Soft Tissue Tumors

Front 1

SMA/MSA, focal CD34 in fibroblasts

Back 1

Fibroblastic differentiation

Front 2

S100 (fat, lipoblasts), CD34 (spindled areas)

Back 2

Lipomatous differentiation

Front 3

CD31, CD34, SMA (pericytes), HHV8 (KS)

Back 3

Vascular differentiation

Front 4

S100, CD34, NFP, GFAP, EMA

Back 4

Nerve sheath differentiation

Front 5

Desmin, MyoD1 (nuclear), myogenin, myoglobin

Back 5

Skeletal muscle differentiation

Front 6

SMA, MSA, desmin

Back 6

Smooth muscle differentiation

Front 7

PAS crystals, no consistent IHC

Back 7

Alveolar soft part sarcoma

Front 8

Keratin, CD34, EMA

Back 8

Epitheliloid sarcoma

Front 9

Keratin, CK7, CK19, CD99, Bcl-2, EMA

Back 9

Synovial sarcoma

Front 10

HMB45 > S100, melan A, MITF (microphtalmia transcription factor), CD57, c-kit

Back 10

Clear cell sarcoma

Front 11

Translocation(s) in Ewing sarcoma?

Back 11

t(11;22)(q24;q11.2-12)
11 = FLI-1
22 = EWS

t(21;22)
21 = ERG
22 = EWS

Front 12

t(11;22)? (2)

Back 12

Ewing sarcoma
t(11;22)(q24;q11.2-12)
11 = FLI-1
22 = EWS

Desmoplastic small round cell tumor
t(11;22)(p13;q12)
11 = WT1
22 = EWS

Front 13

t(21;22)?

Back 13

Ewing sarcoma
21 = ERG
22 = EWS

Front 14

t(12;22)? (2)

Back 14

Clear cell sarcoma
12 = ATF1
22 = EWS

Myxoid/round cell liposarcoma
12 = CHOP = DDIT3
22 = EWS

Note: ATF1 and CHOP both map to 12q13

Front 15

Translocation in desmoplastic small round cell tumor?

Back 15

t(11;22)(p13;q12)
11 = WT1
22 = EWS

Front 16

Translocation in clear cell sarcoma?

Back 16

t(12;22)
12 = ATF1
22 = EWS

Front 17

t(12;16)?

Back 17

Myxoid/round cell liposarcoma
12 = CHOP = DDIT3
16 = FUS = TLS

Front 18

Translocation(s) in myxoid/round cell liposarcoma?

Back 18

t(12;16)
12 = CHOP = DDIT3
16 = FUS = TLS

t(12;22)
12 = CHOP = DDIT3
22 = EWS

Front 19

Translocation in alveolar soft part sarcoma?

Back 19

t(X;17)
X = TFE3
17 = ASPL

Front 20

t(X;17)?

Back 20

Alveolar soft part sarcoma
X = TFE3
17 = ASPL

Front 21

Translocation in synovial sarcoma?

Back 21

t(X;18)
X = SSX1, SSX2, or SSX4
18 = SYT

Front 22

t(X;18)?

Back 22

Synovial sarcoma
X = SSX1, SSX2, or SSX4
18 = SYT

Front 23

Translocation(s) in rhabdomyosarcoma, alveolar type?

Back 23

t(1;13)
1 = PAX7
13 = FKHR

t(2;13)
2 = PAX3
13 = FKHR

Front 24

t(1;13)?

Back 24

Rhabdomyosarcoma, Alveolar type
1 = PAX7
13 = FKHR

Front 25

t(2;13)?

Back 25

Rhabdomyosarcoma, Alveolar type
2 = PAX3
13 = FKHR

Front 26

Translocation in dermatofibrosarcoma protuberans?

Back 26

t(17;22)
17 = COL1A1 (collagen type 1)
22 = PDGF-beta (platelet derived growth factor)

Front 27

t(17;22)

Back 27

Dermatofibrosarcoma proturberans
17 = COL1A1 (collagen type 1)
22 = PDGF-beta (platelet derived growth factor)

Front 28

Translocation in low grade fibromyxoid sarcoma?

