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Actual Soft Tissue Flashcards

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1
Q

T/F Lipoblasts have fat that indents the nucleus?

Pitfall of MDM2 and CDK4 in atypical lipomatous tumor?

A

True! Normal adipose cells lack nuclear indentaiton

Stains histeocytes (can make it appear like a lipsarcoma)!

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2
Q

Clinical presentation of Spindle cell lipoma?
H and E?
IHC?

A

Middle age men on back, neck, and shoulder

Ropey collegen, blunt cells, palasading, floret cells admixed with fat

CD34+, loss Rb (13q), lacks MDM2/CDK4 (seen in atypical lipomatous stain)

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3
Q

Mammary type myofibroblastoma H and E?

Stains?

Genetics?

A

In milk line, spindle cell lipoma and cellular angiofibroma

Well circumscribed bland spindle cells in fasicles, Mast cells

CD34+, Desmin +

Loss Rb (13q 16q)

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4
Q

Well diff liposarcoma/atypical lipomatous tumor stain/genetics?

H and E; tx??

Dedifferentiated liposarcoma genetics?

A

MDM2/ CDX4 (12q amplification; FISH Giant chromosomes); p16+ (66%)

No lipoblasts in background but expanded fibrous with atypical cells in septate; excision

1p/16q (JUN, ASK1); more atypical with more mites

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5
Q

Myxoid liposarcoma body location, H and E?

Genes?

High grade is called?

A

Young adults, extremeties, Lipoblasts and spindle cells with myxoid stroma

t(12;16) DDIT3-CHOP 95%

t(12;22) DDIT3-EWSR1 5%

Round cell (back to back round cells back to back with high NC ratio)

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6
Q

Pleomorphic liposarcoma population and location?

H and E?

Genes?

A

Extremetires of elderlies

Has lipoblasts and round/spindle cell, UGLY

None, complex phenotype

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7
Q

Heart lesion, perinatal, with “Spider cells”, think?

Embryonal Rhabdomyosarcoma, age, location?

H and E?

IHC Staining?

A

Tuberus sclerosis with glycogenated cells!

Kids, Head and neck and GU (botryoid)
Small blue cells in darka nd light areas, Cambrium layer

MYOD1>myogenin

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8
Q

Alveolar Rhabdomyosarcoma, patient population?

H and E?

Translocaiton?

IHC?

A

Kids, extremeties
Lobulated growth of roundish cells with nuclei and septae

t(2;13) PAX3, FOXO1 , or Chr 1, 2, 13 (bad prognosis if translocation)

Myogenin >MyoD+

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9
Q

Desmoplastic SRBCT, patient population?

H and E?
Stains?

Translocation?

A

Usually intraabdominal teenage males

Nests of ugly small blue cells

IHC: Keratin +, EMA+, Desmin dot like, NSE+, WT-1+ (carboxy term nuc + or amino term perinuclear +)

t(11;22) EWSR1-WT1 poor prognosis

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10
Q

Pleomorphic Rhabdomyosarcoma Populatin?
H and E?
MDM2 status?

A

Older adults, extremitis
Spindled to epithelial cells, rare striations, large cells with pink cytoplasm

MDM2 nuclear expression WITHOUT amplification (vs liposarc)

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11
Q

Spindle cell/sclerosisng rhabdomyosarcoma population,

H and E?

IHC?

Prognosis?

A

Adults and kids (paratesticular)

Spindled cells, pale eosinophilic cytoplasm, rare striations, stromal hylanization possible

IHC: Desmin +/-, myogenin in rhabomyoblasts, +/0 in spindle cells

Good prognosis in kids, bad in adults

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12
Q

Glomus tumor, population?

H and E?

IHC/Genes?

A

Young adults, distal extermeties, Painful

Uniform round cells (fired eg), Distinct boarders,

SMA+, Caldesmin +

NF1 or GLMN (glomulin)

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13
Q

Glomangioma?

Glomangiomyoma?

