Bone And Soft Tissue

Front 1

t(X;17)(p11.2;q25) - ASPL/TFE3 fusion gene

Intracytoplasmic PAS+ crystalline inclusions

Spreads to lung via hematogenous route

Back 1

Alveolar soft parts sarcoma

Front 2

t(12;16)(q13;p11) - FUS-ATF1 fusion (M.C.)

t(12;22)(q13;q12) - ATF1-EWSR1

t(2;22)(q33;q12) - CREB1-ESWR1

+) Desmin, EMA, CD99, CD68

-) CD31, CD34, S100, CK

Back 2

Angiomatoid fibrous histiocytoma

Front 3

t(9;22)(q22;q12) - EWSR1/NR4A3 fusion

t(9;17)(q22;q11) TAF15/NRF4A3

Back 3

Extraskeletal myxoid chondrosarcoma

Front 4

t(12;22)(q13;q12) ATF1/EWS

M.C. sarcoma of foot and ankle

In aggressive cases mets to LN, liver, bone, lung in 50%

+) S100, melanocytic markers

Back 4

Clear cell sarcoma

Front 5

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

+) WT1 (carboxy terminus), Desmin, CK, EMA

-) SMA (tumor cells, stroma +)

Aggressive, most die w/in 4 yrs of dx

Back 5

Desmoplastic small round cell tumor

Front 6

t(11;22)(q24;q12) FLI1/EWS

2nd M.C. ERG/EWS

Also subset with FUS (instead of EWS)

Back 6

Ewing’s sarcoma/PNET, extraosseous

Front 7

t(2;13)(q37;q14) PAX3/FOXO1 (FKHR)
t(1;13)(p36;q14) PAX7/FOXO1 (FKHR)

No PAS+ crystalline inclusions

Extremitites > H+N

Diffuse myogenin +

Worst Px

Back 7

Alveolar rhabdomyosarcoma

Front 8

t(X;18)(p11.23;q11) - SYT-SSX1

+) TLE1

Back 8

Synovial sarcoma

Front 9

t(1;2)(p11;q35-37)

CSF1 rearrangements

Back 9

Tenosynovial giant cell tumor

Front 10

Genetic alteration?

Back 10

Front 11

A two year old child had an abdominal mass, which was excised.
Molecular: 1p36.33 deletion, N-myc amplification, 14p deletion

Back 11

Neuroblastoma
Positive: chromogranin, synaptophysin, neurofilament and neuron-specific enolase, as well as vimentin and ALK
negative: keratin, CD45, CD99 and desmin

Front 12

Back 12

Nodular fasciitis
It is highly cellular and composed of plump, immature fibroblasts or myofibroblasts, with a feathery pattern due to the abundance of ground substance. The tumor cells usually have prominent nucleoli. There is often a myxoid stroma, frequent mitotic figures but no atypical forms, a lymphocytic infiltrate and red blood cell extravasation. The vasculature is usually prominent. There may be bands of collagen similar to keloid scars, or metaplastic bone. Rapid growth.

Front 13

The spindle cells had diffuse cytoplasmic immunoreactivity for CD68 and calponin, and were negative for S100 and pan-keratin. CD34 highlighted the blood vessels.

Back 13

Nodular fasciitis

Front 14

Patternless architecture of hypo- and hypercellular areas separated by thick, hyalinized collagen with cracking artifact and staghorn vesselsPerivascular sclerosis

+) CD34, CD99, EMA and Bcl-2 (1/3)

-) Desmin, Keratin, S100, CD117, CD31

NAB2/STAT6 fusion

Back 14

Solitary fibrous tumor

Front 15

variably immunoreactive for smooth muscle actin.

Back 15

Fibrosarcoma

Front 16

Back 16

Benign fibrous histiocytoma: Polymorphous population of histiocytoid and spindled
cells, Touton giant cells and chronic inflammatory
cells.

