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
What is the main dDx for intramuscular myxoma that you must rule out?
myxoid liposarcoma
26
Name some negative prognostic characteristics of osteosarcoma.
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)
27
Chrondroblastomas are located where?
Epiphysis
28
Lifetime risk of malignant transformation for olliers?
30%
29
Describe the Enneking system for classifying malignant tumours.
Three factors: Grade, site and metasteses Grade is high or low Site is intracompartmental (enclosed by natural barriers) or extracompartmental
30
Parosteal osteosarcoma with invasion into medullary cavity - what is the affect on prognosis?
No effect - still good prognosis
31
Name 2 scoring systems that may help you decide whether to operate on a pathologic spine tumour
_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
32
Presentation of glomus Tumour
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
33
Name the 5 subtypes of liposarcoma.
Well differentiated liposarcoma Myxoid liposarcoma Pleomorphic liposarcoma Dedifferentiated liposarcoma Mixed-type liposarcoma
34
What is the most common sarcoma in the foot?
Synovial sarcoma
35
Soft tissue tumours that mets via LN & to places other than lungs
SCREAM Synovial sarcoma Clear cell sarcoma Rhabdomyosarcoma Epitheliod sarcoma Angiosarcoma Myxoid liposarcoma
36
Name 2 sarcomas for which excisional bx can be performed
Parosteal osteosarcoma low grade chondrosarcoma \*not sure I would say this on an exam It's from AAOS CORE2
37
What is a defining feature on histo for extra-articular desmoid tumour?
100% positive for estrogen receptor-beta
38
4 Options for treating PVNS (think techniques)
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%
39
2 negative prognostic indicators of CMF
children lobulated with abundant myxoid material
40
Football player suffered injury after helmet to groin hit. Residual non-tender mass. Diagnosis? Treatment?
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
41
Olliers is characterized by: A) multiple enchondromas, hemangiomas, lymphangiomas B) multiple enchondromas in a unilateral distribution C) auto-dominant transmission, multiple enchondromas and osteochondromas
B
42
What findings differentiate chondrosarcoma from a benign lesion?
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
43
5 tumors that can have an associated ABC?
1. giant cell tumor 2. chondroblastoma 3. fibrous dysplasia 4. chondromyxoid fibroma 5. NOF
44
Fallen leaf sign what is it what is it pathognomonic for?
Cortical fragment in the bottom of an empty cyst UBC
45
4 associated conditions of NOF
Jaffe-Campanacci syndrome Neurofibromatosis Familial multifocal NOF ABC
46
What is the treamtnet of low-grade intramedullary osteosarcoma?
Surgial resection only This is the exception of the usual rule of chemo-sx-chemo for sarcoma
47
Components of local staging?
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
48
Physical exam for metastasis with unknown origin must include?
Thyroid exam Lung exam Abdomen exam Breast/prostate exam
49
Describe the bones in enchondromatosis?
Dysplastic They are not normal and can have deformities
50
Young, thirsty kid with these findings. Diagnosis?
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)
51
What malignant tumour can secondarily arise from a bone infarct, Paget's disease or prior radiation?
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_
52
What do you do following unplanned excision of soft tissue sarcoma?
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)
53
Tumour association with poly-vinyl chloride
Angiosarcoma very aggressive vascular tumour
54
Overall prognosis for multiple myeloma? What confers the worst prognosis?
Poor Median survival 3 years from diagnosis 5 year survival: 30% 10 years survival: 10% Shortest survival in patients with renal failure
55
Name 3 syndromes assocaited with Fibrous Dysplasia
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] 2. Mazabraud - [polyostotic fibrous dysplasia soft-tissue intramuscular myxomas] 3. 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
56
What sarcoma has flat epithelial cells on pathology?
Synovial sarcoma
57
What is radiation dose for HO prophylaxis?
6Gy | (vs. 45-60 for soft tissue sarcoma)
58
Genetics for myxoid liposarcoma
t(12;16) Creates CHOP-TLS fusion protein
59
What physeal zone does osteochondroma/MHE stem from?
Proliferative
60
What Tumor stains for MIC2 antigen?
Ewing's sarcoma. This is how you differentiate it from other small round blue cell tumors.
61
What is the most common malignant bone tumour of the hand?
