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

Epithelioid sarcoma

Front 9

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

Back 9

Synovial sarcoma

Best stain: TLE-1

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

single or multiple nodular, sometimes ulcerated, lesions on the fingers, hands, and forearms of young adults (especially males).

Micro: nodular pattern of growth, central cystic degeneration, and epithelioid cells with eosinophilic cytoplasm

Back 30

Epithelioid sarcoma

Front 31

shoulder or axillary region of male infants

3 components: adipose tissue, some of which may be brown fat; haphazardly placed paucicellular fascicles of spindle cells with abundant collagen; primitive-appearing rounded to spindled cells set in pale amphophilic stroma

no necrosis or mitoses

Back 31

Fibrous hamartoma of infancy

Front 32

infant with tumor on fingers or toes

mitotically active dermal infiltrate of uniform, plump, spindled cells in a collagenous stroma with a fascicular growth pattern

small round eosinophilic cytoplasmic inclusions within the spindle cells staining 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

tumor most commonly in the upper back with 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

painful skin nodule in the legs and feet of females

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

"tissue culture" appearance, numerous mitoses (but no atypical forms), extravasated RBC in the stroma, mononuclear inflammatory cells and sparse multinucleated giant cells

SMA+, typically desmin - but can be rare +

Back 49

Nodular fasciitis

Front 50

3rd and 5th decades, extremities, is slowly growing slightly raised and pigmented cutaneous lesion centered in the dermis with associated epidermal hyperplasia; spindled to oval fibroblasts and round to oval histiocytoid cells in a storiform pattern or in short fascicles; also has multinucleated, osteoclast-like giant cells or xanthoma cells; can have 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

most commonly located in skeletal muscle

multinodular with abundant myxoid matrix; the tumor cells are arranged in cords and clusters, frequently oriented radially

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

cut surface is gray-white and involves the dermis and subcutis, sometimes extending to the underlying muscle; entrapped dermal collagen and appendages; overlying epithelium is thinned or ulcerated

micro: 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's the typical demographic affected by synovial sarcoma?

Back 62

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

Front 63

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

Back 63

Synovial Sarcoma

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

Less common:
- Trunk
- head and neck

Front 64

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

Back 64

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

Front 65

What are the 3 types of rhabdomyosarcoma?

Back 65

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

Front 66

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

Back 66

Embryonal rhabdomyosarcoma, botryoid variant

Front 67

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

Back 67

1. Rhabdomyosarcoma
2. Ewings/PNET

Front 68

What is the most common variant of rahbdomyosarcoma?

Back 68

Embryonal rhabdomyosarcoma

Front 69

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

Back 69

Genitogurinary, retroperitoneal, head and neck

Front 70

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

Back 70

paratesticular

Front 71

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

Back 71

Demographic: Older children (than embryonal rhabdomyosarcoma)

Sites: extremities, head/neck

Front 72

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

Back 72

Pleomorphic Rhabdomyosarcoma:
Demographic: Elderly

Sites: trunk, extremities

Front 73

Which is the most common type of rhabdomyosarcoma?

Back 73

Embryonal RMS (75% of cases)

Front 74

Alveolar rhabdomyosarcoma tends to have what type of giant cells?

Back 74

wreath-like giant cells

Front 75

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

Back 75

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

Front 76

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

Back 76

Psammomatous melanotic schwannoma

Front 77

What is the EM finding in clear cell sarcoma?

Back 77

pre-melanosomes

Front 78

uniform cells arranged in small clusters, nests, and fascicles surrounded by delicate fibrous septa; cells have clear to eosinophilic cytoplasm, and uniform round to oval nuclei with vesicular chromatin and large nucleoli

Back 78

Clear cell sarcoma

Front 79

What is the demographic affected by granular cell tumor?

Back 79

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

Front 80

What % of granular cell tumors are malignant?

Back 80

2%

Front 81

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

Back 81

Hand

Front 82

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

What are the granules made of?

Back 82

PAS-D: +

Granules: phagolysosomes

Front 83

Typical age group affected by Ewing/PNET?

