreasing pain and reducing bone turnover

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56
Q

What sarcoma has flat epithelial cells on pathology?

A

Synovial sarcoma

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57
Q

What is radiation dose for HO prophylaxis?

A

6Gy

(vs. 45-60 for soft tissue sarcoma)

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58
Q

Genetics for myxoid liposarcoma

A

t(12;16)

Creates CHOP-TLS fusion protein

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59
Q

What physeal zone does osteochondroma/MHE stem from?

A

Proliferative

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60
Q

What Tumor stains for MIC2 antigen?

A

Ewing’s sarcoma.

This is how you differentiate it from other small round blue cell tumors.

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61
Q

What is the most common malignant bone tumour of the hand?

A

Chondrosarcoma

THINK: common to have enchondromas in hand, so one of them must transform

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62
Q

3 non-neoplastic things to consider adding on to the end of a ddx for bone lesion?

A

EG

Infection

Hyperparathyroidism (if older)

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63
Q

What is the recurrence rate of osteoid osteoma treated with percutaneous radiogrequency ablation?

A

10-15%

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64
Q

Describe Harington’s Criteria

A

>50% destruction of diaphyseal cortices

>50-75% destruction of metaphysis (>2.5cm)

Permeative destruction of the subtrochanteric fermoal region

persistent pain following irradiation

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65
Q

Treatment of osteofibrous dysplasia?

A

nonoperative - observation

bracing if deformity interfering with walking

OR if you need correction of deformity (osteotomy)

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66
Q

Cause of Paget’s

A

Thought to be a slow viral infection by paramyxovirus or RSV as most are spontaneous

There is a small group of heritable Paget’s also

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67
Q

What kind of matrix does ewings usually have?

A

None

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68
Q

Poor prognostic indicators for EG

A

involvement at young age

rapid disease progression

organ involvement (eg pituitary, lung, hematopoietic, or liver involvement);

*organ dysfunction carries an especially poor prognosis

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69
Q

Older than 40 , blastic lesion DDx (4)?

A

Metastases (prostate)

Bone island

Bone infarct

Osteoblastoma

Infection

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70
Q

Name two enchondroma syndromes.

A
  1. Olliers
  2. Maffuccis
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71
Q

Classic findings of Hans-Christian-Schuller Disease

A

Disseminated form of EG

Triad:

Skull lesions

diabetes insipidus (thirst)

Exopthalmos (double vision)

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72
Q

Osteolysis in tumour is caused by the action of what cytokine on what cell?

A

RANKL on osteoclastic cells (not on tumour cells)

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73
Q

Genetically, how does osteofibrous dysplasia differ than regular fibrous dysplasia?

A

It doesn’t have the Gs alpha activating mutation

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74
Q

Name the basic biopsy principles.

A
  1. Biopsy through (single) involved compartment
  2. Use extensile incision (usually longitudinal)
  3. Meticulous hemostasis
  4. Do not create multiple planes
  5. Bring out drains in line with incision (so the tunnel can be removed with definitive resection)
  6. should biopsy soft tissue component of bone tumour if possible
  7. round holes in bone, not square
  8. do not exanguinate before tourniquet
  9. avoid nv structures
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75
Q

Compare pre vs. post operative radiation for soft tissue sarcoma:

A

Pre/neoadjuvant RTx:

Lower dose

Lower field (b/c you haven’t contaminated it with surgery)

Lower rates of fibrosis

Also lower rates of edema and joint stiffness, but not statistically significant (Lower overall functional rates if they had fibrosis, edema and stiffness)

HOWEVER: higher risk of wound infection

Generally, neoadjuvant (pre) RTx is better, even tho wound complications are higher

SR2 study: know this data - it’s canadian

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76
Q

6 lesions on the posterior elements of spine

A

G: giant cell tumour (although most commonly in vertebral body)

O: osteoblastoma

T: tuberculosis

A: aneurysmal bone cyst (only one PURELY found posteriorly)

P: Paget disease

E: eosinophilic granuloma

GO TAPE

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77
Q

4 posterior element tumours

A

ABC

osteoid osteoma

osteoblastoma

osteochondroma

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78
Q

What systemic pathology (ie non ortho) do people with Paget’s disease get?

A

high output cardiac failure

due to increased turnover of bone

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79
Q

What percentage of vertebral body tumours are malignanet?

A

75%

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80
Q

What bone metasteses are not redio sensitive?

Which is the most readio-sensitive?

A

1) GI and Renal
2) Prostate

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81
Q

Classic spine finding in eosinophilic granuloma

Name 1 other classic finding

A

Vertebra plana aka platyspondia

Multiple punched out/lytic cranial lesions (ask for skull x-ray)

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82
Q

Which side of the spine in an osteoid osteoma normally found in?

A

Concavity of the spine

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83
Q

Histology of ewings sarcoma?

A

Small round blue cells

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84
Q

How do you classify UBC?

A

Active - in continuity with physis

Latent - bony bridge between UBC and physis

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85
Q

Most common location of mets in soft tissue sarcoma

A

Lungs

They can mets to LNs, but lungs are still most common

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86
Q

Survival rate of chondrosarcoma

A

Directly correlates with histological grade:

Grade 1: 90%

Grade 2: 60-70%

Grade 3: 30-50%

De-differentiated: 10%

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87
Q

What are the main steps in treatment of malignant bone tumour?