Front 17

Positive stains: CD34 (strong in 95%), vimentin; also actin (focal), ApoD, bcl2, NKI-C3 (AJCP 1992;97:478), CD99
Negative stains: Factor XIIIa (usually), keratin, EMA, S100, HMB45, desmin, CD117

Back 17

Dermatofibrosarcoma protuberans (DFSP):
prominent storiform pattern of monomorphic fibroblast-like cells that invade into subcutis
Bednar’s tumor: 5-10% of cases; pigmented variant due to dendritic cells with melanin, S100+ only in pigmented cells, HMB45 negative; associated with black patients

Front 18

Spindle cell lesion, M.C. in stomach

DOG1, CD117+

KIT mutation confers sensitivity to TKi

Back 18

GIST

Front 19

Back 19

Myositis ossificans is a benign, ossifying soft-tissue lesion typically occurring within skeletal muscle. Patients are usually adolescents and young adults; myositis ossificans is rare in children under 10 years of age. The most frequent symptoms and signs are pain and tenderness with a soft tissue mass. Approximately 80% of cases arise in the large muscles of the extremities.

Front 20

Positive stains: CD34 (strong in 95%), vimentin; also actin (focal), ApoD ,bcl2, NKI-C3 (AJCP 1992;97:478), CD99

Negative stains: Factor XIIIa (usually), keratin, EMA, S100, HMB45, desmin, CD117

Back 20

Dermatofibrosarcoma protuberans (DFSP)

Front 21

Myxoid liposarcoma

Back 21

t(12;16)(q13;p11) FUS/DDIT3 TLS/CHOP

Front 22

Back 22

Paraganglioma.
Nests of polygonal tumor cells with nuclear atypia and eosinophilic cytoplasm create a Zellballen pattern.

Front 23

Spindle cell lesion

Beta-catenin positive

No mets potential

t(17;22)

CTNNB1 mutation

Can be a/w FAP/Gardner's Syndrome (APC gene)

Back 23

Desmoid fibromatosis

Front 24

High potential for recurrence, no potential for metastasis

MDM2 amplification

Back 24

Atypical lipomatous tumor/well-differentiated liposarcoma

Front 25

• Islands of mature fat
• Poorly organized collagenous fibrous bands with small vessels and inflammatory cells
• Primitive spindle cells and stellate cells arranged in nests within myxoid stroma

Back 25

Fibrous hamartoma of Infancy

Front 26

IDH1 and IDH2

Back 26

Enchondroma/chondroma, chondrosarcoma

Front 27

Highly complex karyotype with high chromosome counts and complex structural rearrangements

Back 27

Pleomorphic liposarcoma

Front 28

Ring or giant marker chromosomes with amplification of 12q13-15 MDM2 (and freq. CDK4)

Back 28

Dedifferentiated Liposarcoma

Front 29

Abberations in chromosome 8

PLAG1

Back 29

Lipoblastoma

Front 30

MDM2

Back 30

ATL/WDLS

Dediff Liposarc

Intimal Sarcoma

Front 31

SDH-deficient

A/w non-hereditary Carney Trida (this, Pulm chondroma, paraganglioma) or Carney-Stratakis syndrome (AD, this, paranganglioma)

Back 31

Epithelioid GIST (of stomach)

Front 32

M.C. soft tissue sarc. in kids and adols

Primitive looking cells in various stages of development

Focal to moderate myogenin IHC

Anaplasia portends poor Px

Back 32

Embryonal Rhabdomyosarcoma

Front 33

t(11;16)(q13;p13) C11orf95/MKL2

Back 33

Chondroid Lipoma

Front 34

ALK (2p23) with TPM3, TPM4, or CLTC

Subset ROS1 instead of ALK

ALK/CLTC: granular cytoplasmic ALK IHC

ALK/RANBP2: paranuclear membranous ALK IHC, epithelioid morphology, poorer px (aggressive bvr and incr. mets)

Back 34

Inflammatory Myofibroblastic Tumor

Front 35

GNAS1

Back 35

• Fibrous dysplasia + cafe au lait + endocrinopathies: McCune Albright Syndrome
  
• Fibrous dysplasia + IM myxomas: Mazabraud Syndrome
• Usu. craniofacial and femur

Front 36

• Classic INDOLENT: Mediterranean/East Europe/Ashkenazi, elderly M, not a/w HIV or EBV
• Endemic African: Kids and adults, not a/w HIV
• Iatrogenic
• AIDS associated: most aggressive bvr
• All forms a/w HHV-8
• No mets potential