Back 28

t(7;16)
7 = CREB
16 = FUS

Front 29

t(7;16)?

Back 29

low grade fibromyxoid sarcoma
7 = CREB
16 = FUS

Front 30

This sarcoma typically presents as single or multiple nodular, sometimes ulcerated, lesions involving the fingers, hands, and forearms of young adults (especially males). Histologically, it is recognized by its characteristic nodular pattern of growth, central cystic degeneration, and epithelioid cells with eosinophilic cytoplasm.

Back 30

Epithelioid sarcoma

Front 31

Typically affecting the shoulder or axillary region of male infants, this lesion displays 3 components: adipose tissue, some of which has tiny cytoplasmic droplets (brown fat); haphazardly placed paucicellular fascicles of spindle cells with abundant collagen; primitive-appearing rounded to spindled cells set in pale amphophilic stroma. There is no necrosis or mitoses.

Back 31

Fibrous hamartoma of infancy

Front 32

This benign tumor of infancy occurs exclusively on the fingers and toes. It consists of a dermal infiltrate of uniform, plump, spindled cells in a collagenous stroma. The spindled cells have a fascicular growth pattern. Mitoses can be prominent. There are numerous small round eosinophilic cytoplasmic inclusions within the spindle cells which stain deep red on Masson trichrome.

Back 32

Infantile digital fibrosis

Front 33

Which sarcoma tends to metastasize to other soft tissue sites rather than to lungs?

Back 33

Myxoid liposarcoma

Front 34

What is the classical location of spindle cell lipoma?

Back 34

Neck or shoulder

Front 35

t(9;22)(q22-31; q11-12)?

Note: this is not BCR-ABL

Back 35

Extraskeletal chondrosarcoma
9 = CHN
22 = EWS

Note: skeletal chondrosarcoma doesn't have this translocation

Front 36

Translocation in extraskeletal chondrosarcoma?

Back 36

t(9;22)
9 = CHN
22 = EWS

Front 37

Occurring most commonly in the upper back, this benign tumor is composed of three types of cells: mulberry cell (multivacuolated adipocyte with a centrally placed round nucleus); cells with granular (rather than vacuolated) cytoplasm, and mature adipocytes.

Back 37

hibernoma

Front 38

This painful skin nodule tends to present in the legs and feet of females. Histologically, the tumor is composed of smooth muscle bundles that appear to originate from medium sized vessels.

Back 38

Vascular leiomyoma

Front 39

What does ANGEL stand for?

Back 39

Angiolipoma
Neuroma (traumatic)
Glomus tumor
Eccrine spiradenoma
Leiomyoma (vascular)

ANGEL is the acronym for painful skin nodules

Front 40

In the setting of HIV, what virus is implicated in leiomyosarcoma?

Back 40

EBV

Front 41

What are the most common demographic and sites for clear cell sarcoma of soft parts?

Back 41

Demographic: 2nd-4th decades

Sites: Foot and ankle

Front 42

What's the most common demographic and location for desmoplastic small round cell tumor?

Back 42

Demographic: Young men (average age =22)

Site: Abdominal

Front 43

What is the proliferative cell in nodular fasciitis?

Back 43

Myofibroblasts

Front 44

What is the typical demographic and location for nodular fasciitis?

Back 44

Demographic: 2nd to 5th decades

Site: Forearm

Other sites include upper extremity (shoulder), head and neck, and thigh.

Front 45

What is the most common site of nodular fasciitis in children and infants?

Back 45

Head and neck

Front 46

Is nodular fasciitis usually deep or superficial?

Back 46

Superficial (subcutaneous)

Front 47

After local excision, is nodular fasciitis prone to recur?

Back 47

No, local excision is usually curative though recurrences occur extremely rarely.

