Oncology Flashcards
1
Q
Name 2 diseases that result from dysfunction of osteoclasts.
A
- ) Paget’s Disease - increased osteoclastic bone resorption
- ) Osteopetrosis - inability of osteoclasts to resorb bone
2
Q
What sarcoma do we routinely get a bone marrow biopsy for?
A
Ewing’s in order to look for marrow metastasis! = small round blue cell tumor! (uncertain of cell of origin but most believe from bone marrow hence the biopsy!)
3
Q
How can myositis ossificans that is in contact with the cortical surface of the bone be differentiated from parosteal osteosarcoma?
A
Myositis Ossificans -> peripheral bone formation w/ central lucency
Parosteal Osteosarcoma -> has mineralization centrally!
**NEITHER have connection to medullary canal!
4
Q
Workup for newly found suspicion for metastatic dz w/ bony involvement should follow what ordering for staging and biopsy? Which goes first?
A
1st = Staging -> CT CAP (will tell you where primary is BLT KP), whole body bone scan (will tell you what bones involved) 2nd = biopsy
5
Q
What is a common secondary surgery needed after the re-excision of a soft tissue tumor that was initially resected and thought to be benign but then found to be malignant?
A
Plastic surgery/FLAP coverage! B/c typically have to take a much wider amount of tissue!
6
Q
Li-Fraumeni syndrome is a/w what gene and what MSK neoplasm?
A
p53
Osteosarcoma
7
Q
Multiple Hereditary Exostoses is a/w what benign lesions? What malignant tumor can occur? What is the gene a/w this syndrome?
A
Osteochondromas
Chondrosarcoma (look for increase in cartilage cap!)
EXT1, EXT2 (tumor suppressor gene)
8
Q
What inheritance pattern does MHE have?
What gene a/w it?
What does this gene mutation cause on micro level?
What deformity can be seen with it?
What are they at risk for transformation into?
A
AD
EXT 1, EXT2 (tumor suppressor gene)
Reduction of Heparan Sulfate which you will see on analysis of physis
Madelung deformity in forearm
Chondrosarcoma! (think about if growing significantly and if cartilage cap»_space; 2cm)
9
Q
Ollier’s Dz vs Maffucci’s Syndrome
A
Both have multiple enchondromas and genetic association is PTHR1
Olliers -> 30% risk chondrosarcoma
Maffucci -> also have multiple hemangiomas; 100% risk chondrosarcoma
10
Q
McCune-Albright is a/w what bony lesion and what other involvement? What is gene involved?
A
Polyostotic Fibrous dysplasia
Cafe au lait spots (Coast of Maine), precaucious puberty
GNAS (alpha subunit of Gs protein) -> leads to over production of cAMP
11
Q
What is the chromosomal translocation for (alveolar) Rhabdomyosarcoma? What fusion gene is created? What population does this tumor occur in? What are 3 special facts about it?
What is the cytochemical stain?
What is the treatment?
A
2:13 -> PAX3-FKHR
MOST soft tissue sarcoma in CHILDREN
Can spread via lymph nodes (“RACES”)
It’s a ST sarcoma sensitive to chemo!
Small round blue cell tumor!
DESMIN+/Vimentin+ (note: Vimentin is general marker for STS)
EXCEPTION! -> Chemo + wide excision (+/- radiation)
12
Q
What 2 soft tissue sarcomas are sensitive to chemo?
A
Rhabdomyosarcoma
Synovial Sarcoma
**Remember it’s the 2 on the outside of the “RACES”
13
Q
What 4 MSK tumors are treated with wide excision/limb-salvage surgery ALONE?
A
"CCAP the treatment with surgery alone!" Chondrosarcoma Chordoma Adamantinoma Parosteal osteosarcoma
14
Q
What 2 MSK tumors are treated w/ chemo sandwich (chemo-surgery-chemo)?
A
- ) Osteosarcoma
2. ) Ewing Sarcoma
15
Q
What is the treatment for ST sarcoma? What are the 2 exceptions and what do they get?
