Soft Tissue

Front 1

Which one of these tumors is associated with 12q abnormality
A. Myxoid liposarcoma
B. Atypical lipomatous tumors
C. Nodular fasciitis
D. Dedifferentiated liposarcoma

Back 1

B. Atypical lipomatous tumor (well differentiated liposarc specifically arising in cape area: neck, back, shoulders). Have an excellent prognosis, and although they can recur, metastasis is rare.

Hint 1

Associated with ring chromosome or giant markers from ring chromosome of 12q14-15=MDM2 (and others i.e.CDK4 amp) amplification in every nucleus. Can see with FISH on paraffin embedded tissue. Much better than immuno.

Front 2

A 60 something year old man presents with a posterior neck mass. Which lipomatous tumor, based on this history alone, should be of concern?

Back 2

Spindle cell/pleomorphic lipoma. Usually in older men in post neck, upper back, shoulders (80%).

Front 3

How do you differentiate atypical lipomatous tumor from spindle cell/pleomorphic lipoma

Back 3

ALT
-doesn't have ropy collagen
-rare CD34 + atypical cells

Hint 3

PL/SCL
-has ropy collagen
-may have characteristic giant flourettes (note: these are not pathognomonic)
-CD34 more diffusely positive due to more cellularity. More helpful in defferentating from MFH (which is CD34-) and cellular PL/SCL (which is CD34+)

Front 4

How should you screen for atypical lipomatous tumor?

Back 4

Should see atypical large hyperchromatic cells in fibrous septa at low power (4x). Don't need to see lipoblasts to make the diagnosis.

Hint 4

May also see atypical large hypwechromatic cells between adipocytes or in blood vessel walls.

Front 5

Which one of these tumors is associated with 16q deletion
A. Pleomorhic lipoma
B. Myxoid liposarc
C. Well differentiated liposar

Back 5

A. Pleomorphic lipoma

Front 6

t(12;16)(q13;p11) is associated with which soft tissue tumor?

Back 6

Myxoid/Round cell liposarc. 12=CHOP/DDIT3 and 16=FUS gene. Need 5% or more to call it a round cell (grade 3/3) over myxoid (grade 1/3)

Hint 6

Less commonly see t(12;22)(q13;q12). 12=CHOP/DDIT3 and 22=EWS

Front 7

T/F WDLS/ALT and myxoid /round cell liposarc occur in the same age distribution?

Back 7

False. ALT occurs in older (60 to70) and MLS in 5th decade (not in children less than 10 note: lipoblastoma is a mimic in this age)

Front 8

T/F retroperitoneal is the most common location for myxoid/round cell liposarc?

Back 8

False. That is the preferred site for liposarc (well and de-differentiated).

Hint 8

The most common site for myxoid/round cell liposarcoma is the thigh and popliteal fossa. Retroperitoneal ALT with myxoid change is a mimic. Often metastasized to retroperitoneal, pleura, soft tissue or plevis and less frequently to lungs.

Front 9

What are some defining characteristics of low grade fibromyxosarcoma aka Evans tumor

Back 9

-Very bland looking.
-Sharp demarcation from fibrous to myxoid
-Elaborate vascular pattern
-Related to hyalinizing spindle cell tumor with giant rosettes

Hint 9

* t(7;16) 7=Kreb3L2 and 16=FUS gene
* less common is t(11;16) Kreb3L1

Front 10

What translocation is seen in extraskeletal myxoid chondrosarcoma?

Back 10

Most commonly t(9;22) and less commonly t(9;17) NR4A3

Front 11

What soft tissue tumor probes are commercially available?

Back 11

FUS on 16
EWS on 22
DDIT3 on 12

Front 12

What are good smooth muscle stains?

Back 12

SMA, h-caldesmin

Front 13

What are some good skeletal muscle markers?

Back 13

Desmin, myogenin, MyoD-1

Front 14

T/F reactive myfibroblastic lesions will be positive for SMA and H-caldesmin?

Back 14

False. This profile goes with a true smooth muscle neoplasm. Reactive myofibroblastic lesions would be + for SMA, but - for desmin/h-caldesmin

Front 15

What chromosomes are affected in lipomas?

Back 15

12q, 6p, 13q

Front 16

What chromosome is effected in hibernoma?

Back 16

11q

Front 17

What chromosome is affected in lipoblastoma?

Back 17

8q

Front 18

What chromosomes are affected in spindle cell/pleomorphic lipoma?

Back 18

16q, 13q

Front 19

What cytokeratins are best to order for a sarcomatoid carcinoma?

Back 19

HMW ie CK5/6 pr P63 good for something from surface epitheliaum. If it is from a gland then LMW ie cam 5.2 should be done.

Front 20

What immunohistochemical stains will help differentiate monophasic synovial sarcoma from malignant peripheral nerve sheath tumor?

Back 20

SS (monophasic)
-TLE1
-CK7
-CK19

Hint 20

MPNST
-is neative for TLEI, CK19 and usually neg for CK7. S100 and EMA or pankeratin is not good because both may stain.

Front 21

What chromosome abnormality is seen in synovial sarcoma?

Back 21

-t(x;18)(p11.2;q11.2)
-detected by FISH or RT PCR
-break apart probe will show a red and a green together in a normal case, but will be apart in synovial sarcoma

Hint 21

18= SYT (95%) or SSXT
X=SSX1> SSX2, or less commonly SSX4

Front 22

What percent is ewings/PNET is positive for this characteristic immunostain?