A
  1. Neo adjuvant chemotherapy
  2. Re-stage with imaging for response to therapy and operative planning
  3. Surgery - limb sparing vs. amputation
  4. Post-operative chemotherapy - use pathology to prognosticate
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88
Q

Treatment?

A

Chordoma

Wide resection is standard of care

± radiation if:

positive margins

inoperable tumour

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89
Q

What is the most common cause of painful scoliosis in the adolescent population?

A

osteoid osteoma

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90
Q

5 complications with treatment of UBC

A

recurrence

fracture

embolizatio of injected material

local reaction to injected material

growth disturbances

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91
Q

What kind of tumour results from malignant transformation of a chronic OM?

what is the treatment?

A

SCC

Treat with wide excision/amputation + adjuvant chemo/rads

Prognosis is poor with late diagnosis, good with early

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92
Q

Patient comes in post-MVC with a pathologic fracture through this. Dx as ABC. What is your plan?

A

Non-operative fracture management

Once healed, then proceed with intralesional curettage

Remember to treat path fractures non-op!

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93
Q

4 predisposing genetic factors for intramedullary osteosarcoma

A

Rb +

p53

Rothmund thomson syndrome

LiFraumeni syndrome

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94
Q

60 yo F with constitutional symptoms, pain palpable mass. (see picture)

Dx & treatment

A

Lymphoma

Treatment is surgical stabilization, chemo, radiation

*Lymphoma can have a soft tissue mass!

*lymphoma: small round blue cell

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95
Q

What do the terms onion skinning and sunburst refer to on radiographs? Which Tumors are each characteristic of?

A

Types of periosteal reaction

Onion skinning = ewings

Sunburst = osteosarcoma

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96
Q

Age range for GCT?

A

20-40

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97
Q

indications for excisional bx

A

superficial to fascia

Not overlying NV structure

excise AND LEAVE BEHIND FASCIA

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98
Q

“Pop corn densities” are useful in differentiating what secondary malignancy from its benign precursor?

A

Secondary chondrosarcoma from enchondroma.

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99
Q

What is the histo of periosteal osteosarcoma

A

osteoid + chondroblastic matrix

If no osteoid, will be classified as chondrosarcoma

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100
Q

What tumor shows predilection for the distribution shown in this figure?

A

Giant Cell Tumor

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101
Q

Desribe AJCC classification system

A

Soft soft tissue tumours:

See picture

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102
Q

Name 5 signs/symptoms that are concerning for chondrosarcoma, you know, that may be in a stem of a question

A

Deep Endosteal scalloping (>2/3)

Pain/progressive pain

Night symptoms/constitutional symptoms

Periosteal reaction

Soft tissue extension

Lysis of prior chondroid mineralization

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103
Q

Ddx for multiple giant cell containing lesions?

A

Multifocal GCT

Browns Tumor

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104
Q

Management of hypercalcemia

A

Fluids (200-300mL/hr initially, then maintaining u/o 100-150mL/hr)

Calcitonin

Bisphosphonates

Can use denosumab if refractory to bisphosphonates

LOOP DIURETICS ARE NOW NOT RECOMMENDED

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105
Q

dDx for Benign Latent Lesion (3)

A

Osteochondroma

NOF

Enchondroma

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106
Q

Treatment for suspected low-grade chondrosarcoma (instead of enchondroma)

A

intralesional curettage

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107
Q

Histopathology of GCT?

A

Multinucleated giant cells.

May see bone.

Mononuclear stromal cells

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108
Q

Where is the most common place for sarcomas to metastasize?

A
  1. Lungs
  2. Another bone
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109
Q

What is Denosumab?

A

RankL inhibitor.

This is a ligand for the Rank receptor and acts as a signal for bone removal.

Denosumab is an analogue and used in GCT tumours to prevent bone turnover.

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110
Q

General treatment approach for bone sarcoma?

A
  1. Neo adjuvant chemotherapy, multi-agent
  2. Re-stage with imaging to assess for interim progression/metastases and pre-op planning
  3. Wide resection
  4. Chemotherapy - use response to pre-op chemo to prognosticate (% necrosis of surgical pathology) and target therapy
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111
Q

Name & decribe the possible life threatening complication of hemangioma

A

Kasabach-Merrit syndrome

entrapped platelets in the hemangioma leading to a possible fatal coaguloapthy

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112
Q

Phases of Paget’s disease

A

Lytic: osteoclastic resorption

Mixed: resorption & compensatory bone formation

Sclerotic: bone formation predominates

All 3 may exist at same time so Paget’s can look lytic, mixed or sclerotic (like OM its a mimicker)

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113
Q

Differential for small round blue cells on histology?

A

Ewing’s sarcoma

Lymphoma

Osteomyelitis

Multiple myeloma

Histiocytosis

Rhabdomyosarcoma

Neuroblastoma

(LERN’M)

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114
Q

What tumor shows predilection for the distribution shown in this figure?

A

Osteoid Osteoma

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115
Q

What happens to NOF as the patient grown and reaches skeletal maturity

A

Migrate to diaphysis as patient grows AND GET LARGER

Will then usually self-resolve (become sclerotic and then go away) as the patient reaches skeletal maturity

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116
Q

Local Recurrence rate for well differentiated vs. high grade?