Chondrosarcoma THINK: common to have enchondromas in hand, so one of them must transform
62
3 non-neoplastic things to consider adding on to the end of a ddx for bone lesion?
EG Infection Hyperparathyroidism (if older)
63
What is the recurrence rate of osteoid osteoma treated with percutaneous radiogrequency ablation?
10-15%
64
Describe Harington's Criteria
\>50% destruction of diaphyseal cortices \>50-75% destruction of metaphysis (\>2.5cm) Permeative destruction of the subtrochanteric fermoal region persistent pain following irradiation
65
Treatment of osteofibrous dysplasia?
nonoperative - observation bracing if deformity interfering with walking OR if you need correction of deformity (osteotomy)
66
Cause of Paget's
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
67
What kind of matrix does ewings usually have?
None
68
Poor prognostic indicators for EG
involvement at young age rapid disease progression organ involvement (eg pituitary, lung, hematopoietic, or liver involvement); \*organ dysfunction carries an especially poor prognosis
69
Older than 40 , blastic lesion DDx (4)?
Metastases (prostate) Bone island Bone infarct Osteoblastoma Infection
70
Name two enchondroma syndromes.
1. Olliers 2. Maffuccis
71
Classic findings of Hans-Christian-Schuller Disease
Disseminated form of EG Triad: Skull lesions diabetes insipidus (thirst) Exopthalmos (double vision)
72
Osteolysis in tumour is caused by the action of what cytokine on what cell?
RANKL on osteoclastic cells (not on tumour cells)
73
Genetically, how does osteofibrous dysplasia differ than regular fibrous dysplasia?
It doesn't have the Gs alpha activating mutation
74
Name the basic biopsy principles.
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
75
Compare pre vs. post operative radiation for soft tissue sarcoma:
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
76
6 lesions on the posterior elements of spine
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
77
4 posterior element tumours
ABC osteoid osteoma osteoblastoma osteochondroma
78
What systemic pathology (ie non ortho) do people with Paget's disease get?
high output cardiac failure due to increased turnover of bone
79
What percentage of vertebral body tumours are malignanet?
75%
80
What bone metasteses are not redio sensitive? Which is the most readio-sensitive?
1) GI and Renal 2) Prostate
81
Classic spine finding in eosinophilic granuloma Name 1 other classic finding
Vertebra plana aka platyspondia Multiple punched out/lytic cranial lesions (ask for skull x-ray)
82
Which side of the spine in an osteoid osteoma normally found in?
Concavity of the spine
83
Histology of ewings sarcoma?
Small round blue cells
84
How do you classify UBC?
Active - in continuity with physis Latent - bony bridge between UBC and physis
85
Most common location of mets in soft tissue sarcoma
Lungs They *can* mets to LNs, but lungs are still most common
86
Survival rate of chondrosarcoma
Directly correlates with histological grade: Grade 1: 90% Grade 2: 60-70% Grade 3: 30-50% **De-differentiated: 10%**
87
What are the main steps in treatment of malignant bone tumour?
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
88
Treatment?
Chordoma Wide resection is standard of care ± radiation if: positive margins inoperable tumour
89
What is the most common cause of painful scoliosis in the adolescent population?
osteoid osteoma
90
5 complications with treatment of UBC
recurrence fracture embolizatio of injected material local reaction to injected material growth disturbances
91
What kind of tumour results from malignant transformation of a chronic OM? what is the treatment?
SCC Treat with wide excision/amputation + adjuvant chemo/rads Prognosis is poor with late diagnosis, good with early
92
Patient comes in post-MVC with a pathologic fracture through this. Dx as ABC. What is your plan?
Non-operative fracture management Once healed, then proceed with intralesional curettage Remember to treat path fractures non-op!
93
4 predisposing genetic factors for intramedullary osteosarcoma
Rb + p53 Rothmund thomson syndrome LiFraumeni syndrome
94
60 yo F with constitutional symptoms, pain palpable mass. (see picture) Dx & treatment
Lymphoma Treatment is surgical stabilization, chemo, radiation \*Lymphoma can have a soft tissue mass! \*lymphoma: small round blue cell
95
What do the terms onion skinning and sunburst refer to on radiographs? Which Tumors are each characteristic of?
Types of periosteal reaction Onion skinning = ewings Sunburst = osteosarcoma
96
Age range for GCT?
