Soft Tissue

Question 1

What is the most common sarcoma in children?

Answer 1

Rhabdomyosarcoma

Question 2

What is the most common sarcoma in young adults?

Answer 2

Synovial sarcoma

Question 3

What is the most common sarcoma in mid to late adults?

Answer 3

Undifferentiated pleomorphic sarcoma

Liposarcoma

Question 4

What is the most common adult soft tissue tumor?

Answer 4

Lipoma

Question 5

What is a conventional lipoma?

Answer 5

Thinly encapsulated yellow tumor

Lobules of mature fat

Question 6

What are characteristic findings of a liposarcoma that differentiate it from a lipoma?

Answer 6

Atypical spindle cells

Cytoplasmic vacuoles, scallop nucleus

Question 7

What is MDM2 amplification associated with?

Answer 7

Liposarcoma

Question 8

What mutation is associated with a myxoid/round cell liposarcoma?

Answer 8

t(12;16) FUS-DDIT3 fusion protein

Question 9

Where are myxoid/round cell liposarcomas usually located?

Answer 9

Intramuscular, most often the thigh

Question 10

What is the most common location of nodular fasciitis?

Answer 10

Volvar forearm

Question 11

What is nodular fasciitis?

Answer 11

Spindle cell tumor of fibroblasts

Question 12

What is myositis ossificans?

Answer 12

Fibroblastic tumor that eventually develops bone

Occurs subcutaneously and in muscle

Question 13

What is Palmer fibromatosis?

Answer 13

Nodular thicening of palmer fascia; puckers skin and digit flexion contraction

Dupuytrens contractions*

Question 14

What is plantar fibromatosis?

Answer 14

Irregular of nodular thickening of plantar fascia

Question 15

What is penile fibromatosis?

Answer 15

Peyronie’s disease

Induration of mass on dorsolateral penis

Question 16

What mutations occur in deep-seated fibromatosis?

Answer 16

APC or B-catenin

Question 17

What are the types of deep-seated fibromatosis?

Answer 17

Abdominal

Intra-abdominal

Extra-abdominal

Question 18

What syndrome are intra-abdominal desmoid tumors associated with?

Answer 18

Gardner syndrome

Question 19

What are fibrosarcomas?

Answer 19

Infiltrative fish-flesh with hemorrhage and necrosis

Spindle cell withaareas of herringbone pattern

Question 20

Where do rhabdomyosarcomas usually occur?

Answer 20

Head/neck

GU tract (not kidney)

Question 21

What rhabdomyosarcoma has the best prognosis?

Answer 21

Embryonal rhabdomyosarcoma

Question 22

What is the sarcoma botryoides variant of embryonal rhabdomyosarcoma?

Answer 22

Clust of grapes protrudes into lumen

Cambium layer: submucosal hypercellular layer

Question 23

What three markers are associated with rhabdomyoblasts?

Answer 23

MYOD1

Myogenin

Desmin

Question 24

What are tadpole cells and strap cells?

Answer 24

Striated cells characteristic of rhabdomyosarcoma

Question 25

What is an alveolar rhabdomyosarcoma?

Answer 25

Fibrous speta divide tumor into alveolar-like spaces centrally containing discohesive cells while peripheral cells stick to wall

Question 26

What translocation is associated with alveolar rhabdomyosarcoma?

Answer 26

t(2;13) or t(1;13)

Question 27

What is a leiomyoma?

Answer 27

Benign smooth muscle tumor Bland smooth muscle in fascilcles Most common tumor in the uterus

Question 28

What is a leiomyosarcoma?

Answer 28

White masses Malignant spindle cells Positive for smooth muscle actin and desmin

Question 29

What is a synovial sarcoma?

Answer 29

Biphasic tumor Typically around large joints Cytokeratin positive

Question 30

What what translocation is associated with synovial sarcoma?

Answer 30

t(X;18) STY-SSX1 or STY-SSX2 fusion

Question 31

What is an undifferentiated pleomorphic sarcoma?

Answer 31

Group of aggressive neoplasms Diagnosis of exclusion