A
Radiation + excision
Rhabdomyosarcoma
Synovial sarcoma
16
Q
What 3 MSK-related tumors are treated w/ ORIF + radiation?
A
Mets
Lymphoma
Myeloma
17
Q
What 3 MSK situations do you use whole body bone scan during the staging?
A
- ) Osteosarcoma
- ) Ewing Sarcoma
- ) Mets
18
Q
What are the small round blue cell tumors?
A
"LERNM" (ERN are in < 30 yo; LM are in > 30 yo!) Lymphoma Ewings Rhabdomyosarcoma Neuroblastoma Myeloma
19
Q
What malignant primary bone tumors typically occur in pts < 30 yo? (2)
A
- ) Osteosarcoma
2. ) Ewing Sarcoma
20
Q
What benign MSK tumors typically occur in pts < 30 yo? (7)
A
"Oh, Oh, Oh Children Lucky And Nice" Osteoid osteoma Osteoblastoma Osteofibrous dysplasia Chondroblastoma LCH ABC NOF
21
Q
CD 99
A
Ewing Sarcoma
22
Q
Smooth muscle actin
A
Leiomyoma
Leiomyosarcoma
23
Q
Desmin
A
Rhabdomyosarcoma
24
Q
S100
A
Melanoma
Nerve sheath tummors -> MPNST, Schwannoma (Neurilemmoma)
25
CD 34, CD 31
Vascular tumors ->
Hemangioma
Hemangioendothelioma
Angiosarcoma
26
Nuclear Beta catenin
Fibromatosis (aka Desmoid)
| a/w Familial adenomatosis polyposis!
27
Keratin
All carcinomas
Epithelioid sarcoma
Synovial sarcoma
28
Loss of INI 1 (Integrase Interactor 1)
Epithelioid sarcoma
29
Epithelial Membrane Antigen (EMA)
Epithelioid sarcoma
| Synovial sarcoma
30
What metastatic cancer leads to acral mets (ie mets distal to the elbow/knee)?
MOST commonly LUNG
| Sometime Renal
31
What 2 tumors do we get bone marrow biopsy/aspirate for?
1. ) Ewings
| 2. ) Multiple Myeloma
32
What is know side effect/toxicity of Doxirubicin?
Cardiotoxicity!
| If have hx of taking this drug/chemo then needs Echo prior to surgery!!
33
What is the treatment for Ewings?
| What problem should you watch out for later? Presentation? How do you screen for it?
Chemo sandwich!
| Leukemia (chemo associated!) -> look for it w/ a BLOOD SMEAR. Presents with fatigue, bleeding of gums, easy bruising
34
What is the typical treatment for soft tissue sarcoma? (Which 2 ST sarcoma's are exceptions to the rule?)
What are the options for giving typical treatment and what complication are associated?
Radiation + Surgery! (Rhabdomyosarcoma & Synovial Sarcoma -> Chemo + Surgery!)
Preop Rads: 50 Gy -> early rads = early complication = wound healing problems
Postop Rads: 60 Gy -> late rads = late complication = fibrosis and fracture!
35
What 3 lesions are seen in the epiphyseal region?
CGC!
Chondroblastoma (children)
Giant Cell (adults)
Clear Cell Sarcoma (femoral head epiphysis is classic!)
36
Flat bone lesion in kid?
| Flat bone lesion in adults?
Kid -> Ewing
| Adult -> Chondrosarcoma
37
What does chondromyxoid fibroma look like on XR? What bone is it typically in?
What does histology look like?
And what is treatment?
```
Tibial lesion (metaphysis), bubbly, lytic, eccentric lesion that can take out cortex (SHARK BITE TO PROXIMAL TIBIA!).