A

Well differentiated: 10%

High grade: 20%

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117
Q

Benign tumours that can metastasize to lungs (2)

A

GCT

Chondroblastoma

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118
Q

Epiphyseal Lesion with this biopsy.

Diagnosis?

A

Clear cell chondrosarcoma

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119
Q

7yo M severe pain after ice skating.

How do you treat?

A

UBC (fallen leaf sign)

Weight bearing area

Treat with bone grafting and internal fixation

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120
Q

What is the most common soft tissue sarcoma in kids?

A

Rhabdomyosarcoma

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121
Q

On an exam, what tumour has codman’s, what has onionskinning?

A

Codman’s: osteosarc

Onion-skinning; Ewings

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122
Q

2 risk factors for malignant transformation of osteochondroma

A

Cartilage cap >2cm in adults (some say >0.9cm is a risk factor)

Sessile lesions

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123
Q

What primary cancer diagnosis is associated with the shortest life expectancy after pathologic fracture?

A

Lung cancer

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124
Q

Medical managmeent for fibrous dysplasia?

A

bisphosphonate

decreases pain and bone turnover

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125
Q

How do you differentiate between ABC/GCT vs. UBC on x-ray

A

UBC is not as expansile

It generally doesn’t go past the width of the physis

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126
Q

Which chondrosarcoma variant is typically epiphyseal?

A

Clear cell chondrosarcoma

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127
Q

Osteosarcoma: poor prognostic indicators:

A

Advanced stage of disease (most prognostic of survival)

Mets on presentation

Axial location/mets

Skip lesions

Poor response to chemotherapy (>98% necrosis is a GOOD sign)

Increased LDH and/or ALP

Tumour site & size

Expression of P-glycoprotein

Vascular involvement

Margins

Site & size of tumour

Type of sarcoma (parosteal better than intramedullar/conventional)

128
Q

Imaging for metastasis with unknown origin?

A

Chest X-ray

X-ray of involved bone

Any painful extremities

Bone scan

If suspect myeloma, skeletal survey

Consider MRI of bone lesion

Ct chest/abdo/pelvis

129
Q

What is a unique treatment option of neuroblastoma?

A

In young kids, nonoperatiave as they may spontaneously regress

130
Q

4 extraosseous findings in fibrous dysplasia

A

cafe-au-lait spots

precocious puberty

intramuscular myxoma

Chereubism

131
Q

Define the stages and survival for AJCC

IA

IVB

A

IA:

low grade

<8cm

No LN

No mets

5 year survival 98%

IVB:

Any tumour with mets to site other than lungs

5 year survival 30%

132
Q

What tumor shows predilection for the distribution shown in this figure?

A

Ewings Sarcoma

133
Q

What is the primary difference between sarcomas and carcinomas? (In terms of lineage)

A

Carcinomas = epithelial Sarcomas = mesemchymal

134
Q

What is the most important factor for 5 year disease free survival in soft tissue sarcoma?

What happens if this is not achieved?

A

Size of operative margin

larger margin = better b/c it avoids microscopic contamination

If you have + margins, must take back for re-resection of tumour bed

135
Q

What is the most common malignancy of bone?

What is the most common primary malignancy of bone?

A

What is the most common malignancy of bone?

Mets

What is the most common primary malignancy of bone?

myeloma

136
Q

Describe Mirel’s score

137
Q

What mutation is characteristic of fibrous dysphasia?

A

G-Protein mutation

138
Q

Treamtent of leiomyosarcoma?

A

Surgical resection and CHEMO

Although effet is variable, some studies show better outcomes with surgical resection + chemo. No role for radiation except if you can’t get margins

This is one of the exceptions of regular ST sarcoma treamtent of surgery and radiation

Recall: synovial sarcoma may be respnsive to chemo also, but in that one you use RTx

139
Q

Knee pain and this picture. Previous aspiration showed hematoma.

Diagnosis & management?

A

PVNS

clue: affects both sides of the joint and causes OA like destruction (cysts)

Debridement ± radiation

140
Q

What % of plexiform neurifibroma’s transform into neurifibrosarcoma?

141
Q

Post-op radiation after prophylactic surgery for impending pathologic fracture has what 3 goals

A

Decrease pain

slow progression

treat remaining tumour burden not removed at surgery

142
Q

Recurrence for PVNS?

A

30-50% - high

Therfore for treatment, may start with minimally invasive (arthorscopi) but make patient aware you may have to open later on

143
Q

Diagnosis & treatment?

A

Synovial chondromatosis

(calcified stippling affecting both sides of joint)

Treatment:

Arthroscopic debridement (symptomatic treatment)

144
Q

Why do you take out a retroperitoneal lipoma?

A

It has a higher chance of being a liposarcoma

145
Q

Dosing side effects of wound healing post external beam radiation

A

<45 Gray: usually leads to uncompliated tissue healing

45-55 Gray: tissue heals but with problems

>60 Gray: tissue will likely not heal

146
Q

Jaffe-Companacci syndrome

A

Congenital syndrome of:

multiple NOFs

cafe-au-lait pigmentation

mental retardation

heart, eyes, gonads involved

147
Q

What tumour is associated with Dupuytrens, Ledderhose and Garrods?

What other inherited condition is it associated with?