20-40
97
indications for excisional bx
superficial to fascia Not overlying NV structure excise AND LEAVE BEHIND FASCIA
98
"Pop corn densities" are useful in differentiating what secondary malignancy from its benign precursor?
Secondary chondrosarcoma from enchondroma.
99
What is the histo of periosteal osteosarcoma
osteoid + chondroblastic matrix If no osteoid, will be classified as chondrosarcoma
100
What tumor shows predilection for the distribution shown in this figure?
Giant Cell Tumor
101
Desribe AJCC classification system
Soft soft tissue tumours: See picture
102
Name 5 signs/symptoms that are concerning for chondrosarcoma, you know, that may be in a stem of a question
Deep Endosteal scalloping (\>2/3) Pain/progressive pain Night symptoms/constitutional symptoms Periosteal reaction Soft tissue extension Lysis of prior chondroid mineralization
103
Ddx for multiple giant cell containing lesions?
Multifocal GCT Browns Tumor
104
Management of hypercalcemia
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**
105
dDx for Benign Latent Lesion (3)
Osteochondroma NOF Enchondroma
106
Treatment for suspected low-grade chondrosarcoma (instead of enchondroma)
intralesional curettage
107
Histopathology of GCT?
Multinucleated giant cells. May see bone. Mononuclear stromal cells
108
Where is the most common place for sarcomas to metastasize?
1. Lungs 2. Another bone
109
What is Denosumab?
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.
110
General treatment approach for bone sarcoma?
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
111
Name & decribe the possible life threatening complication of hemangioma
Kasabach-Merrit syndrome entrapped platelets in the hemangioma leading to a possible fatal coaguloapthy
112
Phases of Paget's disease
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)
113
Differential for small round blue cells on histology?
Ewing's sarcoma Lymphoma Osteomyelitis Multiple myeloma Histiocytosis Rhabdomyosarcoma Neuroblastoma (LERN'M)
114
What tumor shows predilection for the distribution shown in this figure?
Osteoid Osteoma
115
What happens to NOF as the patient grown and reaches skeletal maturity
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
116
Local Recurrence rate for well differentiated vs. high grade?
Well differentiated: 10% High grade: 20%
117
Benign tumours that can metastasize to lungs (2)
GCT Chondroblastoma
118
Epiphyseal Lesion with this biopsy. Diagnosis?
Clear cell chondrosarcoma
119
7yo M severe pain after ice skating. How do you treat?
UBC (fallen leaf sign) Weight bearing area Treat with bone grafting and internal fixation
120
What is the most common soft tissue sarcoma in kids?
Rhabdomyosarcoma
121
On an exam, what tumour has codman's, what has onionskinning?
Codman's: osteosarc Onion-skinning; Ewings
122
2 risk factors for malignant transformation of osteochondroma
Cartilage cap \>2cm in adults (some say \>0.9cm is a risk factor) Sessile lesions
123
What primary cancer diagnosis is associated with the shortest life expectancy after pathologic fracture?
Lung cancer
124
Medical managmeent for fibrous dysplasia?
bisphosphonate decreases pain and bone turnover
125
How do you differentiate between ABC/GCT vs. UBC on x-ray
UBC is not as expansile It generally doesn't go past the width of the physis
126
Which chondrosarcoma variant is typically epiphyseal?
Clear cell chondrosarcoma
127
Osteosarcoma: poor prognostic indicators:
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
Imaging for metastasis with unknown origin?
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
What is a unique treatment option of neuroblastoma?
In young kids, nonoperatiave as they may spontaneously regress
130
4 *extraosseous* findings in fibrous dysplasia
cafe-au-lait spots precocious puberty intramuscular myxoma Chereubism
131
# Define the stages and survival for AJCC IA IVB
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
What tumor shows predilection for the distribution shown in this figure?
Ewings Sarcoma
133
What is the primary difference between sarcomas and carcinomas? (In terms of lineage)
Carcinomas = epithelial Sarcomas = mesemchymal
134
What is the most important factor for 5 year disease free survival in soft tissue sarcoma? What happens if this is not achieved?
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
What is the most common malignancy of bone? What is the most common primary malignancy of bone?
What is the most common malignancy of bone? Mets What is the most common primary malignancy of bone? myeloma
136
Describe Mirel's score
137
What mutation is characteristic of fibrous dysphasia?