Front 48

Is nodular fasciitis encapsulated?

Back 48

No

Front 49

This lesion has a "tissue culture" appearance, numerous mitoses (but no atypical forms), extravasated RBC in the stroma, mononuclear inflammatory cells and sparce multinucleated giant cells. The proliferative cells are positive for SMA with rare desmin positive cells. CD68 highlight histiocytes.

Back 49

Nodular fasciitis

Front 50

This lesion which tends to occur between the 3rd and 5th decades involving the extremities, is slowly growing, presents as slightly raised and pigmented cutaneous lesion centered in the dermis with associated epidermal hyperplasia. Histologically, the lesion is arranged in a storiform pattern or in short fascicles and is composed of spindled to oval fibroblasts and round to oval histiocytoid cells. The lesion also contains multinucleated, osteoclast-like giant cells; xanthoma cells may be seen and may be prominent. The stroma can be myxoid or densely fibrotic with a sparse chronic inflammatory infiltrate as well as focal hemosiderin pigment.

Back 50

Fibrous histiocytoma

Front 51

Is extraskeletal myxoid chondrosarcoma usually deep or superficial?

Back 51

Deep

Front 52

Is extraskeletal myxoid chondrosarcoma usually well-circumscribed or infiltrative? Is there a capsule?

Back 52

Extraskeletal myxoid chondrosarcoma is usually well-circumscribed surrounded by a fibrous pseudocapsule.

Front 53

Histologically, this tumor which is most commonly located in skeletal muscle, is multinodular with abundant myxoid matrix; the tumor cells are arranged in cords and clusters, frequently oriented radially. The tumor cells are round to stellate with eosinophilic cytoplasm, round and hyperchromatic nuclei, and sparse mitotic activity.

Back 53

Extraskeletal myxoid chondrosarcoma

Front 54

What demographic is most commonly affected with dermatofibrosarcoma protuberans?

Back 54

Middle-aged males, African American predominance

Front 55

What are the clinical characteristics of dermatofibrosarcoma protuberans?

Back 55

Most cases are slow and progressive with a plaque-like phase followed by a nodular phase where the overlying epidermis becomes thinned and ulcerated.

Front 56

The cut surface of this tumor is gray-white and the tumor involves the dermis and subcutis, sometimes extending to the underlying muscle. There is entrapped dermal collagen and appendages. The overlying epithelium is thinned or ulcerated. The tumor is cellular and composed of uniform, mildly atypical fibroblasts arranged in a monotonous, tight and repetitive storiform pattern.

Back 56

Dermatofibrosarcoma protuberans

Front 57

What is a dermatofibrosarcoma protuberans with malanocytic differentiation (i.e. S100+, melan A+, HMB-45+) called?

Back 57

Bednar tumor

Front 58

What is the juvenile variant of dermatofibrosarcoma protuberans called?

Back 58

Giant cell fibroblastoma

Front 59

What is the IHC for dermatofibrosarcoma protuberans?
CD34
S-100
SMA
Desmin
Keratins
EMA
CD99
Factor 13A
c-kit

Back 59

Dermatofibrosarcoma protuberans
CD34: +
S-100: -
SMA: -
Desmin: -
Keratins: -
EMA: -
CD99: +
Factor 13A: -
c-kit: -

Front 60

What is the age of peak incidence for well-differentiated liposarcoma?

Back 60

6th to 7th decades

Front 61

What location is most commonly affected by well-differentiated liposarcoma?

Back 61

Most common: deep muscles of extremities.

Less common: retroperitoeneum and contiguous areas (inguinal canal and paratesticular region).

Front 62

What are the proliferative cells in malignant fibrous histiocytoma?

Back 62

fibroblastic and myofibroblastic cells

Front 63

IHC of malignant fibrous histiocytoma?
Vimentin
SMA
CK
S100
CD45
CD68

Back 63

Malignant Fibrous Histiocytoma:
Vimentin: +
SMA: +
CK: -
S100: -
CD45: -
CD68: +

Front 64

What is the peak age-range for malignant fibrous histiocytoma?