A

Extra-abdominal desmoid tumour

familial adenomtaous polypsis (FAP)

148
Q

Indications for non-excisional bx

A

>5cm

deep to fascia

surrounds NV structure and NOT a lipoma

overlies bone

Hx of “treated” carcinoma

unclear diagnosis

149
Q

What are Birbeck granules diagnostic of?

A

Eosinophilic granuloma

150
Q

You should embolize mets from which lesions?

A

RCC

Thyroid

Hepatocellular carcinoma

151
Q

Local recurrence rate of chordoma?

A

50% (very high)

Proton-photon beams may help decrease this

152
Q

Inheritance pattern of enchonromatosis (Ollier’s/Maffuci)?

A

No inheritance pattern

MHE is AD though

153
Q

What primary bone malignancies are more likely in patients over 40?

A

Plasmocytoma (solitary myeloma)

Primary lymphoma of bone

Chondrosarcoma

MFH

154
Q

Genearlly, what do soft tissue sarcomas look like on MRI?

A

Dark on T1

Bright on T2

155
Q

Management of pathologic fracture through enchondroma?

A

Cast with delayed curettage until after healing

156
Q

dDx for Benign Active lesion (6)

A

Fibrous dysplasia

UBC

EG

Osteoid osteoma

Chondroblastoma

Painful enchondroma

157
Q

GCT location mainly?

A

Prox tibia, distal femur, proximal humerus, distal radius

I.e. Where a lot of growth occurs

158
Q

Poor prognostic indicators for Ewing’s (5)

A

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

159
Q

Diagnosis & Treatment?

A

Schwannoma

Classic “String Sign” (Soap on a rope)

Marginal excision

Try not to damage nerve to preserve function

160
Q

Best cure rate of SCC?

A

Moh’s microsurery

161
Q

t(12;16)(q13;p11.2) (CHOP transcripton factor)

A

Myxoid liposarcoma

162
Q

Treatment for solitary plasmacytoma

A

External beam irradiation alone

± surgical stabilization

163
Q

What is the approach to neoadjuvant chemotherapy for pre-operative malignant bone tumour?

A

Multiagent chemotherapy:

  1. Methotrexate
  2. cisplatin
  3. Doxorubicin
  4. Ifosfamide
164
Q

Most common complication of TKA & THA in Paget’s popuation?

A

TKA: malalignment

THA: excessive bleeding

Therefore, treat medically before elective hip/knee surgery to decrease bleeding risk

165
Q

What is the most common subungla malignancy?

166
Q

GNAS mutation is associate with what condition?

A

Polyostotic fibrous dysplasia

Mcune Albright syndrome

167
Q

What percentage response to chemo is a good prognostic indicator?

168
Q

Where do neurofibrosarcoma’s arise?

A

From a peripheral nerve or from a neurofibroma (malignant transformation)

169
Q

What must you do to gain adequate margins when resecting a chordoma?

A

Sacrifice the nerve roots

170
Q

You suspect soft tissue sarcoma but MRI is indeterminant.

Your core biopsy comes back as hematoma only

Next step?

A

Open biopsy

CANNOT resect this tumour if you suspect soft tissue sarcoma as many of the core’s come back as hematoma

171
Q

Recurrence of chondrosarcoma is directly related to what?

A

Increased telomerase activity

172
Q

In synovial chondromatsis, is the cartilage normal?

A

No - metaplastic

173
Q

Benign bone lesion in spine is usually what?

A

Osteoblastoma

174
Q

Does radiation have a role in treatment of Ewing’s sarcoma?

A

Yes, if wide resection is technically difficult or if a Tumor was inadequatly resected based on pathology

175
Q

Synovial sarcoma is a misnomer. What percentage of these tumours actually arise is a major joint?

176
Q

At what age does metastases become more common than primary bone malignancy?

177
Q

Diagnosis?

A

Chondroblastoma

classic:

chondroid matrix

epiphyseal crossing into metaphysis

lots of surrounding edema

dDx: clear cell chondrosarc

178
Q

How can you differentiate schwannoma from neurofibroma on MRI?

A

Schwannoma is eccentric and separate from nerve.

Neurofibroma is central with the nerve runnign through it.

179
Q

What is the most important factor in preventing local recurrence of a tumour?

A

adequacy of surgical margin

180
Q

Treatmet for symptomatic, large NOFs

A

curettage & bone graft

181
Q

This patient is having leg pain unresponsive to conservative mangaement.

Diagnosis?

Treatment?

A

Paget’s disease

Treatment with metaphyseal osteotomy with plate fixation

(Metaphyseal > diaphyseal, plate > IMN - Parvizi)

182
Q

3 Manifestations of fibrous dysplasia

A

McCune Albright

Mazabraud

Osteofibrous dysplasia

183
Q

Treatment of lymphoma

A

Multiagent chemotherapy ± local radiation

± surgical fixation for instability/pathologic fractures

184
Q

Accuracy of core biopsy done in the office?

185
Q

What is the classification system for GCT?

A

Campanacci:

I: Intramedullary lesion confined to bone

II: Thinned, expanded lesion

III: Cortical breach

186
Q

Name 3 reasons why prophylactic fixation is better than fixing an actual pathologic fracture

A

Shorter OR time

Less morbidity

Quicker recovery

187
Q

You diagnose myxoid liposarcoma. How do you stage?