G-Protein mutation
138
Treamtent of leiomyosarcoma?
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
Knee pain and this picture. Previous aspiration showed hematoma. Diagnosis & management?
PVNS clue: affects both sides of the joint and causes OA like destruction (cysts) Debridement ± radiation
140
What % of plexiform neurifibroma's transform into neurifibrosarcoma?
10%
141
Post-op radiation after prophylactic surgery for impending pathologic fracture has what 3 goals
Decrease pain slow progression treat remaining tumour burden not removed at surgery
142
Recurrence for PVNS?
30-50% - high Therfore for treatment, may start with minimally invasive (arthorscopi) but make patient aware you may have to open later on
143
Diagnosis & treatment?
Synovial chondromatosis (calcified stippling affecting *both sides of joint*) Treatment: Arthroscopic debridement (symptomatic treatment)
144
Why do you take out a retroperitoneal lipoma?
It has a higher chance of being a liposarcoma
145
Dosing side effects of wound healing post external beam radiation
\<45 Gray: usually leads to uncompliated tissue healing 45-55 Gray: tissue heals but with problems \>60 Gray: tissue will likely not heal
146
Jaffe-Companacci syndrome
Congenital syndrome of: multiple NOFs cafe-au-lait pigmentation mental retardation heart, eyes, gonads involved
147
What tumour is associated with Dupuytrens, Ledderhose and Garrods? What other inherited condition is it associated with?
Extra-abdominal desmoid tumour familial adenomtaous polypsis (FAP)
148
Indications for non-excisional bx
\>5cm deep to fascia surrounds NV structure and NOT a lipoma overlies bone Hx of "treated" carcinoma unclear diagnosis
149
What are Birbeck granules diagnostic of?
Eosinophilic granuloma
150
You should embolize mets from which lesions?
RCC Thyroid Hepatocellular carcinoma
151
Local recurrence rate of chordoma?
50% (very high) Proton-photon beams may help decrease this
152
Inheritance pattern of enchonromatosis (Ollier's/Maffuci)?
No inheritance pattern MHE is AD though
153
What primary bone malignancies are more likely in patients over 40?
Plasmocytoma (solitary myeloma) Primary lymphoma of bone Chondrosarcoma MFH
154
Genearlly, what do soft tissue sarcomas look like on MRI?
Dark on T1 Bright on T2
155
Management of pathologic fracture through enchondroma?
Cast with delayed curettage until after healing
156
dDx for Benign Active lesion (6)
Fibrous dysplasia UBC EG Osteoid osteoma Chondroblastoma Painful enchondroma
157
GCT location mainly?
Prox tibia, distal femur, proximal humerus, distal radius I.e. Where a lot of growth occurs
158
Poor prognostic indicators for Ewing's (5)
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
Diagnosis & Treatment?
Schwannoma Classic "String Sign" (Soap on a rope) Marginal excision Try not to damage nerve to preserve function
160
Best cure rate of SCC?
Moh's microsurery
161
t(12;16)(q13;p11.2) (CHOP transcripton factor)
Myxoid liposarcoma
162
Treatment for solitary plasmacytoma
External beam irradiation alone ± surgical stabilization
163
What is the approach to neoadjuvant chemotherapy for pre-operative malignant bone tumour?
Multiagent chemotherapy: 1. Methotrexate 2. cisplatin 3. Doxorubicin 4. Ifosfamide
164
Most common complication of TKA & THA in Paget's popuation?
TKA: malalignment THA: excessive bleeding Therefore, treat medically before elective hip/knee surgery to decrease bleeding risk
165
What is the most common subungla malignancy?
SCC
166
GNAS mutation is associate with what condition?
Polyostotic fibrous dysplasia Mcune Albright syndrome
167
What percentage response to chemo is a good prognostic indicator?
\>98%
168
Where do neurofibrosarcoma's arise?
From a peripheral nerve or from a neurofibroma (malignant transformation)
169
What must you do to gain adequate margins when resecting a chordoma?
Sacrifice the nerve roots
170
You suspect soft tissue sarcoma but MRI is indeterminant. Your core biopsy comes back as hematoma only Next step?