Back 36

Kaposi Sarcoma

Front 37

Spindle cell lesions, numberous in multiple tissue planes

+) CD31, ERG, Diffuse AE1/AE3

-) CD34, EMA, Pankeratin MNF116

Retains INI1

t(7;19) SERPINE1/FOSB

Back 37

Pseudomyogenic hemangioendothelioma

Front 38

BCOR-CCNB3

Back 38

Round Cell Sarcoma

Front 39

USP6-CDH11

Back 39

ABC

Nodular Fasciitis

Front 40

Spindle cell lesion

+) CD31, CD34, FLI1, F.VIII, ERG, CD45

t(1;3)(p36;q23-25) CAMTA-WWTR1

Back 40

Epithelioid Hemangioendothelioma

Front 41

Aka myxoid MFH

one of M.C. adult sarcomas

Pleomorphic spindle cells in myxoid to fibrous matrix in varying proportions

Myxoid areas with curvilinear vessels

Back 41

Myxoid Fibrosarcoma

Front 42

Pathognomonic for NF1

Can undergo malignant transformation to MPNST

Back 42

Plexiform Neurofibroma

Front 43

Painful vascular/fatty lesions with fibrin thrombi

Back 43

Angiolipoma

Front 44

Types of Collagen

Back 44

I: Bone, skin

II: Cartilage

III: Reticular Fibers

IV: Basement membrane

Front 45

Wagner-Meissner Bodies

Back 45

Diffuse Neurofibroma

Front 46

• Atrial myxomas
• Endocrine abnormalities
• Pigment d/o
• Psammomatous melanocytic schwanommas

Back 46

Carney Complex

Front 47

• GIST
• Pulmonary chondroma
• Extraadrenal paraganglioma

Back 47

Carney Triad

Front 48

Epithelioid cells surrounded by zone of necrosis

Loss of INI-1 (SMARCB-1)

Back 48

Epithelioid Sarcoma (Distal)

Front 49

t(7;16)(q34;p11) FUS/CREB3L2

MUC4

Back 49

Low-Grade Fibromyxoid Sarcoma/Evan's Tumor

Front 50

Mafucci's Syndrome

Back 50

Multiple enchondromas and (spindle cell) hemangiomas

Front 51

Ollier Disease

Back 51

Multiple enchondromas

Front 52

Epiphyseal Lesions

Back 52

• Chondroblastoma
• Clear cell chondrosarcoma
• Giant cell tumor
• Pigmented villonodular synovitis

Front 53

Metaphyseal Lesions

Back 53

• Chondromyxoid fibroma
• Osteochondroma
• Osteosarcoma
• ABC
• Non-ossifying fibroma
• Chondrosarcoma
• Solitary bone cyst

Front 54

Jaw bones and ribs

Biphasic tumor

Hemangiopericytoma-like vascularity

Back 54

Mesenchymal Chondrosarcoma

Front 55

M.C. malignant bone tumor

Back 55

Osteosarcoma

Front 56

2nd M.C. malignant bone tumor

Back 56

Chondrosarcoma

Front 57

Soap bubble appearance on imaging

Back 57

ABC

Adamantinoma

Front 58

Aka Osteitis Deformans

Polyostotic form: Incr. alk phos; normal ca, phos

Back 58

Paget's Disease of Bone

Front 59

Lytic metastatic bone tumors

Back 59

Kidney

GI

Thyroid

Melanoma

Front 60

Blastic metastatic bone tumors

Back 60

Prostate

Front 61

Blastic AND lytic metastatic bone tumors

Back 61

Breast

Lung

Front 62

4th M.C. primary bone tumor

Back 62

Ewing's Sarcoma

Front 63

• Immature trabeculae
  
• calcific spherules
• cellular spindle stroma

Back 63

Ossifying Fibroma

Front 64

• Aka metaphyseal fibrous defect
• plump spindle cells
• loose storiform stroma
• MNGC
• Foam cells
• Incr MF

Back 64

Non-ossifying fibroma

Front 65

Giant Cell Ddx

Back 65

Non-ossifying fibroma (Metaphysis)

