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Exam 2 Skin block > soft tissues > Flashcards

soft tissues Flashcards

1
Q

General principles

A

benign outnumber malignant at least 100:1

metastasis tend to be “blood born” to lungs, liver, bone

40% lower extremity; especially thigh

30% trunk and retroperitoneum

bone involved by direct extension or metastasis

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2
Q

Etiology

A

most common cause is unkown

associations: radiation, trauma (post massectomy: angiosarcoma, HHV8 (kapsi’s sarcoma), chemical exposure, and thermal burns

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3
Q

Genetic syndromes

A

neurofibromatosis types 1: malignant peripheral nerve sheath tumor

gardner syndrome: fibromatosis (desmoid)

li-fraumeni syndrome (mutant p53): osft tissue sarcoma and other malignancies

hereditary hemorrhagic telangiectasia (osler-weber-rendu disease): teleangiectasias over skin and mucosal surfaces

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4
Q

Ewing sarcoma and primitive neuroectodermal tumor

A

t (11:22) (q24;q12) EWS FLI1 fusion

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5
Q

liposarcoma myxoid and round cell type

A

t(12:16)(p13;q11) FUS-DDIT3 fusion

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6
Q

Synovial sarcoma

A

t(x;18)(p11;q11) SS18-SSX fusion gene

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7
Q

rhabdomyosarcoma alveolar type

A

t(2;13)(q35;q14) PAX3-FOX01 fusion

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8
Q

extraskeletal myxoid chondrosarcoma

A

t(9;22)(q22;q12) EWS-CHN fusion

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9
Q

Age: What is most common for each age group

A

15% arise in children

rhabdomyosarcoma in children

synovial sarcoma in young adults

undifferentiated pleomorphic sarcoma (malignant fibrous histiocytoma) and liposarcoma in mid to late adulthood.

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10
Q

What affects Prognosis

A

Type (recapitulation of tissue type)

stage (location and size): superficial vs deep, size, metastasis, etc

grade: cell pleomorphism, necrosis, mitotic

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11
Q

Cell type: Spindle cell

A

rod shaped, long axxis at least twice short axis

fibrous, schwann cell, firbohistocytic, smooth msulce

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12
Q

Cell type: small round (blue) cell

A

round, little cytoplasm

rhabdomyosarcoma, PNET/EWINgs

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13
Q

Cell type: epitheliod

A

polyhederal; more cytoplasm

epithelioid sarcoma

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14
Q

Cell type: matrix producing

A

bone, cartilage

extrasekeletal osteosarcoma

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15
Q

Cell type: biphasic

A

spindle and epithelial cells

biphasic synovial sarcoma

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16
Q

architectural patterns: fascicles of spindle cells

A

smooth muscle

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17
Q

architectural patterns: short fasciles of spindle cells radiating from center (storiform; pinwheel-like)

A

fibrohistiocytic

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18
Q

architectural patterns: nuclei in columns (palisading)

A

schwann cell

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19
Q

architectural patterns: herringbone

A

fibrosarcoma

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20
Q

architectural patterns: biphasic: spindle and epithelioid

A

synovial sarcoma

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21
Q

lipoma: description, location, and convention

A

most common adult soft tissue tumor: soft, mobile. Painless with one exception (angiolipoma)

most common location: subcutaneous trunk and proximal extremities

conventional: thinly encapsulated yellow tumor. Lobules of mature fat

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22
Q

other lipoma variaants

A

fibrolipoma: fibrous areas
angiolipoma: small vessels

dpinle cell lipoma: spindle cell areas

intramusclular lipoma: lipoma within muscle

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23
Q

liposarcoma age, location, and types (4)

A

adults 40-60

deep soft tissues: retroperitoneum (often very large). Proximal extremities

types: well differentiated (retroperitoneal), myxoid/round cell (extremities), pleomorphic-very aggressive course, dedifferentiated

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24
Q

liposarcoma, well differentiated: overview, gross, histo, growth description, genetics