Back 22

-CD99 in 95% of cases
-MYC2 gene product is CD99 a membranous glycoprotein therefore IHC is membranous.

Hint 22

Other round cell tumors positive for CD99 are lymphobalstic lymphoma (use TdT)

Front 23

What round cells tumors are postive for CD99

Back 23

**Ewings/PNET
**Acute lymphoblastic lymphoma (TdT)
*Desmoplastic round cell tumor
*Rhabdomysarcoma
*Small cell osteosarcoma
*Small cell carcinoma
-Synovial sarcoma
-Myeloid sarcoma
_Acute myelogenous leukemia
-Mesenchymal chondrosarcoma

Hint 23

In Ewings?/PNET translocation is
-*t(11;22)(q24;q12)
-11=FL1, 22=EWS (85%)
Probe for EWS via break apart probe
less commonly may see t(21;22) which is the second most common, (7;22), (17;22), (2;22), inv(22q12;22q12)

Front 24

Many tumors have abberatjions in chromosome 22 (EWS)Match the appropriate transolation with the appropriate tumor.
1. t(11;22)
2. t(21;22)
3. t(12;22)
4. t(9;22)
5. t(16;22)

Back 24

a. Myxoid/RC liposarc (5)
b. Clear cell sarcoma (3)
c. ES Myxoid chondrosarc (4)
d.EWS/PNET (1,2)
e.Desmoplastic round cell tumor (1,2)

Hint 24

a. t(16;22) CHOP(DDIT3) EWS but t(12;16) is most common (FUS/DDIT3)
b. t(12;22) ATF-1/EWS
c. t(9;22) CHN/EWS
d. t(11;22) FLI1/EWS
t(21;22) ERG/EWS
e. t(11;22) WT-1/EWS
t(21;22) ERG/EWS

Front 25

What two unrelated tumors have the exact same translocation involving t(2;22)?

Back 25

Angiomatoid fibrous histiocytomas and primary clear cell sarcoma of the GI tract.

Hint 25

Note: clear cell sarcomas of the peripheral soft tissue is usually a t(12;22) KREB1/ EWS

Front 26

Which of these muscle markers is expressed earliest in myogenesis
a. SMA
b. Desmin
c. Actin
d. MyoD1
e. Myogenin

Back 26

e. Myogenin

Hint 26

Myogenin is a nucelar regulatory protein that therefore with immunohistochemsitry you see nuclear staining.

Front 27

Alveolar rabdomyosarcoma has what translocation?

Back 27

most commonly t(2;13) PAX3/FKHR (FOXO1A) but can have t(1:13) PAX7/FKHR

Hint 27

Note; 20% of rhabdomyosarcomas do not have either of these two translocations and the translocation remains unkown.

Front 28

How can one dfferentiate estraskeletal myxoid concrosarcoma from chordoma

Back 28

ESCS
1. Deep soft tissue of extremities, thigh most common, retprpertonieum, head neck also
2. Weak S100
3. Weak Keratin (but postive for EMA and CD57)
4. No physaliphorous cells

Hint 28

Chrodoma
1. Bone axial skeleton
2. Strong S100
3. Strong keratin (CK8, CK190
4. Physaliphorous cells common

Front 29

Desmoid tumors
1. what age goup is affected?
2. Where do they occur?
3. What is the staining pattern?
4. cytogenetic abnormalities?

Back 29

1. Young adults ,40 F>M
2. Deep muscle of shoulder, chest, upper extremety, thigh, head and neck
3. + for SMA, focal for desmin, neg for CD 34, S100
4. Trisomy 8, 20

Front 30

DFSP
1. Who does it affect?
2. Where does it occur
3. What is the histologic pattern?
4. What are some varients?
5. How doesit stain?

Back 30

1. Young and middle aged adults M>F, more in AA
2. Usually dermis and subcutis of chest/abdomen>buttock/ proximal extremities>>>distal extemities, head and neck
3. Storioform with some atypia, entrapment of fat (checker board), and colagen
4. Bednar, Juvenile/giant cell fiibroblastoma
5. +++CD34, Bednar ++ for mlanocytic markers, neg for EMA,desmin, keratin, SMA

Front 31

1. What are thecomone sites for hibeernom?
2. What stain

Back 31

1. Thigh, neck, chest, arm abdomen
2. Uncoupling protein (brownfat differentiatin), S100

Front 32

Myxoid/round cell liposarc
1. Whar are common locations?
2. What type metastasizes more?
3. What is the major constituent of the myxoid matrix?
4. Where does it metastasize
5. how does it stain?

Back 32

1. Deep soft tissue of extremetis, theigh 70%, calf, popliteal area, upper estremity
2. Round cell
3. Hyaluronic acid
4. Soft tissue and bone many years after excision. t(12;16)
5. + for vimentin, variable for CD34, actin, and desmin, nef for S100

Front 33

1. Pleomorphic sarcomas that express vimentin, SMA, and actin (HHF-35, muscle specific actin) are considered what kind of tumor?
2. When does this tumor peak?
3. Where does it occur?
4. What are the variants?
5. T/F radiation is a risk factor
6. T/F CD68 is positive marking histiocytic lineage

Back 33

1. MFH
2.6th/7th decade. Rare before 40
3. Proximal extremeties thigh>trunk
4. Myxoid (>50% myxoid to cal), pleomorphic, inflammatory(rare), giant cell(rare)
5. True. most common sarcma arising after radiation
6. False. It is positive, but is so becaue of high content of lysosomal enzymes not because of histocytic lineage