Back 64

6th to 7th decades.

Note: MFH is rare before age 40.

Front 65

What is the most commonly affected site of malignant fibrous histiocytoma?

Back 65

Thighs

Front 66

What is the most common sarcoma arising after radiation therapy?

Back 66

Malignant fibrous histiocytoma

Front 67

What are the 4 types of malignant fibrous histiocytoma? What are the 2 most common?

Back 67

Most to least common:
1. pelomorphic-storiform type
2. myxoid variant (>50% myxoid)

Less common:
- Inflammaotry: when inflammatory component becomes prominent, including abundant neutrophils
- Giant cell variant: when the giant cells become a predominant element

Front 68

Name the 4 most common sarcomas.

Back 68

1. MFH
2. Liposarcoma
3. Rhabdomyoarcoma
4. Synovial sarcoma

Front 69

What's the typical demographic affected by synovial sarcoma?

Back 69

Young adults (median age 30-35), but can occur in children and older people.

Front 70

What is the most common site of involvement with synovial sarcoma?

Back 70

Synovial Sarcoma

Most common:
Extremities (60% lower, 20% upper), typically arises in the proximity of large joints

Less common:
- Trunk
- head and neck

Front 71

Is synovial sarcoma typically fast or slow growing?

Back 71

Synovial sarcoma presents as a slow growing swelling, sometimes present for years. Poorly differentiated cases may present directly with metastases though.

Front 72

What are the 3 microscopic types of synovial sarcoma list from most to least common.

Back 72

1. Monophasic (~66%)
2. Biphasic (20-30%)
3. Poorly differentiated (least common)

Front 73

What are the 3 types of rhabdomyosarcoma?

Back 73

- Embryonal: includes botryoid, spindle cell, and anaplastic variants
- Alveolar: only type with a translocation
- Pleomorphic

Front 74

Cambium layer, "strap cells", and rhabdomyoblasts characterize what lesion?

Back 74

Embryonal rhabdomyosarcoma, botryoid variant

Front 75

What are the first and second most common malignant soft tissue tumor of children?

Back 75

1. Rhabdomyosarcoma
2. Ewings/PNET

Front 76

What is the most common variant of rahbdomyosarcoma?

Back 76

Embryonal rhabdomyosarcoma

Front 77

What are the most common sites for embyronal rhabdomyosarcoma? (3)

Back 77

Genitogurinary, retroperitoneal, head and neck

Front 78

What is the most common site for embryonal rhabdomyosarcoma, spindle cell variant?

Back 78

paratesticular

Front 79

What demographic is affected by alveolar rhabdomyosarcoma and what are the 2 most common site of involvement?

Back 79

Demographic: Older children (than embryonal rhabdomyosarcoma)

Sites: extremities, head/neck

Front 80

What demographic is most commonly affected with pleomorphic rhabdomyosarcoma? What are the most common sites?

Back 80

Pleomorphic Rhabdomyosarcoma:
Demographic: Elderly

Sites: trunk, extremities

Front 81

Which is the most common type of rhabdomyosarcoma?

Back 81

Embryonal RMS (75% of cases)

Front 82

Alveolar rhabdomyosarcoma tends to have what type of giant cells?

Back 82

wreath-like giant cells

Front 83

IHC for rhabdomyosarcoma:
Desmin
Actin
Myoglobin
Myogenin
Myo D1 (nuclear)
CK
NFP
NSE
Leu7
S100
CD99
CD10
CD19
CD20

Back 83

Rhabdomyosarcoma:
Desmin: +
Actin: +
Myoglobin: +
Myogenin: +
Myo D1 (nuclear): +
CK: +/-
NFP: +/-
NSE: +/-
Leu7: +/-
S100: +/-
CD99: +/-
CD10: +/-
CD19: +/-
CD20: +/-

Front 84

This rare variant of schwannoma contains melanin pigment and can be associated with Carney complex.