Glomangiopericytoma?

Symplastic?

A

All associated with some fried egg cells
Glomangiomoma: Cavernous vessesls

  • Angiomyoma: Spindled smooth muscle like cells
  • angiopericytoma: HPC like vessels

Symplastic: random nuclear atypia

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14
Q

Infantile hemangia patient population?

H and E?

Gene?

A

Young females, post natal arise and then shrinks
Flat–> elevated lesion
Vaso formation present

GLUT1+ (NEGATIVE IN OTHER HEMANGIOMAS)

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15
Q

Epithelioid hemangioma?

H and E?

Stains?

Differs from EHE by?

A

Eosinophils and vessels

Capillaries sized vessels lined by plump cells, large grooved nuclei

CD31>CD34, ERG+

Is vasoformative, no/rare intracytoplasmic lumina

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16
Q

Epithelioid Hemangioendothelioma H and E?

Can it metastasize?

IHC?

Translocation?

A

Angiocentric, epithelioid cells in myxoid stroma, EOSONOPHILIC CYTO WITH VACUOLES (RBCs)

Not vasoformative vs angiosarc which is vasoformative

Yes, death in up to 15%

CD34+, CD31+, FLI1+, ERG+, Keratin in half

t(1;3) WWTR1-CAMTA

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17
Q
A
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18
Q

Angiosarcoma in beast H and E?

Radiation related H and E?
Gene?

A

20-30 year old females, BLAND endometrial cells; deeply INFILTRATIVE

Cutaneous, vasoformative

MYC (8q24) >90% or FLT4 ~25%

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19
Q

Kaposi H and E?

Virus?

A

Various presentation: Vascular spaces with dissecting collagen, spindle cells, extravasated RBCs/hemosiderin

HHV8

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20
Q

Schwanoma H and E?

IHC’s?

Associated with?

What is melanocytic Schwannoma?

A

Antoni A and B areas

S100+, Collagen IV/Laminin around individual cells, AE1/AE3 + (AE3 is GFAP cross reactive), NF+

NF2 and Schwannomatosis (SMARCB1)

Young women, HMB45, MelanA +, half with psammoma bodies, MAY METASTASIS, Carney Complex

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21
Q

Neurofibromas associated with?

Perineurioma H and E?
IHC?

Study These Flashcards
A

NF1 (17q) and plexiform

Storiform with long fsicles, collagenous myxoid stroma

EMA+, Claudin1+, GLUT1+, CD34+, S100-

22
Q

MPNST H and E?

IHC?

Study These Flashcards
A

Conventional: Spindled fasicles with marbling, HPC like blood vessels, heterologous elevements including skeletal muscle (Triton–poor prognosis)

~50% S100+, SOX10+

Epithelioid: from Schwannoma (not NF1)

S100+ (strong), possible keratin +, INI1 loss 50%

23
Q

Nodular fascitis classic history?

H and E?

Genes?

Tx?

Study These Flashcards
A

Young adults with possible trauma but rapid growth

Well circumscribed or infiltrative, Tissue culture myofibroblassts, myxoid stroma,

SMA+

MYH9-USP6 (22q-17p)

Excision, should not recur

24
Q

Desmoid Fibromatosis, common clinical presentation?

H and E?

Genes?

Study These Flashcards
A

Pregnant/peripartem or Gardner’s

Long fasicles of bland spindle cells in collagenous stroma with prominent vessels, Vesicular chromatin