A

CT chest, abdo, pelvis

(not just CT chest)

Myxoid liposarcoma specifically has the tendency to spread to areas other than lungs

188
Q

2 differentiating factors from myositis ossificans and tumour

A

Calcifies from outside - in (vs. tumour - inside out)

Intramuscular origin

189
Q

What Tumor has characteristic fluid levels on MRI?

190
Q

Most common site for bony mets

Most common site for pathologic fracture secondary to mets to bone

A

Most common site: thoracic spine

Most common site of pathologic fracture due to mets: proximal femur

191
Q

Treatment algorithm for soft tissue sarcoma

A

Radiation + wide excision

Whether you do pre-vs. post op radiation is controversial with pros and cons

192
Q

Risk of metasteses with Liposarcoma by grade?

A
  • Low grade (well differentiated) =
  • High (undifferentiated) = 50%
193
Q

Cell type of synovial sarcoma

A

Usually biphasic with spindle cells (fibrous) & epithelial cells

NOT SYNOVIAL CELLS

194
Q

Lifetime risk of malignant transformation for multiple hereditory exostosis?

195
Q

Name the radio-resistant tumours

A

(TUMOR)

Thyroid

Undifferentiated soft tissue tumour & chondrosarcoma (Except mesenchymal and dedifferentiated??)

Melanoma

Osteosarcoma

RCC

196
Q

What is the most common solid tumour of childhood?

Is it malignant or benign?

A

Neuroblastoma

Malignant

197
Q

Patient with RTC symptoms and solitary enchondroma found on imaging. Plan?

A

Treat mechanical symptoms of RTCT.

No need to treat incidentally found enchondromas as they normally have nothing to do with adjacent joint mechnical symptoms

If enchondroma is truly symptomatic, then intralesional curettage

198
Q

Distal radius lytic lesion DDH? (4)

A

GCT

Abc

Chondrosarcoma

Telientatic osteosarcoma

199
Q

Poor prognostic indicators in osteosarcoma (11)

A

advanced stage of disease (most predictive of survival)

response to chemotherapy (as judged by percent tumor necrosis of resected specimen)

  • >98% is a good prognostic indicator, but doesn’t say what’s bad

tumor site and size

expression of P-glycoprotein

tumor cells can pump chemotherapy out of cell with MDR expression

present in 25% of primary lesions and 50% of metastatic lesions

high serum alkaline phosphatase

high lactic dehydrogenase

vascular involvement

surgical margins

type of chemotherapy regimen

200
Q

Genetic translocation association with osteosarcoma?

A

None

no translocation

However it has associted mutations: p53, Rb

201
Q

dDx for path with giant cells

A

GCT

ABC

telangiectatic osteosarcoma

conventional osteosarcoma

202
Q

Metastatic Tumor that is blastic?

203
Q

A translocation of chromosome 11 and 22 resulting in a chimeric protein is characteristic of what Tumor?

A

Ewing’s sarcoma

Translocation results in EWS gene

204
Q

You are working up a malignant looking tumor. It is bright on T2 with liquid, purulent looking gross pathology and no osteointegration matrix. what is it?

A

Ewing’s sarcoma

205
Q

Synovial sarcoma treatment

A

Same as any other soft tissue sarcoma:

wide resection + radiation

Radiation can be pre or post op (controversial)

UNLIKE other ST sarcomas, chemo may help in synovial sarcoma in both local control and overall survival

206
Q

What colour is chondroid matrix on staining?

207
Q

You’re suspicious of multiple myeloma. How do you stage it?

A

Skeletal survey

NOT bone scan, b/c they will be cold in 30% (b/c it lacks osteoblastic activity)

208
Q

What is the diagnostic criteria for MM?

A

requires one major and one minor (or three minor) criteria for diagnosis

major criteria

biopsy confirmation of plasmacytoma

>10% plasma cells on bone marrow biopsy

serum IgG > 3.5g/dL, IgA > 2g/dL

urine IgA > 1g/24hr or presence of Bence Jones proteins

minor criteria

10-30% plasma cells on bone marrow biopsy

serum or urine protein levels below those listed for major criteria

presence of multiple lytic bone lesions (“punched out” lesions without evidence of surrounding sclerosis)

decreased serum IgG levels

209
Q

Recurrence rate of UBC

A

depends on treatment modality but generally up to 25-30% is reasonable

With steroids, some have reported recurrence up to 88%

(JAAOS 2014)

210
Q

What benign tumour is characterized by fatty infiltration of sub synovial connective tissue?

A

Lipoma aborecens

211
Q

What primaries metastasize to bone?

A

Lymphoma

Melanoma

Visceral carcinomas: Breast Prostate Lung Kidney Thyroid

212
Q

Driving cell behind Paget’s?

A

ostoclasts

213
Q

What primaries are most likely to present as metastasis with unknown origin?

A

Lung and kidney (because they are more likely to be occult)

214
Q

How do you differentiate between ABC and telangiectatic osteosarcoma on MRI?

A

It’s hard - you can’t really

orthobullets says you need bx to truly differentiate

top: TO

Bottom: ABC

215
Q

What part of bone does ewings usually occur?

216
Q

Most common tumour of hand?

A

enchondroma

217
Q

What on histo is pathognomonic for Schwannoma?