Back 84

Psammomatous melanotic schwannoma

Front 85

What is the EM finding in clear cell sarcoma?

Back 85

pre-mlanosomes

Front 86

The histology of this tumor consists of uniform cells arranged in small clusters, nests, and fascicles surrounded by delicate fibrous septa. The tumor cells have clear to eosinophilic cytoplasm, and uniform round to oval nuclei with vesicular chromatin and large nucleoli.

Back 86

Clear cell sarcoma

Front 87

What is the demographic affected by granular cell tumor?

Back 87

Middle-aged (4th-6th decade), predilection for African-Americans

Front 88

What % of granular cell tumors are malignant?

Back 88

2%

Front 89

What is the most common soft tissue site affected by granular cell tumor?

Back 89

Hand

Front 90

How do the granules in granular cell tumor stain with PAS-D?

What are the granules made of?

Back 90

PAS-D: +

Granules: phagolysosomes

Front 91

Typical age group affected by Ewing/PNET?

Which ethnic group is usually spared of Ewing/PNET?

Back 91

Affected group:
Adolescents, young adults

Spared group:
African Americans

Front 92

Typical soft tissue sites for Ewing/PNET are? (4)

Back 92

1. Paravertebral area
2. Retroperitonum
3. Chest wall
4. Extremities

Front 93

What is the demographic which alveolar soft part sarcoma affects?

Back 93

Children and young adults

Front 94

Is alveolar soft part sarcoma fast or slow growing?

Back 94

Slow

Front 95

Dermatofibrosarcoma protuberans is to t(17;22)

as

Giant cell fibroblastoma is to _______?

Back 95

ring chromosomes 17 and 22

Front 96

The most common type of angiosarcoma is???

Back 96

Cutaneous angiosarcoma not associated with lymphedema

Front 97

The typical demographic affected by angiosarcoma is???

Back 97

Elderly with sun-damage

Front 98

3 positive IHC for angiosarcoma?

Back 98

CD31, CD34, Factor 8

Front 99

IHC for epithelioid hemangioendothelioma:
CD31
CD34
Factor 8
CK7
CK8
CK18
CK19

Back 99

Epithelioid Hemangioendothelioma:
CD31: + (diffuse)
CD34: +/-
Factor 8: +/-
CK7: +/- (focal)
CK8: +/- (focal)
CK18: +/- (focal)
CK19: -

Front 100

Are soft tissue epithelioid hemangioendotheliomas usually painful or not?

Solitary or multiple?

Superficial or deep?

Back 100

Painful

Solitary

Both superficial and deep

Front 101

IHC for desmoplastic small round cell tumor?
Desmin
CK
EMA
WT1
CD99
NSE

Back 101

Desmoplastic small round cell tumor:
Desmin +
CK +
EMA +
WT1 +
CD99 +
NSE +

Front 102

Inflammatory myofibroblastic tumor is associated with what mutation?

Back 102

ALK-1 on chromosome 2.

Note: 60% of IMT are ALK+ by IHC.

Front 103

This lesion can affect people of all ages but has a predilection for young children (average age 10) and may present with systemic symptoms. Abnormal lab values include anemia, elevated ESR, thrombocytosis, and hypergammaglobulinemia. Microscopically, the lesion is characterized by 3 possible patterns: myxoid spindle cell lesion w/ tissue-culture-like appearance; cellular with fascicular-storiform archiecture; or spindle cells in a dense fibrous stromal background. The spindle cells may express ALK-1 by IHC.

Back 103

Inflammatory myofibroblastic tumor

Front 104

Solitary fibrous tumor IHC? (3)

Back 104

CD34, CD99, Bcl2

Front 105

What is Doege-Potter syndrome?

Back 105

Hypoglycemia associated with solitary fibrous tumor.

Insulin-like growth factor is implicated. This only occurs in 4% of SFT. This is usually associated with larger tumors with higher mitotic rates.