PAINFUL lesion (helps differentiate from NOF which is painless)
Histology -> myxoid = stellate cells (so realize that these cells can be seen in tumor path w/ "myxoid" in the name!) w/ cartilaginous matrix
Tx -> remove it w/ curettage and bone grafting b/c benign aggressive
```
38
In a kid with mutiple lesions what tumor/dz should you think about?
In adult?
LCH (pink and punched out!)
| Multiple Myeloma
39
What does disseminated LCH involve and what is it called?
| What is treatment?
Hand-Schuller-Christian dz
Multifocal LCH + exopthalmos, DM
Chemo
40
What dz is an example of benign metaplastic change of synovial tissue?
Synovial chondromatosis
41
Describe the Enneking Staging System
```
A= intracompartmental/ B = extracompartmental
Stage I (A/B) = low grade
Stage II (A/B) = high grade (STAGE IIB IS MOST COMMONLY THE STAGE THAT SARCOMAS PRESENT!)
Stage III = Mets
```
42
What descriptors are associated with a ST sarcoma?
Size > 5 cm and > 1.5 cm in hands/feet!
Deep to fascia
Growing
**Need 3D imaging (CT or MRI) of any growth > 5 cm and > 1.5 cm in hands/feet
43
What criteria does a lesion need to have in order to consider performing an excisional biopsy?
**The things that make you think is a lipoma and you just want to perform a marginal excision
Size < 3cm (< 1.5 cm in hands/feet), consistent with FAT on all sequences (and no heterogeneity seen in the middle of the lesion), AND NOT near critical structures (ie NV bundle)!
44
What is the treatment for the unplanned excision of a ST sarcoma?
Re-image area, radiation/surgery (doesn't matter which order as usual); likely will need FLAP coverage
*note: if is just a SUPERFICIAL lesion (not deep to fascia, no involvement of muscle) then you don't need to radiate, just need re-excision (regardless of size)!
45
Factor that most determines the local recurrence rate?
A positive margin!
46
What can synovial sarcoma lesion look like on MRI that we should be aware of?
Large cystic, bright T2 lesion!
47
What marker is a/w atypical lipomatous tumor/well-differentiated liposarcoma?
What is the treatment?
```
MDM2
Marginal resection (don't need radiation!) - remember this by the fact that you marginally resect all lipoma's...and you send them all for MDM2 and it's not until then that you know it is a well-differentiated liposarcoma (all you change is the long-term monitoring for recurrence!)
```
48
Vimentin (+)
Generalized marker for ST Sarcomas!
49
Is fibromatosis (aka desmoid) benign or malignant?
Where can they commonly occur?
What do they look like on T1/T2 MRI sequences?
Treatment?
Immunohistochemistry findings?
What dz/syndrome is this a/w?
Benign
Hand/Feet - think about association w/ Dupuytrens
Dark ON BOTH T1 and T2 MRI!!! -> can look like soft tissue sarcoma - but look for it to actually be more invasive looking (sarcoma's often times have nice, round boarders)
Wide local excision b/c HIGH RECURRENCE RATE!! (locally aggressive - invades muscle!)
Nuclear Beta-catenin AND Estrogen!!!
Familial Adenomatosis Polyposis (FAP!)
50
What intra-articular masses are malignant?
NONE!!
51
What is Cat Scratch Dz?
What bacteria causes it?
Treatment?
Can mimic a STS! - but is an inflammed lymph node and histology will show this.
Look for usually a forearm mass + history of cat exposure
Bartonella henselae
Doxycycline
52
What does osteoid osteoma produce that makes the lesion painful?
Prostaglandin E2
53
What are the major differences b/t osteoid osteoma and osteoblastoma?
Osteoid osteoma: < 2 cm, NSAIDS/RFA (en bloc reserved for lesion in spine/fingers by NV bundle!)
Osteoblastoma: > 2cm, pain NOT typically relieved by NSAIDS, tx w/ curettage and grafting
Otherwise -> they look the same on path (woven bone with a bunch of osteoblasts)
54
1. ) What cells is dysfunctional in Pagets?
2. ) What do XR look like?