Open biopsy CANNOT resect this tumour if you suspect soft tissue sarcoma as many of the core's come back as hematoma
171
Recurrence of chondrosarcoma is directly related to what?
Increased telomerase activity
172
In synovial chondromatsis, is the cartilage normal?
No - metaplastic
173
Benign bone lesion in spine is usually what?
Osteoblastoma
174
Does radiation have a role in treatment of Ewing's sarcoma?
Yes, if wide resection is technically difficult or if a Tumor was inadequatly resected based on pathology
175
Synovial sarcoma is a misnomer. What percentage of these tumours actually arise is a major joint?
10%
176
At what age does metastases become more common than primary bone malignancy?
40
177
Diagnosis?
Chondroblastoma classic: chondroid matrix epiphyseal crossing into metaphysis lots of surrounding edema dDx: clear cell chondrosarc
178
How can you differentiate schwannoma from neurofibroma on MRI?
Schwannoma is eccentric and separate from nerve. Neurofibroma is central with the nerve runnign through it.
179
What is the most important factor in preventing local recurrence of a tumour?
adequacy of surgical margin
180
Treatmet for symptomatic, large NOFs
curettage & bone graft
181
This patient is having leg pain unresponsive to conservative mangaement. Diagnosis? Treatment?
Paget's disease Treatment with metaphyseal osteotomy with plate fixation (Metaphyseal \> diaphyseal, plate \> IMN - Parvizi)
182
3 Manifestations of fibrous dysplasia
McCune Albright Mazabraud Osteofibrous dysplasia
183
Treatment of lymphoma
Multiagent chemotherapy ± local radiation ± surgical fixation for instability/pathologic fractures
184
Accuracy of core biopsy done in the office?
80%
185
What is the classification system for GCT?
Campanacci: I: Intramedullary lesion confined to bone II: Thinned, expanded lesion III: Cortical breach
186
Name 3 reasons why prophylactic fixation is better than fixing an actual pathologic fracture
Shorter OR time Less morbidity Quicker recovery
187
You diagnose myxoid liposarcoma. How do you stage?
CT chest, abdo, pelvis (not just CT chest) Myxoid liposarcoma specifically has the tendency to spread to areas other than lungs
188
2 differentiating factors from myositis ossificans and tumour
Calcifies from outside - in (vs. tumour - inside out) Intramuscular origin
189
What Tumor has characteristic fluid levels on MRI?
ABC
190
Most common site for bony mets Most common site for pathologic fracture secondary to mets to bone
Most common site: thoracic spine Most common site of pathologic fracture due to mets: proximal femur
191
Treatment algorithm for soft tissue sarcoma
Radiation + wide excision Whether you do pre-vs. post op radiation is controversial with pros and cons
192
Risk of metasteses with Liposarcoma by grade?
* Low grade (well differentiated) = * High (undifferentiated) = 50%
193
Cell type of synovial sarcoma
Usually biphasic with spindle cells (fibrous) & epithelial cells NOT SYNOVIAL CELLS
194
## Footnote Lifetime risk of malignant transformation for multiple hereditory exostosis?
10%
195
Name the radio-resistant tumours
(TUMOR) **T**hyroid **U**ndifferentiated soft tissue tumour & chondrosarcoma (Except mesenchymal and dedifferentiated??) **M**elanoma **O**steosarcoma **R**CC
196
What is the most common solid tumour of childhood? Is it malignant or benign?
Neuroblastoma Malignant
197
Patient with RTC symptoms and solitary enchondroma found on imaging. Plan?
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
Distal radius lytic lesion DDH? (4)
GCT Abc Chondrosarcoma Telientatic osteosarcoma
199
Poor prognostic indicators in osteosarcoma (11)
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
Genetic translocation association with osteosarcoma?
None no ***translocation*** However it has associted mutations: p53, Rb
201
dDx for path with giant cells
GCT ABC telangiectatic osteosarcoma conventional osteosarcoma
202
Metastatic Tumor that is blastic?
Prostate
203
A translocation of chromosome 11 and 22 resulting in a chimeric protein is characteristic of what Tumor?
Ewing's sarcoma Translocation results in EWS gene
204
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?
Ewing's sarcoma
205
Synovial sarcoma treatment
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
What colour is chondroid matrix on staining?
Blue
207
You're suspicious of multiple myeloma. How do you stage it?