A

Verocay bodies

Composed of 2 rows of aligned nuclei in a palisading formation

218
Q

Preferred treatment of EG. Name 5 modalities

Salvage procedures

A

Nonop with treatment of mechanical symptoms preferred

  • Obervation alone
  • bracing (if amenable)
  • Low dose radiation (for spinal lesions that cause neuro symptoms)
  • Chemo (diffuse HSC)
  • Corticosteroid injections
  • Bisphosphonates

Operative if fails:

  • Curettage and bone grafting for lesions endangering articular surface
  • Spinal deformity correction
219
Q

Dose for adjuvant radiation in soft-tissue sarcoma?

What are complications of 45 Gy vs 60Gy?

A

45-65Gy

45Gy: woudn has delayed healing

60Gy: wound not expected to heal

220
Q

CD99

A

Ewing’s sarcoma

221
Q

8 lesiosn that can be found in vertebral body

A

multiple myeloma (most common primary tumor of spine)

  • chordoma
  • osteosarcoma
  • hemangioma
  • giant cell tumor of bone: sacrum;
  • eosinophilic granuloma
  • osteosarcoma of the spine
  • osteoid-osteoma and osteoblastoma of the spine
222
Q

dDx for Benign aggressive lesion

A

Osteoblastoma

GCT

ABC

CMF

Chondroblastoma

±UBC

223
Q

Long term survival of parosteal osteosarcoma when local contrl has been achieved

224
Q

What subtype of lipoma is painful?

A

AngioLipoma

225
Q

Poor prognostic indicators in Ewing’s

A

male age >14

fever

anemia

high LDH

axial location

use of radiotherapy without surgery

poor histologic response to chemo

Transcription Type II (Survival benefit with transcription type I EWS/FLI1 mutation)

Poor response to chemo

No effect of pathologic fracture

Local recurrence not as poor prognostic factor as in osteosarcoma (“death sentence” if local recurrence in osteosarcoma)

226
Q

Most common site of mets of osteosarcoma

A

Lung: most common

Another bone: second most common

227
Q

Tumours that mets to bone

A

breast

lung

thyroid

renal

prostate

*all the paired organs

228
Q

What are the three treatment facets of a MM bone lesion?

A

With a confirmed diagnosis, the treatment of multiple myeloma involves radiotherapy, bisphosphonates +/- surgical stabilization.

Surgical stabilization is utilized when there is a complete or impending fracture.

229
Q

CD31 & polyvinyl chloride exposure is a risk for what?

A

ANgiosarcoma

rare

230
Q

What is the local recurrence rate with ABC?

Name 1 adjunct that may help decrease local recurrence?

A

25% local recurrence

High speed burr

Phenol

Less common in older kids

231
Q

Bloodwork for metastasis with unknown origin?

A

Spep/upep

CBC

Esr

Lytes

Liver enzymes

Psa

Alp

LDH

232
Q

Survival of Chordoma?

A

60% 5 year survival

25% long term survival

local extension may be fatal

233
Q

What kind of biopsy is contraindicated in chordoma?

A

transrectal

234
Q

Maffuccis is characterized by:

A) multiple enchondromas, hemangiomas, lymphangiomas

B) multiple enchondromas in a unilateral distribution

C) auto-dominant transmission, multiple enchondromas and osteochondromas

235
Q

What does it mean if a Tumor is biphasic?

A

It contains both epithelial and mesemchymal neoplastic cells

236
Q

For ewings sarcoma, what blood markers should be added to the typical malignant work up and why?

A

CBC, esr and crp to differentiate it from osteomyelitis

In ewings esr will be up (but not crp?)

237
Q

Indications for marginal resection of lipoma (4)

A

Symptomatic lesions

Rapidly growing mass

Deep to fascia or in retroperitoneum

Spindle cell & pleomorphic variants

238
Q

What term is typically used to describe the following genes: p53 and RB-1

A

Tumor suppressor genes.

239
Q

In terms of tumour location, what are apophyses treated like?

A

Epiphysis

THINK: locations for chondroblastoma (GT apophysis, calcaneal apophysis etc…)

240
Q

3 dDx for anterior tibial liesion

A

osteofibrous dysplasia

adamantinoma

malignant fibrous histiocytoma

241
Q

What tumor am I?

Estrogen Beta Positive

Locally invasive but benign

Can treat with Tamoxifen

Associated with FAP and Dupuytrens

A

Extra-Abdominal Desmoid Tumor

242
Q

Lifetime risk of malignant transformation for solitary enchondroma?

243
Q

What is special about biphasic sarcomas in terms of metasteses?

A

They can go to lymph nodes, which is unusual for sarcomas.

Also they metastasize to locations other than the lungs.

244
Q

Name 2 types of benign periepheral nerve sheath tumours

What’s the difference

A

Scwannoma: only made up of Schwan cells

Neurofibroma: Arises from Schwann cells but also has other cells (fibroblasts etc)

245
Q

PVNS typically contains which pigment?

A

Hemosiderin deposits

246
Q

Gene associated with primary ABC?

247
Q

3 Predictors of UNSUCCESSFUL treatment of UBC with steroids

A

active lesion

large size

multiloculated

248
Q

Long standing chronic draining wounds or burn scar are at risk of what?

A

Transformation into SCC (Marjolin’s ulcer)

249
Q

What Tumor is usually epiphyseal in skeletally immature with chicken wire calcifications?