3. ) What do you typically see on path?
4. ) What can it transform into and what does this look like on path? What is prognosis?
5. ) What treatment should you give them?
6. ) What treatment can increase their risk of transformation to the above bad?
7. ) What labs for diagnosis?
1. )Osteoclast -> increased osteoclast activity and bone turnover (over resorbes, over lays down bone - continuous cycle)!
2. ) XR -> moth eaten appearance, bowing deformity due to weakened bone
3. ) Path -> look for cement lines w/in regular looking bone
4. ) Can transform into osteosarcoma!! -> think about this if you see woven bone! -> Prognosis is death!
5. ) Treat Pagets people w/ Bisphosphonates!
6. ) Teraperitide -> increases risk of Pagets Osteosarcoma!
7. ) Alk Phos, Urine hydroxyproline, N-telopeptides
55
CD 45 marker
| What is the treatment?
Lymphoma
| Chemo & radiation
56
For treatment of a metastatic lesion that is concerning for fracture/fractured, in addition to surgery, what are 2 other treatments you should give?
1. ) Radiation to whole bone (typically doing a nail and you will spread the mets throughout the whole bone)
2. ) Bisphosphonates - reduces bone resorption caused by osteoclasts
57
What do you need to look for on path to let you know it's a Giant Cell Tumor (and not just a tumor that has some giant cells in it - like ABC, Chondroblastoma)?
What is the treatment?
What does the result of treatment depend on?
Where can mets?
What med can be given? And what's the MoA?
What lineage is the Giant Cell derived from?
It is DEFINITELY GIANT CELL TUMOR if the stromal cells (background cells) look the same as the nuclei that are in the giant cells (****chocolate chip cookies surrounded by chocolate chips!!) Also, stromal cells = the pimps that tell the giant cells = the hoes what to do!
Tx: AGGRESSIVE INTRALESIONAL RESECTION +/- adjuvant +/- reconstruction (cement, ORIF, bone graft)
***RESULTS DEPEND ON THOROUGHNESS OF RESECTION!!
Mets to lungs
Denusomab -> Antibody to RANKL (Giant cells express RANK...this is what makes these tumors so lytic!!)
Lineage -> preosteoblastic mononuclear cell (*Note this b/c it feels confusing: osteoblasts come from mesenchymal line/osteoclasts come from hematopoietic/monocyte lineage)
58
What finding on pathology is found w/ mets to bone?
Nests and glands by bone!
59
Which bone sarcoma's are treated with surgery (wide resection) ONLY?! (2)
1. ) Parosteal osteosarcoma
| 2. ) Chondrosarcoma
60
On XR of a humerus, for example, how can you tell an enchondroma from a chondrosarcoma apart if they both have arcs/whirls?
Cover up the lesion with your finger and look at the surrounding bone! If surrounding bone looks normal -> enchondroma! If you notice scalloping/expansion -> chondrosarcoma!
61
Difference b/t fibrous dysplasia and osteofibrous dyspasia. Describe XR, path, mutation
Fibrous dysplasia ->
XR: ground glass lytic lesion
Path: Chinese letter (NO osteoblastic rimming)
Mutation: GNAS gene (Gs protein alpha subunit) -> over production of cAMP
Osteofibrous dysplasia ->
XR: lytic lesions typically on anterior cortex of tibia
Path: Chinese letters but also has OSTEOBLASTIC RIMMING!
Mutation: NOT GNAS!
62
What are 2 main downsides to radiation?
| What are treaments of these?
1. ) Radiation-induced bone osteonecrosis -> WILL NEVER HEAL B/C DEAD SO DON'T TRY TO FIX BONE -> CUT IT OUT AND USE PROSTHESIS!
2. ) Radiation-induced soft tissue sarcoma -> Chemo and surgery (can't treat like regular ST sarcoma w/ rads + surg b/c the rads already caused cancer!!)
63
For a femoral head or neck met in a breast CA patient that needs surgery what is best treatment?