A

most common type of liposarcoma

gross appearance same as lipoma

micro can be very close to benign lipoma: atypical spindle cells. Lipoblast: cytopalsmic vacuoles scallop nucleus

tend to be indolent with local recurrence

MDM2 amplification (inhibits p53)

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25
Q

liposarcoma, myxoid/round cell: genetic, location, histo

A

t(12;16) FUS-DDiT3 fusion protein

intramuscular, most often the thigh

round cell component is aggressive: rare lipoblast

myxoid tends to be indolent: chicken wire pattern of thin capillaries, rare lipoblast

26
Q

Liposarcoma, others

A

pleomorphic liposarcoma: very aggressive

dedifferentiated liposarcoma: sarcoma arising from a well differentiated liposarcoma

27
Q

Fibrous (myofibroblastic) tumors/proliferations Types

A

benign reactive proliferations: nodular fasciitis, myositis ossificans

fibromatoses: deep fibromatosis (desmoid), superficial fibromatosis

fibroma

fibrosarcoma

28
Q

nodular fasciitis: location, growth description

A

most common on volvar forearm

solitary rapidly growing 2-3 cm: may be painful. 10% history of trauma

deep dermis, subcutis, or muscle

reactive process, rarely recurs if excised

spindle cell proliferation of fibroblasts

29
Q

Myositis/panniculitis ossificans: age, cause, location, differentatiation

A

atheltetic adolescents and young adults

50% trauma

subcutaneous and muslce

early painful, circumscribed and firm

3-6 cm

fibroblastic but later develops bone

ends as trabecular bone with marrow

30
Q

fibromatoses types

A

superficial fibromatosis: palmer fibromatosis, plantar fibromatosis, penile fibromatosis

deep seated fibromatosis (desmoid tumor): extra abdominal, abdominal, intra abdominal

31
Q

superficial fibromatosis: histo, differentiation,

A

nodular or poorly defined fascicles of fibroblasts and abundant collagen

course: may progress, regress or stabilize

may recur after excision

32
Q

Palmer fibromatosis (dupuytren’s contracture)

A

nodular thickening of palmer fascia; puckers skin and digit flexion contraction (mostly 4 and 5)

50% bilateral

33
Q

Plantar fibromatosis

A

bilateral infrequent

irregular of nodular thickening of plantar fascia

34
Q

penile fibromatosis (peyronie’s disease)

A

induration of mass on dorsolateral penis

can cause abnormal curvature of penis

can constrict urethra

35
Q

deep-seated fibromatosis (desmoid tumors)

A

large infiltrative: locally aggressive, but does not metastasize

teens-30s

mutation in APC or b-catenin

recur if not completely excised: wide margin needed

36
Q

deep seated fibromatosis types

A

abdominal: anterior abdominal wall, women during or after preganncy or c section.

intra abdominal desmoid: mesentry and pelvic walls, gardner syndrome

extra abdominal

gray white poorly demarcated

fibroiblasts in fascicles infiltrate tissue

37
Q

fibrosarcoma

A

rare; most common in deep extremities

infiltrative fish-flesh with hemorrhage and necrosis

spindle cell with areas of herringbone pattern

50% recur, 25% metastasize

38
Q

skeletal muscle tumors: types

A

rhabdomyoma

cardiac rhabdomyoma

rhabdomyosarcoma: embryonal, alveolar, pleomorphic

39
Q

rhabdmyosarcoma: age, location on body

A

most common soft tissue sarcoma of childhood and adolescence; rare after age 20

head/neck (nasal cavity, orbit, middle ear)

gu tract

40
Q

embryonal rhabdomyosarcoma: age and subtypes

A

60% of rhabdomyosarcomas

chidlren < 10 years

subtypes: sarcoma botryoides in walls of hollow viscera and mucosal lined structures

