Lecture 110

Question 1

t(11:22)(q24;q12) or t(21;22)(q22;q12)
Fusion genes: EWS-FLI1 or EWS-ERG

Answer 1

Ewing sarcoma

Results in disordered protein affecting transcription, cell cycle, RNA splicing, and telomerase

Question 2

Large round cells with peripheralized nuclei and lipid droplets

Answer 2

Normal white adipose

Question 3

Most common soft tissue tumor in adults

Answer 3

Lipoma

Question 4

Soft tissue tumor that is composed of mature adipocytes and is well-encapsulated

Answer 4

Lipoma

Question 5

Variants of lipomas can rarely be:

Answer 5

intramuscular

Question 6

Lipoma treatment

Answer 6

Simple excision

Question 7

Malignant tumor of adipose tissue

Answer 7

Liposarcoma

Question 8

Adipose tumors that often occur in deep soft tissues and cause pain, swelling, decreased ROM

Answer 8

Liposarcoma

Question 9

What liposarcoma variant is a result of amplification of chromosome region 12q13-q15?

Answer 9

Well-differentiated liposarcoma

Question 10

Common histology of well-differentiated liposarcoma

Answer 10

Mature adipocytes with scattered atypical spindle cells

Question 11

Which liposarcoma variant is a result of t(12;16)(q13;p11) –> FUS-DDIT2 fusion gene

Answer 11

Myxoid liposarcoma

Question 12

Common histological findings of myxoid liposarcoma

Answer 12

Abundant basophilic stroma, “chicken wire” capillaries, lipoblasts are various stages of differentiation

Question 13

Which liposarcoma variant has a variety of complex karyotypes?

Answer 13

Pleomorphic liposarcoma

Question 14

Common histological findings of pleomorphic liposarcoma

Answer 14

Pleomorphic spindle cells with bizarre nuclei, often with necrosis and hemorrhage

Question 15

Which liposarcoma variant is the most aggressive?

Answer 15

Pleomorphic liposarcoma

Question 16

Self-limited fibroblastic/myofibroblastic proliferation seen in young adults

Answer 16

Nodular fascitis

Question 17

Common sites of nodular fasciitis

Answer 17

Forearm, chest, back

Question 18

t(17;22) –> MYH9-USP6 fusion

Answer 18

Nodular fasciitis

Question 19

What is the prognosis for nodular fasciitis?

Answer 19

Regresses spontaneously

Question 20

What term is used to describe hypercellular areas with myxoid stroma and hypocellular areas with fibrous stroma?

Answer 20

Zonation

Question 21

Histological findings of plump, immature-appearing fibroblasts/myofibroblasts in myxoid to fibrous stroma with zonation indicate what type of lesion?

Answer 21

Nodular fasciitis

Question 22

What type of fibroblastic lesion are benign proliferations of fibroblasts that cause local deformity?

Answer 22

Superficial fibromatoeses

Question 23

Palmar superficial fibromatoses, AKA

Answer 23

Dupuytren contracture

Question 24

Plantar superficial fibromastoses, AKA

Answer 24

Ledderhose disease

Question 25

Penile superficial fibromatoses, AKA

Answer 25

Peyronie disease

Question 26

Often bilateral nodular thickening of palmar fascia leading to progressive flexion contracture

Answer 26

Palmar fibromatoses (Dupuytren contracture)

Question 27

Induration or mass on dorsolateral penis that can cause abnormla curvature

Answer 27

Peyronie disease (penile fibromatoses)

Question 28

Deep fibromatoses, AKA

Answer 28

Desmoid tumors

Question 29

Lage infiltrative masses of fibroblasts that frequently recur but do not metastasize

Answer 29

Deep fibromatoses

Question 30

# 25 What are the most common site of deep fibromatoses?

Answer 30

Anterior abdominal wall, limb girdles, mesentery

Question 31

Patients with familial adenomatous polyposis (Gardner syndrome) are predisposed to:

Answer 31

Deep fibromatoses

Question 32

People with mutations in APC or CTNNB1 are predisposed to type of fibroblastic lesions?

Answer 32

Deep fibromatoses

Question 33

Histological findings of bland fibroblasts in parallel fascicles amind dense collagen that resemble scar tissue are indicative of:

Answer 33

Deep fibromatoses

Question 34

Rar malignant tumor of fibroblasts that occur in deep soft tissue of lower extermities or trunk

Answer 34

Fibrosarcoma

Question 35

High cellularity and high mitotic rate correlate with increased metastasis of this fibroblastic lesion:

Answer 35

Fibrosarcoma

Question 36

Gross morphology of a tumor that is "pseudo-encapsulated" with a "fish-flesh" appearance indicates:

Answer 36

Fibrosarcoma

Question 37

Histological findings that show "herringbone" pattern and hyperchromatic nuclei indicate:

Answer 37

Fibrosarcoma

Question 38

Tissue samples that are vimentin-positive and S-100 negative indicate:

Answer 38

Fibrosarcoma

Question 39

Malignant tumor of skeletal muscle differentiation

Answer 39

Rhabdomyosarcoma

Question 40

What is the most common soft tissue sarcoma of childhood?

Answer 40

Rhabdomyosarcoma

Question 41

Rhabdomyosarcomas typically arise:

Answer 41

Head/neck, genitourinary tract, extremities

Question 42

Which rhabdomyosarcoma subtype appears as a mixture of primitive round/spindled cells, sometimes "strap cells" with visible cross striations?

Answer 42

Embryonal

Question 43

The embryonal variant also includes which varient that arises in hollow, mucosal-lined strcutures?

Answer 43

Sarcoma botryoides

Question 44

The botryoid variant of embryonal rhabdomyosarcoma often looks like:

Answer 44

Grape-like masses

Question 45

Which rhabdomyosarcoma varian has the best prognosis?

Answer 45

Embryonal variant

Question 46

t(2;13) or t(1;13) resulting in PAX3-FOXO1 or PAX7-FOXO1

Answer 46

Rhabdomyosarcoma alveolar variant

Question 47

Histological findings of fibrous septae dividing tumor cells into clusters resembling lung alveoli indicate?

Answer 47

Rhandomyosarcoma alveolar subtype

Question 48

Which rhanbomyosarcoma variant appears as large, pleomorphic, multinucleated tumor cells with eosinophilic cytoplasm?

Answer 48

Pleomorphic variant

Question 49

Malignant mesenchymal tumor that often arises near joints

Answer 49

Synovial sarcoma

Question 50

t(X;18)(p11;q11) resulting in SS18-SSx1/SSX2/SSX4 fusion genes

Answer 50

Synovial sarcoma

Question 51

What type of soft tissue tumor has monophasic and biphasic morphological types?

Answer 51

Synoval sarcoma

Question 52

Uniform spindle cells in dense fascicles indicate:

Answer 52

Monophasic synovial sarcoma

Question 53

Both spindle cells and gland-like epithelial structures indicate:

Answer 53

Biphasic synovial sarcoma