Skeletal survey NOT bone scan, b/c they will be cold in 30% (b/c it lacks osteoblastic activity)
208
What is the diagnostic criteria for MM?
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
Recurrence rate of UBC
depends on treatment modality but generally up to 25-30% is reasonable With steroids, some have reported recurrence up to 88% (JAAOS 2014)
210
What benign tumour is characterized by fatty infiltration of sub synovial connective tissue?
Lipoma aborecens
211
What primaries metastasize to bone?
Lymphoma Melanoma Visceral carcinomas: Breast Prostate Lung Kidney Thyroid
212
Driving cell behind Paget's?
ostoclasts
213
What primaries are most likely to present as metastasis with unknown origin?
Lung and kidney (because they are more likely to be occult)
214
How do you differentiate between ABC and telangiectatic osteosarcoma on MRI?
It's hard - you can't really orthobullets says you need bx to truly differentiate top: TO Bottom: ABC
215
What part of bone does ewings usually occur?
Diaphysis
216
Most common tumour of hand?
enchondroma
217
What on histo is pathognomonic for Schwannoma?
Verocay bodies Composed of 2 rows of aligned nuclei in a palisading formation
218
Preferred treatment of EG. Name 5 modalities Salvage procedures
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
Dose for adjuvant radiation in soft-tissue sarcoma? What are complications of 45 Gy vs 60Gy?
45-65Gy 45Gy: woudn has delayed healing 60Gy: wound not expected to heal
220
CD99
Ewing's sarcoma
221
8 lesiosn that can be found in vertebral body
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
dDx for Benign aggressive lesion
Osteoblastoma GCT ABC CMF Chondroblastoma ±UBC
223
Long term survival of parosteal osteosarcoma when local contrl has been achieved
95%
224
What subtype of lipoma is painful?
AngioLipoma
225
Poor prognostic indicators in Ewing's
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
Most common site of mets of osteosarcoma
Lung: most common Another bone: second most common
227
Tumours that mets to bone
breast lung thyroid renal prostate \*all the paired organs
228
What are the three treatment facets of a MM bone lesion?
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
CD31 & polyvinyl chloride exposure is a risk for what?
ANgiosarcoma rare
230
What is the local recurrence rate with ABC? Name 1 adjunct that may help decrease local recurrence?
25% local recurrence High speed burr Phenol Less common in older kids
231
Bloodwork for metastasis with unknown origin?
Spep/upep CBC Esr Lytes Liver enzymes Psa Alp LDH
232
Survival of Chordoma?
60% 5 year survival 25% long term survival local extension may be fatal
233
What kind of biopsy is contraindicated in chordoma?
transrectal
234
Maffuccis is characterized by: A) multiple enchondromas, hemangiomas, lymphangiomas B) multiple enchondromas in a unilateral distribution C) auto-dominant transmission, multiple enchondromas and osteochondromas
A
235
What does it mean if a Tumor is biphasic?
It contains both epithelial and mesemchymal neoplastic cells
236
For ewings sarcoma, what blood markers should be added to the typical malignant work up and why?
CBC, esr and crp to differentiate it from osteomyelitis In ewings esr will be up (but not crp?)
237
Indications for marginal resection of lipoma (4)
Symptomatic lesions Rapidly growing mass Deep to fascia or in retroperitoneum Spindle cell & pleomorphic variants
238
What term is typically used to describe the following genes: p53 and RB-1
Tumor suppressor genes.
239
In terms of tumour location, what are apophyses treated like?
Epiphysis THINK: locations for chondroblastoma (GT apophysis, calcaneal apophysis etc...)
240
3 dDx for anterior tibial liesion
osteofibrous dysplasia adamantinoma malignant fibrous histiocytoma
241
What tumor am I? Estrogen Beta Positive Locally invasive but benign Can treat with Tamoxifen Associated with FAP and Dupuytrens
Extra-Abdominal Desmoid Tumor
242
Lifetime risk of malignant transformation for solitary enchondroma?
1%
243
What is special about biphasic sarcomas in terms of metasteses?
They can go to lymph nodes, which is unusual for sarcomas. Also they metastasize to locations other than the lungs.
244
Name 2 types of benign periepheral nerve sheath tumours What's the difference
Scwannoma: only made up of Schwan cells Neurofibroma: Arises from Schwann cells but also has other cells (fibroblasts etc)
245
PVNS typically contains which pigment?