Giant Cell Tumor (Metaphysis to Epiphysis)

Brown Tumor

ABC

Front 66

Herringbone pattern/cellular spindle cell ddx

Back 66

FibrosarcomaSynovial sarcomaMPNSTLeiomyosarcCellular schwannoma

Front 67

Myxoid ddx

Back 67

Benign:

Nodular fasciitis

Myxoma

Nerve sheath tumors: NF, NT, Schwannoma

Malignant:

Extraskeletal myxoid chondrosarc

Myxoid Fibrosarc t(9;22)

Myxoid liposarc

LGFMS t(7;16) CREB/FUS

Front 68

Clean geographic necrosis in sarcomas

Back 68

MPNST

Ewing's/PNET t(11;22) EWS/FLI1, t(21;22) EWS/ERG

Poorly diff SS

Front 69

Pseudogranulomas

Back 69

Granuloma Annulare

Rheumatoid Nodule

Epithelioid Sarcoma

NL?

Front 70

Genetic alteration?

Back 70

USP6-CDH11

ABC

Front 71

Genetic alteration?

Back 71

C11orf95-MKL2

Chondroid lipoma

Front 72

Fusion?

Back 72

USP6/MYH9

Nodular Fasciitis

Front 73

59 to M, posterior neck mass. Chromosomal abnormalities?

Back 73

13q14 abnormalities

Spindle cell/pleomorphic lipoma

Front 74

31 yo M, ankle pain. IHC + S100, HMB45; - desmin, CD34, pan-keratin, EMA. Fusion?

Back 74

EWSR1-ATF1

Clear cell sarcoma

Front 75

57 yo F, pleural based mass. Positive IHC?

Back 75

STAT6

SFT

Front 76

Newborn with posterior shoulder mass.

Back 76

Fibrous hamartoma of infancy

Front 77

Genetic alteration?

Back 77

GNAS

Front 78

Back 78

Elastofibroma

Front 79

46 yo F, IHC pos for CD31 and ERG, focal CK. What is this IHC?

Back 79

CAMTA1

Epithelioid Hemangioendothelioma

Front 80

EWSR1-NR4A3 fusion

Back 80

Extraskeletal myxoid chondrosarcoma

Front 81

Most important predictor of unfavorable outcome?

Back 81

Round cell compoinent >5%

Myxoid liposarcoma

Front 82

Back 82

Calcifying aponeurotic fibroma

Front 83

t(x;18). IHC?

Back 83

TLE1

Front 84

Pos for CD34. What would confirm DFSP?

Back 84

t(17;22)(q21;q13) COL1A1-PDGFB

Front 85

Back 85

Myxofibrosarcoma

Front 86

Pos for CK, Desmin. Neg for S100, CD99. EWSR1 gene rearrangement. Dx?

Back 86

DSRCT

Front 87

Focal pos SMA, pos nuclear B-catenin. Mutation in most sporadic tumors?

Back 87

CTNNB1

Desmoid Fibromatosus

Front 88

Pos for HMB45, Mart1, S100; Neg for SMA, desmin, TFE3, CD34, Pankeratin, EMA. Genetic alteration?

Back 88

t(12;22)(q13;q12) EWSR1/ATF1

Clear cell sarcoma

Front 89

47 yo M, HIV Pos. What is IHC or ISH?

Back 89

HHV8

Kaposi Sarcoma

Front 90

Pos CD34, CD31; Neg MDM2, HHV8, desmin, myogenin, S100, CK. Dx?

Back 90

Angiosarcoma

Front 91

Pos KIT and DOG1; Neg CK, SMA, S100. Genetic alteration?

Back 91

SDH-deficient, SDHB gene mutation

Epithelioid GIST

Front 92

Back 92

Hibernoma

Front 93

t(7;16). Confirmatory IHC?

Back 93

MUC4

LGFMS/Evan's tumor

Front 94

Painful nodule on digit. Pos for SMA, caldesmon; Neg for PanCK, EMA, S100, Mart1, HMB45. Dx?

Back 94

Glomus Tumor

Front 95

s/p chemo. Most important Px indicator?

Back 95

>90% post-chemo necrosis

Osteosarcoma