What time frame for survival of mets disease is good/you should choose more aggressive/surgical fixation option?
THA (better than hemi b/c they have a long lifespan!!)
| > 3 months!
64
What pathology can osteoid osteoma cause in the spine?
Scoliosis & paraspinal pain -> will resolve when treat osteoid osteoma!
65
What sarcomas can metastasize to the lymph nodes?
| What is their primary location of mets?
```
"RACES"
Rhabdomyosarcoma
Angiosarcoma
Clear Cell Sarcoma
Epithelioid sarcoma
Synovial sarcoma
***LUNG IS STILL MOST COMMON LOCATION FOR METS!!!
```
66
Compare Neurilemmoma (Schwannoma) vs Neurofibroma
```
***BOTH derived from Schwan cells!
Neurilemomma (Schwanomma):
* Can be a/w NF-2
* Located eccentrically in nerve - Grows on top of nerve fibers - well encapsulated -> can just cut open and take this ball of tumor out
* (+) Tinels
* Verocay bodies on path
```
Neurofibroma:
* A/w NF-1
* Located centrally in nerve - Arises from w/in the nerve. So to remove it you lose nerve function distal to it
* (-) Tinels, local pain/nerve compression, rarely radiating pain
* Target sign on MRI
67
Which carcinoma's that metastasize to bone are considered highly vascular and should often have preop embolization?
Thyroid
| Renal
68
What can cause Vertebra Plana?
```
"MELT" the vertebrae!
Mets, MM
Eosinophilic granuloma
Lymphoma, Leukemia
Trauma, TB
```
69
What lesions are primarily diaphyseal?
```
Vowels -> "AEIOUY"
Adamantanoma
Eosinophilic granuloma
Infection
Osteoid osteoma, osteoblastoma
Uings - Ewings
Y - fibrous dYsplasia, lYmphoma, mYeloma
```
70
What are the blue cell tumors and what immunohistochemical marker helps to ID each?
1. ) Lymphoma -> CD 45+, Leukocyte Common Antigen+
2. ) Ewings -> CD99+
3. ) Rhabdomyosarcoma -> Desmin+
4. ) Neuroblastoma -> Neuron Specific Enolase+, NB 84
5. ) Myeloma -> CD 20+, Kappa or lambda light chains
71
What tumors are commonly seen in the sacrum?
1. ) ABC/GCT (commonly occur together!)
2. ) Chordoma
3. ) Mets
72
In a patient w/ suspected myeloma, what is the test ordered for:
1.) Diagnosis
2.) Staging
What is the treatment for Myeloma
1. ) SPEP/UPEP; bone marrow biopsy
2. ) Skeletal survey (cold on bone scan - so NOT useful!)
Chemo/radiation (ORIF for any impending fx)
73
What benign bone tumors must you fix and not just observe?
1. ) CMF (destructive of proximal tibial cortex)
2. ) Chondroblastoma (destructive in epiphysis)
3. ) ABC's b/c ballooning (wider than the physis they are close to) and destructive
4. ) Giant Cell
5. ) Osteoblastoma
74
What are the 2 main locations of chondrosarcoma?
Flat bone and metaphysis
| can arise from enchondroma - esp Ollier's & Maffuci's pts or osteochondroma - MHE pts
75
What is Rothmund-Thomson Syndrome?
Sun-sensitive rash w/ prominent telangiectasias.
A/w osteosarcoma
Genetic assoc -> RECQL4
76
What is Mazabraud Syndrome?
Fibrous dysplasia + soft tissue myxomas
| Genetic assoc -> GNAS
77
What is Jaffe-Campanacci Syndrome?
Multiple NOF's + Cafe au lait patches
78
What is Stuart-Treves Syndrome?
Seen in patients w/ chronic lymphedema (think about in woman w/ hx of lymph node dissection, typically seen in UE's)
Develop Angiosarcoma
79
What does lymphoma look like on XR and MRI imaging?