41
Q

embryonal rhabdomyosarcoma: histo

A

soft gray infiltrative mass

mimic skeletal muscle embryogenesis: round and spindle cells

sarcoma botryoides variant: cluster of grapes protrude into lumen. Cambium layer: submucosal hypercellular area, best prognosis amongst embryonal rhabdomyosarcoma

42
Q

rhabdmyosarcoma: rhabdomyoblast

A

eccentric eosinophilic cytoplasm with thick and thin filaments

tadpole cells and strap cells

EM: sarcomeres

myogenic markers positive: MYOD1, myogenin, desmin

small round blue cell

may see cross striations in some cells

43
Q

alveolar rhabdmyosarcoma: age, location in body, histo, genetic, prognosis

A

early to mid adolescence

deep muscles of extremities

fibrous septa divide tumor into alveolar-like spaces centrally containg discohesive cells while peripheral cells stick to wall

t(2;13) or t (1;13): PAX3 or PAX7 to FOXO1

poor prognosis vs embryonal

44
Q

pelomorphic rhabdmyosarcoma: location in body, histology, caveats

A

deep soft tissue of adults

very pleomorphic with rhabdomyoblasts

often mistaken for undifferentiated pelomorphic sarcoma: desmin positive, myod1 or myogenin positive

45
Q

rhabdmyosarcoma prognosis ranking

A

sarcoma botryoides: best

embroyonal: second best

alveolar and pleomorphic: poor

65% of children are cured

adults do poorly

46
Q

Leiomyoma: description, location,

A

benign smooth muscle tumor

most common in uterus

skin: erector pili, niipples, scrotum, labia. Pilar leiomyoma often painful and multiple. Adolescent and early adults.

bland smooth muscle cells in fascicles

rare in deep tissues

47
Q

Leiomyosarcoma: gender, description histo, tests

A

10-20% of soft tissue sarcomas

F>M

skin, deep extremities and retroperitoneum

white masses can be large

malignant spindle cells

smooth muscle actin and desmin

negative for MyoD1 and myogenin

48
Q

synovial sarcoma: age, location in body and histo. genetics

A

originally believed to recaptiulate synovium but cell of origin is unknown: <10% intra articular

10% of soft tissue sarcomas

age 20-40

deep and around large joints: 60-70% lower extremities, especially knee/thigh. often microcalcifications on xray

t(x;18) SYT-SSX1 or SYT-SSX2 fusion

prognosis: 25-60% 5 years

49
Q

synovial sarcoma:

A

biphasic (dual) differentiation: epithelial cells as glands, cords, or nests. SPindle cells: cellular in fascilces

monophasic (spindle cell) variant: positive for cytokeratins (epithelial marker), epithelial membrane antigen (EMA)

50
Q

undifferentiated pleomorphic sarcoma (malignant fibrous histiocytoma): description, pattern, location, histo

A

group of aggressive neoplasms

cell pleomorphism and storiform pattern (diagnosis of exclusion)

proximal extremities and retroperitoneum

large grey, hemorrhagic and or necrotic

metastasis 30-50%

51
Q

immunohistochemistry: epithelial membrane antigen and cytokeratin

A

general epithelial marker

52
Q

immunohistochemistry: MyoD1 and myogenin

A

skeletal muscle

53
Q

immunohistochemistry: actin and desmin

A

skeletal or smooth muscle

54
Q

immunohistochemistry: vimentin

A

nonepithelial tumors

55
Q

immunohistochemistry: smooth muscle actin

A

smooth muscle

56
Q

immunohistochemistry: Epithelium

A

cytokeratin and EMA

57
Q

immunohistochemistry: skeletal muscle

A

myoD1, myogenin, desmin and actin

58
Q

immunohistochemistry: smooth muscle:

A

desmin and actin

59
Q

immunohistochemistry: nonepithelial tumors

A

vimentin

60
Q

painful skin lesions “blue angel”

A

blue rubber bleb nevus

angiolipoma

neuroma (traumatic)

glomus tumor

eccrine spiradenoma

leiomyoma (cutaneous)

Exam 2 Skin block (22 decks)

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