SMA+, B-catenin+

Gardner: APC, Sporatic; B-catenin

25
Low grade fibromyxoid sarcoma (Hylanizing spindle cell tumor with giant rosettes), population? H and E? IHC? Gene rearrangements? Association?
**Low grade, trunk/extremities young adults** Well circumscribed, discrete myxoid/collagenous mass, variable cellular, minimal atypia. **EMA+, MUC4+ t(7;16)FUS-CREB3L2** A/W: Sclerosing epithelial fibrosarcoma
26
Myofibrosarcoma, patient pop? H and E? IHC?
Elderly, extremeties: **ANY GRADE Highly infiltrative, nodules of atypical spindle cells in myxoid stroma, perivascular atypical tumor cell and curvilinear blood vessels** SMA+, no specific genetics
27
Sclerosisng epithelioid fibrosarcoma history? H and E? Genetics?
On spectrum with LGFMS Epithelioid cells in strands (single file), collagenous stroma (resembels osteoid) **EMA+ 50%, MUCA4+** **t(7;16) FUS-CREB3:2** **t(11;16)FUS-CREB3L1** **Pure SEF: EWSR1-CREB3L1/2**
28
Synovial Sarcoma patient population? H and E? Genes? IHC?
Young adults mostly in deep extremeties Monophasic: Hypercellular spindle cells in fasicles, **bland nuclei, HPC like vessels, t(X;18) SYT-SSX2** Biphasic: Spindle and epitheliod cells; **t(X;18) SYT-SSX1** **IHC: EMA\>keratin, TLE1+ nuclear,** CD99+, S100+/-
29
Solitary fibrous tumor, population/correlate? H and E? Gene? IHC?
Adults, considered a sarcoma; **IGF2 secretion--hypoglycemia** Well circumscibed; **patternless pattern of bland spindle/oval cells in collagen, HPC like vessels** **STAT6 (inv12 NAB2-STAT6), CD34+**, EMA+/-, SMA+/-,S100+/-, Keratin +/-
30
Inflammatory myofibroblastic tumor, population? H and E? Gene? IHC?
Children\>adults, anywhere in body Spindled myofibroblasts (Hypocellular myxoid, Fasiculated with ganglion like, scar like), **all have lymphocytes, plasma cells and eos** ALK+ rearrangement 60% SMA +/-, MSA+/-, Desmin +/-, **ALK+ cyto**
31
Fibrosarcoma population? H and E? Gene?
1st year of life rapidly enlarging **Herringbone with intersecting fasicles of round/spindle cells** **ETV6-NTRK3 t(12;15)**
32
Two types of Epithelioid Sarcoma? H and E? IHC?
Distal: Young adults, **Epithelioid cells, central necrosis/amporphus material** Proximal: Ugly pleomorphic cells with large nuclei, **prominent nuclei** **Keratin +,** EMA+ CD34+, 50%, ERG some +, **INI1 (SMARCB1) loss**
33
Alveolar Soft Part Sarcoma population? H and E? IHC? What is common?
Yonger patients in head and neck, older in deep thigh Polygonal cells with eosinophilic granular cytoplasm (**crystals on EM)**, Prom nuclei, vessels surrounding **TFE3+ (TFE3-ASPSCR1 der (17)t(X;17)(p11q25)** Mets!
34
Clear cell sarcoma populatin? H and E? IHC? Gene?
Young adults, distal extremities Nests of short fasciles, **Pale/pink cytoplasm, prominent nucleoli, some giant cells** HMB45 stronger than S100, MelanA, MiFT+, Keratin-, EMA- **t(12;22) EWSR1-ATF1**
35
External Rhabdoid Tumor population? H and E? Genes?
Infants, children **deep axial region** and liver. Rapid growth Sheets of Rhabdoid cells with large round nuclei **PAS+, Keratin +**, EMA+, S100/CD99 +/- **INI1 Loss (SMARCB1)** **POOR PROGNOSIS**
36
PEComa's (3 of them) H and E? IHC?
Angiomyolipoma, Clear cell sugar tumor of lung, LAM; **Melanocytic/myogenic diff** Nest surrounded by delicate capillary vessels, polygonal cells with eosinophilic cytoplasm IHC: **SMA+, HMB45+, MelanA, MiTF/S100 -**
37
Chordoma locations? H and E? IHC?