What is the marker?
What is the treatment?
XR -> is in the "Y" category of AEIOUY! So is diaphyseal...usually underwhelming/unimpreesive looking on XR but on MRI there will be a big soft tissue mass that you didn't appreciate on XR!
CD 45+, Leukocyte Common Antigen
Chemo/radiation!
80
What is the cell on pathology that is pathognomonic for chordoma?
Physaliferous cell (bubbly cytoplasm w/ signet ring)
81
What is the characteristic cell with LCH?
| What cell is seen on EM?
```
Langerhans cell ( = histocyte w/ curved nucleus like a kidney bean); NOT the eosinophil, but you will see LOTS of these on the path!
Bierbeck granules (shaped like Tennis rackets!)
```
82
1. ) In GCT - molecularly what causes the lytic lesion?
| 2. ) What drug can be given? How does it work?
1.) Stromal cells ("pimps/chocolate chips") produce RANKL
Giant cells ("fat hoes") have RANK on surface and are responsible for osteolytic effect (Giant cells come from monocyte lineage, just like osteoclasts...but can be called preosteoblastic mononuclear cell - confusing!)
2.) Denosumab -> antibody to RANKL
83
Where is the MC location of GCT?
Distal femur!
| Can also be seen in sacrum and DR
84
What is the typical treatment for Ewing's Sarcoma?
| Caveat to this?
Chemo Sandwich! Neo-chemo, wide resection/LSS, Chemo
Caveat -> if wide resection too detrimental to function and going to have positive margins then will also use radiation (which it is VERY sensitive to!) - the reason don't always use radiation is b/c these patients are young and risk of radiation-induced sarcoma is higher
85
What is the appearance of Adamantinoma vs Osteofibrous Dysplasia on Pathology?
(since on XR they can look similar - lytic lesion/deformity of anterior tibia)
Adamantinoma = epithelial nests in fibrous stroma
| Osteofibrous dysplasia = "Chinese Letters" w/ OSTEOblastic rimming
86
What are the 3 main factors that are made by tumor cells that signal osteoblasts to secrete RANKL?
1. ) PTHrP
2. ) TGF-beta
3. ) VEGF
87
Chronic draining wound is a risk for what type of cancer?
Squamous Cell Carcinoma = Marjolin's Ulcer
88
Most common location for skeletal mets?
Spine!
89
When you see an ABC - what malignant lesion should you also think about and give second thought to make sure that it is NOT this?
Telangictatic Osteosarcoma (on XR the ABC will be VERY expansile but will have a shell of bone around it....the Telangictatic OS will often not have shell b/c growing fast and destructive - may see fluid/fluid levels with both on MRI)
90
What 2 benign MSK tumors can metastasize? Where do they go?
GCT and Chondroblastoma
| Lungs -> get Chest CT or XR to eval!
91
Where else can a Chondroblastoma be found (besides the epiphysis)?
Talus and Calcaneus
92
1. ) What lesion has 12:16 translocation?
| 2. ) Where can it metastasize?
1. ) Myxoid liposarcoma
| 2. ) Lungs, and BONE!***
93
If you have a patient with an osteosarcoma that sustains a pathologic fracture during their neoadjuvant chemo treatment regimen - what do you do?
Cast them and continue to completion with their neoadjuvant chemo and then perform wide-excision/LSS. (So basically nothing changes and you just immobilize the fracture)
94
What MSK tumor benefits from Estrogen receptor blockade (Tamoxifen)?
Desmoid (Fibromatosis)
95
What 2 medicines should be considered for patients with carcinoma and metastatic lesions to bone?
Bisphosphonates (a/w significant reduction in path fx, need for ortho surgery, need for radiation, spinal cord compression, and hypercalcemia!!)
Denusomab
96
When resecting a sacral chordoma what roots must be saved in order to have normal/near normal bowel/bladder fxn?
Either:
Bilateral S2 or
Unilateral S2-4