A

Chondroblastoma

Also has mononuclear chondroblasts

250
Q

Name 3 soft tissue tumours that chemo can help with:

A

Synovial sarcoma

Leiomyosarcoma

Rhabdomyosarcoma (only in paediatric population, not adults)

251
Q

Common presentations in Chordoma?

A

bladder & bowel changes are common!

palpable on rectal exam

252
Q

What staging investigation must you include in epitheliod sarcoma (and certain other soft tissue sacomas)

A

Sentinel LN biopsy

253
Q

Li-Fraumeni syndrome:

what germ line mutation & tumour is it associated with

A

p53

osteosarcoma

also: breast, colon Ca

254
Q

Name 4 vertebral body tumours

A

hemangioma

GCT

chordoma

multiple myeloma

Lymphoma

255
Q

What is the most common benign radiation induced tumour of bone?

A

osteochondroma

256
Q

Indications for bone marrow biopsy?

(i.e. what tumors necessitate this)

A
  1. Ewings
  2. Multiple Myeloma
  3. Rhabdomyosarcoma
257
Q

Patient is complaining of on and off pain, tenderness to palpation and cold intoleratnce. This is the clinical picture. Diagnosis & Management?

A

Glomus tumour

Classic triad

  • paroxysmal pain
  • exquisite tenderness to palpation
  • Cold intolerance

Treatment: marginal resection is curative

258
Q

Tretment of fibrous dysplasia?

A

Observation ± bisphosphonates: asymptomatic lesions

Operative with internal fixation and autologous bone graft (symptomatic)

259
Q

What is unique in Ewing’s as part of staging?

A

Bone marrow biopsy

looks for BM mets that would change prognosis

260
Q

Which primary has worst prognosis once mets are present in bone?

261
Q

How do you differentiate between ABC and telangiectatic osteosarcoma?

A

Biopsy

Telengiectatic osteosarcoma is Slayers Sarcoma because it is Lakes of Blood

262
Q

A 75-year-old man presents with a displaced femoral neck fracture. During your surgical exposure for a hemiarthroplasty, the femoral neck has fractured through a pathologic lesion which is diagnosed as a lymphoma on frozen section. The lesion is located in the center of the femoral neck and the calcar femorale is not involved. Your treatment should include

A

Hemiarthroplasty & postoperative staging and chemo-radiotherapy as needed

Lymphoma can be treated with chemo-rads for local and distant disease

If it was osteosarc: close and regular sarcoma protocol

263
Q

SYT-SSX1, SYT-SSX2, or SYT-SSX4 translocation (t(X;18)(p11;q11))

A

Synovial sarcoma

264
Q

Classic spine finding in EG?

A

vertebra plana

Couldn’t find a good picture but can be subtle - remember the SPORC case??

265
Q

What is the risk of malignante transformation of fibrous dysplasia?

A

1%

into osteosarcoma, fibrosarcoma or MFH

266
Q

What is the usual indication for radiation therapy at the following doses?

a) 6 Gy
b) 30 Gy
c) 60 Gy

A

a) HO prophylaxis
b) Treatment of a boney Met
c) Adjuvant therapy for soft tissue sarcoma

267
Q

Poor prognostic sign in neuroblastoma?

268
Q

Name 2 tumours affecting multiple vertebra

A

metastatic disease

multiple myeloma (remember may be cold on bone scan)

269
Q

Benign aggressive looking lesion. Must rule out what malignancy?

A

Telangiectatic osteosarcoma

270
Q

Enchondromas always central metaphyseal t/f?

271
Q

Which sarcoma is radio/chemo resistant?

A) liposarcoma

B) osteosarcoma

C) chondrosarcoma

D) Ewing’s sarcoma

A

C) chondrosarcoma

272
Q

What are the three biphasic sarcomas?

A
  1. Synovial sarcoma
  2. Angiosarcoma
  3. Mixed liposarcoma
273
Q

UBC on MRI?

A

very Dark on T1

Very bright on T2

274
Q

What percentage of UBCs heal after fracture?

A

10-15%

(JAAOS 2014 says

275
Q

Which soft tissue tumour is relatively insensitive to both radiation and chemo?

A

Angiosarcoma

276
Q

Man has radicular buttock pain

MRI shows large nerve lesion in sciatic

What is it likely?

A

Neurofibrosarcoma (malignant peripheral nerve sheath tumour)

They affect large nerves

Must resect entire affected nerve

277
Q

What type of nerve cells is responsible for the neurofibromas in NF1?

A

non-myelinating Schwann cells

(vs. myelinating in solitary neurofibromas)

278
Q

What type of resection are you performing with PVNS

A

Marginal resection is what you’re aiming for

(with arthroscopic, probably doing intralesional)

279
Q

Host lamellar bone entrapment is characteristic of what malignancy?

A

Chondrosarcoma

280
Q

Best UBC lesions to pursue steroid injections

A

Predictors of success following UBC treated with steroids

approaching skeletal maturity

Not loculated

smaller size

Fracture of inner wall

(radiograhpically active - Can’t find a reference - probabyl a POOR sign though. This contrasts “approaching skeletal maturity”)

281
Q

Most common presentation of chondroBLASTOMA

282
Q

Treatment for multiple myeloma

A

multiagent chemotherapy (mainstay)

bisphosphonates (helps reduce number of skeletal events)

Surgical stabilization

283
Q

Orthopaedic Tumours that Spread to LN’s

A

Synovial Sarcoma

epitheliod sarcoma

angiosarcoma

rhabdomyosarcoma

clear cell sarcoma

284
Q

Diagnosis & Treatment?