Base of skull or sacrum Lobules of strands and chords in myxoid stroma; clear to eosinophlic bubbly cells with bland nuclei Keratin +, EMA+, S100+, **Brachyury +** 7 year survival
38
Extraskeletal Myxoid Chondrosarcoma population/location? H and E? IHC? Genes?
Adults in thigh Well-demarcated; multilobulated, **interconnected forming cords, cartilage tends to be ABSENT** S100+ 20%, CD117+ 30%, **INI1 loss in rhabdoid cells** **t(9;22) EWSR1-NRA3** **t(9;17) TAF15-NR4A3**
39
DFSP? H and E? IHC? Gene?
Wide age range, pale brown raised lesion on legs; **Also has ugly giant cell variant--same molecular** Diffuse involvement of dermis/subcutis, **Honeycomb entrapment of fat, skin appendages, whorled pattern** **CD34 diffuse +** t(17;22) COL1A1-PDGFB
40
Dermatofibroma? H and E? IHC?
Raised lesion on legs of adults **Epidermal hyperplasia and basal hyperpigmentation**; negative zone and then spindle cells win haphazard fasicles, **Entrap dermal collagen** **Some peripheral CD34 + (DFSP more +), FXIIIa+ dendridic cells**
41
Juvenile Xanthrogranuloma? H and E? IHC? Tx?
Infants/children Nodular, polypoid growth, flattened epidermis, mononuclear histeocytes, **Touton giant cells with foamy cytoplasm** CD68+, CD163+, SMA+
42
Atypical fibroxanthoma population? H and E? IHC? Behavior?
Old people, sun damaged skin **High grade UPS like lesion that has pushing border, no infiltration of subcutis** Maybe SMA+, exclude other lesions! Benign; if aggressive features it is dermal sarcoma
43
Plexiform fibrohisteocytic tumor, population? H and E? Can it metastasize?
Children, young adults **Spindle cells in fasciles/whorls, histeocytes, osteoclast like giant cells, rare/no mites, no necrosis** CD68+, SMA+ Rarely
44
Myxoma associated with? H and E? Mutation?
Mazabraud syndrome (intramuscular myxoma and skeletal fibrous dysplasia) **CD34+, SMA+ (EMA-, MUC4-, vs LGFMS)** **GNAS in intramuscular**
45
Superficial angiomyxoma? H and E?
Like a myxoma in the skin, **thin walled vessels, entraped skin adnexa, single/clustered neutrophils** **Associated with Carney Complex**
46
Acral fibromyxoma locations? H and E?
Hands, feet, peri-ungual, **painful** Dome shaped dermis mass, spindle-stellate fibroblasts in myxoid stroma, small vells, mast cells, **no adnexal entrapment or neutrophils (vs superficial fibromyoma)**
47
Deep/agressive angiomyxoma, location? H and E? IHC?
Adult females in perineum/pelvis Lobulated, **infiltrative, hypocellualr spindle cells in lose matrix, VESSELS,** **ER/PR+** **Can recur!**
48
Angiofibroma of soft tissue? H and E? Translocation?
Circumscribed bland spindle cells in collagenous-myxoid stroma, branching thin-walled ectatic vessels. t(5;8)
49
Fibrous hamartoma of infancy? H and E? IHC?
Before age 2 axilla/inginal Ill defined, Fibrous tissue **SMA+**, immature mesenchymal tissue **SMA-**, adipose tissue **S100+** Needs local excision
50
Phosphaturic mesenchymal tumor? H and E? Serum marker?
Rare tumor of middle aged adults **Bland spindle-stellate cells in "smudy MATIX", grungy calcs, microcysts** FGF23=tumor induced osteomalacia
51
Angiomatoid fibrous histeocytoma? H and E? IHC? Translocation?
Teens, young adults, **lymph node rich area** Fibrous pseudocapsule, **pericapsular lymphoid tissue, nodules of spindled cells with syncytial growth, pseudoangiomatous blood filled spaces** **50% EMA, CD99 +, Desmin +** t(2;22) EWSR1-CREB1 90%

Soft Tissue and Bone (2 decks)

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