Hemosiderin deposits
246
Gene associated with primary ABC?
Tre2
247
3 Predictors of UNSUCCESSFUL treatment of UBC with steroids
active lesion large size multiloculated
248
Long standing chronic draining wounds or burn scar are at risk of what?
Transformation into SCC (Marjolin's ulcer)
249
What Tumor is usually epiphyseal in skeletally immature with chicken wire calcifications?
Chondroblastoma Also has mononuclear chondroblasts
250
Name 3 soft tissue tumours that chemo can help with:
Synovial sarcoma Leiomyosarcoma Rhabdomyosarcoma (only in paediatric population, not adults)
251
Common presentations in Chordoma?
bladder & bowel changes are common! palpable on rectal exam
252
What staging investigation must you include in epitheliod sarcoma (and certain other soft tissue sacomas)
Sentinel LN biopsy
253
Li-Fraumeni syndrome: what germ line mutation & tumour is it associated with
p53 osteosarcoma also: breast, colon Ca
254
Name 4 vertebral body tumours
hemangioma GCT chordoma multiple myeloma Lymphoma
255
What is the most common benign radiation induced tumour of bone?
osteochondroma
256
Indications for bone marrow biopsy? (i.e. what tumors necessitate this)
1. Ewings 2. Multiple Myeloma 3. Rhabdomyosarcoma
257
Patient is complaining of on and off pain, tenderness to palpation and cold intoleratnce. This is the clinical picture. Diagnosis & Management?
Glomus tumour _Classic triad_ * paroxysmal pain * exquisite tenderness to palpation * Cold intolerance Treatment: marginal resection is curative
258
Tretment of fibrous dysplasia?
Observation ± bisphosphonates: asymptomatic lesions Operative with internal fixation and autologous bone graft (symptomatic)
259
What is unique in Ewing's as part of staging?
Bone marrow biopsy looks for BM mets that would change prognosis
260
Which primary has worst prognosis once mets are present in bone?
Lung
261
How do you differentiate between ABC and telangiectatic osteosarcoma?
Biopsy Telengiectatic osteosarcoma is Slayers Sarcoma because it is Lakes of Blood
262
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
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
SYT-SSX1, SYT-SSX2, or SYT-SSX4 translocation (t(X;18)(p11;q11))
Synovial sarcoma
264
Classic spine finding in EG?
vertebra plana Couldn't find a good picture but can be subtle - remember the SPORC case??
265
What is the risk of malignante transformation of fibrous dysplasia?
1% into osteosarcoma, fibrosarcoma or MFH
266
What is the usual indication for radiation therapy at the following doses? a) 6 Gy b) 30 Gy c) 60 Gy
a) HO prophylaxis b) Treatment of a boney Met c) Adjuvant therapy for soft tissue sarcoma
267
Poor prognostic sign in neuroblastoma?
bony mets
268
Name 2 tumours affecting multiple vertebra
metastatic disease multiple myeloma (remember may be cold on bone scan)
269
Benign aggressive looking lesion. Must rule out what malignancy?
Telangiectatic osteosarcoma
270
Enchondromas always central metaphyseal t/f?
True
271
Which sarcoma is radio/chemo resistant? A) liposarcoma B) osteosarcoma C) chondrosarcoma D) Ewing's sarcoma
C) chondrosarcoma
272
What are the three biphasic sarcomas?
1. Synovial sarcoma 2. Angiosarcoma 3. Mixed liposarcoma
273
UBC on MRI?
very Dark on T1 Very bright on T2
274
What percentage of UBCs heal after fracture?
10-15% (JAAOS 2014 says
275
Which soft tissue tumour is relatively insensitive to both radiation and chemo?
Angiosarcoma
276
Man has radicular buttock pain MRI shows large nerve lesion in sciatic What is it likely?
Neurofibrosarcoma (malignant peripheral nerve sheath tumour) They affect large nerves Must resect entire affected nerve
277
What type of nerve cells is responsible for the neurofibromas in NF1?
non-myelinating Schwann cells (vs. myelinating in solitary neurofibromas)
278
What type of resection are you performing with PVNS
Marginal resection is what you're aiming for (with arthroscopic, probably doing intralesional)
279
Host lamellar bone entrapment is characteristic of what malignancy?