A

Adamantinoma

(NOT OFD b/c not confined to cortex - much more malignant. ALso associated with bowing)

Treatment: wide resection + reconstruction (intercalary graft)

285
Q

Histologically, how do you differentiate ABC from telangiectatic osteosarcoma?

A

TO: lakes of blood filled mixed with neoplastic cells (top)

ABC: Cavernous blood filled spaces with no endothelial lining (bottom)

286
Q

Describe Mazabraud syndrome

A

Polyostotic fibrous dysplasia

intramuscular myxoma

287
Q

What is a Bence Jones Protein?

A

Light chain immonuglobulin found in multiple myeloma.

288
Q

What kind of chondrosarc does enchondrama change into?

A

low grade chondrosarcoma

289
Q

Lifetime risk of malignant transformation for Maffucis?

290
Q

Name the 3 manifestations of eosinophilic granuloma and their characteristics

A

Eosinophilic granuloma

  • Single self-limited lesion in younger patients

Hand-Schuller-Christian disease (HSC)

  • chronic, disseminated form with bone and visceral lesions

Letterer-Siwe Disease

  • Fatal form that occurs in young kids

Can be Monoostotic or Polyostotic

291
Q

What tumor shows predilection for the distribution shown in this figure?

292
Q

Recurrence rate of GCT of tendon sheath after marginal excision?

293
Q

Treatment for neurofibrosarcoma (malignant peripheral nerve sheath tumour)?

What must you specifically do?

A

Wide resection

Must resect the entire affected nerve

294
Q

Name the syndromes associted with the spectrum of Langerhans cell histiocytosis

A

Eosinophilic Granuloma

  • Self-limited in younger patients

Hand-Schuller-Christian Disease

  • Chronic, disseminated form

Lettere-Siwe Disease

  • Fatal for in young kids
295
Q

Do osteochondroma transform into high or low grade chondrosarcoma?

296
Q

Which primary can have mets distal to knee and elbow?

297
Q

Name 1 poor prognostic factor for parosteal osteosarcoma

A

de-differentiation

298
Q

Indicatios for surgical resection in osteoid osteoma

A

painful scoliosis

Too close to vital strucures for radiofrequency ablation (neural elements, skin)

299
Q

NOF can be associated with what other tumour

300
Q

In operative management of fibrous dysplasia, what should you never use

A

Autograft

It will rapidly be turned into fibrous dysplastic woven bone

301
Q

Benign aggressive lesions? (3)

A

GCT, ABC, osteoblastoma

302
Q

Seven questions for evaluating bone lesion on x-ray?

(as per Toronto group)

A
  1. where is it?
  2. how big is it?
  3. What is it doing to bone? (destructive? geographic? permeative?etc.)
  4. what is bone doing to it? (sclerosis? periosteal rxn?)
  5. matrix?
  6. cortex? (eroded? preserved? neocortex? endosteum?)
  7. soft tissue mass?
303
Q

Which soft tissue sarcomas need chemo?

A

RSSD

Rhabdomyosarcoma

Synovial sarcoma

Soft-tissue Ewings

de-differentiated chondrosarc

HOWEVER THIS IS CONTROVERSIAL

SAFE ANSWER FOR TREATMENT OF SOFT TISSUE SARCOMA IS WIDE RESECTIO + RADIATION

304
Q

Treatment of this pathologic fracture?

A

Sling for comfort

Nonop of pathologic fractures through UBC, unless they are in a weight bearing zone (proximal femur)

305
Q

Which way do pedunculated osteochondraom lesions point?

A

Away from the joint

306
Q

3 poor prognostic indicators for soft tissue sarcoma?

A

High grade

size >5cm

tumour location below deep fascia

307
Q

how often is ABC associated with another tumour?

A

30% of the time

308
Q

Synovial sarcoma genetics

A

t(X;18)

Forms SYT/SSX1 & SYT/SSX2 fusion transcripts

309
Q

Medical treatment options in UBC

A

Bone graft (poor success with high recurrence rates)

Steroid

autologous bone marrow (controversial - may be better than steroids)

JAAOS 2014

310
Q

Why must you follow chordoma patients long term post resection?

A
  1. High recurrence rate
  2. Mets occurs late in disease

Occurs in 30-50% to lung, rarely bone

311
Q

Nonoperative mangement of Paget’s

When is it indicated

A

Bisphosphonates

Calcitonin

Indicated in sympomatic cases

asymptomatic you just treat supportively

312
Q

What tumor shows predilection for the distribution shown in this figure?

A

Chondroblastoma

313
Q

What extra-articular tumor has pathology similar to PVNS?

A

Giant cell tumor of tendon

314
Q

What are the basic types of soft tissue sarcoma (think histo)

A

Synovial (sarcoma)

Lipo(sarcoma)

Rhabdomyo(sarcoma)

Fibro(sarcoma)

315
Q

GCT histology?

A

Mononuclear cells with Uniformly distributed multinucleated giant cells

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