Which ethnic group is usually spared of Ewing/PNET?

Back 83

Affected group:
Adolescents, young adults

Spared group:
African Americans

Front 84

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

Back 84

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

Front 85

What is the demographic which alveolar soft part sarcoma affects?

Back 85

Children and young adults

Front 86

Is alveolar soft part sarcoma fast or slow growing?

Back 86

Slow

Front 87

Dermatofibrosarcoma protuberans is to t(17;22)

as

Giant cell fibroblastoma is to _______?

Back 87

ring chromosomes 17 and 22

Front 88

The most common type of angiosarcoma is???

Back 88

Cutaneous angiosarcoma not associated with lymphedema

Front 89

The typical demographic affected by angiosarcoma is???

Back 89

Elderly with sun-damage

Front 90

3 positive IHC for angiosarcoma?

Back 90

CD31, CD34, Factor 8

Front 91

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

Back 91

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

Front 92

Are soft tissue epithelioid hemangioendotheliomas usually painful or not?

Solitary or multiple?

Superficial or deep?

Back 92

Painful

Solitary

Both superficial and deep

Front 93

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

Back 93

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

Front 94

Inflammatory myofibroblastic tumor is associated with what mutation?

Back 94

ALK-1 on chromosome 2.

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

Front 95

predilection for young children (average age 10) but can affect people of all ages;
may present with systemic symptoms, anemia, elevated ESR, thrombocytosis, and hypergammaglobulinemia

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

may express ALK-1

Back 95

Inflammatory myofibroblastic tumor

Front 96

Solitary fibrous tumor IHC? (3)

Back 96

CD34, CD99, Bcl2

Front 97

What is Doege-Potter syndrome?

Back 97

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.

Front 98

Well circumscribed lesion in young person where LNs are usually found

Micro: Thick fibrous pseudocapsule with prominent lymphoid infiltrate and a center with blood-filled spaces

Back 98

Angiomatoid fibrous histiocytoma

Thought to arise from myoid cells of lymphoid tissue

Front 99

MC malignant solid tumor in kids <1 year

Back 99

Neuroblastoma

Front 100

Homovanillic acid

Vanillylmandelic acid

Back 100

Urine catecholemines helpful in diagnosis of neuroblastoma

Front 101

Abdominal mass in kid

Micro: sheets of primitive neural cells with rosettes, dark nucleii, scant cytoplasm, poorly defined cell boundaries, mitotic activity, karyorrhexis, and pleomorphism; matrix is neuropil

Back 101

Neuroblastoma

(can have "maturation" into ganglioneuroblastoma or ganglioneuroma)

Front 102

Molecular: neuroblastoma

Back 102

Loss of large portion of chrom 1

Amplification of n-myc on chrom 2

Hyperdiploid do better

Front 103

Poor prognosis in neuroblastoma

Back 103

High mitosis-karyorrhexis index (MKI)

Older age (over 5 years)

Front 104

Infant/young child with multiple cutaneous lesions, +/- involvement of deep soft tissues, bone and visceral organs

biphasic with fascicular periphery of interlacing bundles of myofibroblastic cells and a hemangiopericytoma-like center

Back 104

myofibromatosis

Front 105

dermal-based lesion underlying a thinned epidermis, composed of bland mononuclear cells with ovoid nucleii with dispersed chromatin and eosinophilic to vacuolated cytoplasm, interspersed with Touton-type giant cells with nucleii in ring-like distribution

Back 105

Juvenile xanthogranuloma

Front 106

Major differential for juvenile xanthogranuloma

Back 106

LCH: CD1a +

Front 107

Woman of reproductive age with large soft tissue mass in pelvis/perineum and extension to genitalia

Infiltrative margins, small spindled to stellate cells in loose matrix with variable collagen and open vascular channels

Back 107

Aggressive angiomyxoma

Front 108

Woman with small well-circumscribed lesion in vulva with delicate vascular pattern and tumor cell crowding around vessels

Back 108

Angiomyofibroblastoma