Chondrosarcoma
280
Best UBC lesions to pursue steroid injections
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
Most common presentation of chondroBLASTOMA
pain
282
Treatment for multiple myeloma
multiagent chemotherapy (mainstay) bisphosphonates (helps reduce number of skeletal events) Surgical stabilization
283
Orthopaedic Tumours that Spread to LN's
Synovial Sarcoma epitheliod sarcoma angiosarcoma rhabdomyosarcoma clear cell sarcoma
284
Diagnosis & Treatment?
Adamantinoma (NOT OFD b/c not confined to cortex - much more malignant. ALso associated with bowing) Treatment: wide resection + reconstruction (intercalary graft)
285
Histologically, how do you differentiate ABC from telangiectatic osteosarcoma?
TO: lakes of blood filled mixed with neoplastic cells (top) ABC: Cavernous blood filled spaces with no endothelial lining (bottom)
286
Describe Mazabraud syndrome
Polyostotic fibrous dysplasia intramuscular myxoma
287
What is a Bence Jones Protein?
Light chain immonuglobulin found in multiple myeloma.
288
What kind of chondrosarc does enchondrama change into?
low grade chondrosarcoma
289
## Footnote Lifetime risk of malignant transformation for Maffucis?
100%
290
Name the 3 manifestations of eosinophilic granuloma and their characteristics
_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
What tumor shows predilection for the distribution shown in this figure?
Lymphoma
292
Recurrence rate of GCT of tendon sheath after marginal excision?
5-50%
293
Treatment for neurofibrosarcoma (malignant peripheral nerve sheath tumour)? What must you specifically do?
Wide resection Must resect the entire affected nerve
294
Name the syndromes associted with the spectrum of Langerhans cell histiocytosis
_Eosinophilic Granuloma_ * Self-limited in younger patients _Hand-Schuller-Christian Disease_ * Chronic, disseminated form _Lettere-Siwe Disease_ * Fatal for in young kids
295
Do osteochondroma transform into high or low grade chondrosarcoma?
Low grade
296
Which primary can have mets distal to knee and elbow?
Lung
297
Name 1 poor prognostic factor for parosteal osteosarcoma
de-differentiation
298
Indicatios for surgical resection in osteoid osteoma
painful scoliosis Too close to vital strucures for radiofrequency ablation (neural elements, skin)
299
NOF can be associated with what other tumour
ABC
300
In operative management of fibrous dysplasia, what should you never use
Autograft It will rapidly be turned into fibrous dysplastic woven bone
301
Benign aggressive lesions? (3)
GCT, ABC, osteoblastoma
302
Seven questions for evaluating bone lesion on x-ray? (as per Toronto group)
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
Which soft tissue sarcomas need chemo?
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
Treatment of this pathologic fracture?
Sling for comfort Nonop of pathologic fractures through UBC, unless they are in a weight bearing zone (proximal femur)
305
Which way do pedunculated osteochondraom lesions point?
Away from the joint
306
3 poor prognostic indicators for soft tissue sarcoma?
High grade size \>5cm tumour location below deep fascia
307
how often is ABC associated with another tumour?
30% of the time
308
Synovial sarcoma genetics
t(X;18) Forms SYT/SSX1 & SYT/SSX2 fusion transcripts
309
Medical treatment options in UBC
Bone graft (poor success with high recurrence rates) Steroid autologous bone marrow (controversial - may be better than steroids) JAAOS 2014
310
Why must you follow chordoma patients long term post resection?
1. High recurrence rate 2. Mets occurs late in disease Occurs in 30-50% to lung, rarely bone
311
Nonoperative mangement of Paget's When is it indicated
Bisphosphonates Calcitonin Indicated in *sympomatic* cases asymptomatic you just treat supportively
312
What tumor shows predilection for the distribution shown in this figure?
Chondroblastoma
313
What extra-articular tumor has pathology similar to PVNS?
Giant cell tumor of tendon
314
What are the basic types of soft tissue sarcoma (think histo)
Synovial (sarcoma) Lipo(sarcoma) Rhabdomyo(sarcoma) Fibro(sarcoma)
315
GCT histology?
Mononuclear cells with Uniformly distributed multinucleated giant cells