B6.016 - Soft Tissue Tumors Prework Part 1 Flashcards Preview

MM1 Leesy > B6.016 - Soft Tissue Tumors Prework Part 1 > Flashcards

Flashcards in B6.016 - Soft Tissue Tumors Prework Part 1 Deck (63):
1

what are soft tissue tumors

mesenchymal proliferations that occur in nonepithelial, extraskeletal tissues of the body most are benign

 

2

describe where soft tissue tumors occur in the body

40% lower extremities, 20% upper extremities, 10% head and neck, 30% trunk and retroperitoneum

3

3 categories of soft tissue tumors

1. benign 2. intermediate (borderline), frequently recur and rarely metastasize 3. malignant (sarcoma)

4

describe treatment of sarcomas

depends on:

Type/site

grade

stage

5

what are the classifications of soft tissue tumors (adipose, fibrous tissue, smooth muslce)

1. tumors of adipose tissue

  • Lipoma
  • Atypical lipomatous tumor
  • Liposarcoma

2. Tumors and tumor like lesions of fibrous tissue

  • fibroma, nodular fasciitis
  • fibromatoses
  • fibrosarcoma

3. Tumors of smooth muscle

  • leiomyom
  • leiomyosarcoma

 

6

what are the tumors of skeletal muscle

Rhabdomyoma

rhabdomyosarcoma

7

what are the vascular tumors

hemangioma

kaposi sarcoma

angiosarcoma

8

what are peripheral nerve sheath tumors

neurofibroma, schwannoma

malignant peripheral nerve sheath tumor

9

what are the tumors of uncertain histogenesis

synovial sarcoma

undifferentiated pleomorphic sarcoma

10

describe typical ages for rhabdomyosarcoma, synovial sarcoma, liposarcoma

rhabdomyosarcoma - children

synovial sarcoma - young adults

liposarcoma - mid to late adult life

11

important prognostic information for tumor

size, mitotic activity, degree of necrosis

12

lipoma features, tx, histo

benign tumor of fat, most common soft tissue tumor of adults

soft, mobile, usually painless

histo - encapsulated mass of mature fat cells

cured by simple excision

13

Q image thumb

lipoma

14

liposarcoma features, where it arises

one of the most common sarcomas in adults

arise in deep soft tissues of proximal extremities and retroperitoneium

15

subtypes of liposarcomas

1. well differentiatied (indolent)/dedifferentiated (aggressive) (Amplif. of MDM2 oncogene)

2. myxoid/round cell (t(12;16)(q13;11);fusion with DDIT3(CHOP) gene with FUS)

3. pleomorphic liposarcoma (aggressive)

16

liposarcoma histology

lipoblasts are almost always present

resemble fetal fat cells

contain cytoplasmic vacuoles of lipid that scallop the nucleus 

wide excision necessary

17

Q image thumb

Well differentiated liposarcoma

has lipoblasts and MDM2 amplification

18

Q image thumb

well differentiated liposarcoma

has lipoblasts and MDM2 amplification

19

Q image thumb

dedifferentiated liposarcoma

has MDM2 amplification, arises from WD liposarcoma, higher grade

20

Q image thumb

Myxoid liposarcoma

t(12;16)(q13;11), DDIT3-FUS fusion

21

Q image thumb

arrows pointing to lipoblasts

myxoid liposarcoma

t(12;16)(q13;11), DDIT3-FUS fusion

22

nodular fasciitis features

reactive pseudosarcomatous proliferation (sarcoma LIKE)

presents with recent hx of solitary rapid growing sometimes painful mass

often occurs in adults on the volar aspect of the forearm, chest and back

arises in deep dermins, subcutis or muscle, nodular and poorly defined

23

histo of nodular fasciitis

plump, randomly arranged spindle cells (fibroblasts) in a myxoid background containing lymphocytes and extravasated RBCs. Abundant mitotic figures. 

May regress spontaneously

rarely recurs after excision

24

Q image thumb

nodular fasciitis

25

types of superficial fibromatosis and histo appearance

palmar (dupuytrens contracture)

plantar (ledderhosens disease)

penile (peyronies disease)

histo: nodular or poorly defined bundles of mature fibroblasts surrounded by abundnat dense collagen

26

Q image thumb

superficial fibromatosis

27

describe deep seated fibromatosis

desmoid tumors

more aggressive than superfiical ones

more common in females

divided into extra abdominal, abdominal and intra

mutations in beta catenin genes are present in majority

 

28

intra abdominal desmoids often occur in patients having 

gardner syndrome, variant of familial adenomatous polyposis

29

histo/gross appearance of deep seated fibromatosis (desmoid tumors), tx

grossly: grey/white, firm, infiltrative masses

histo: long fascicles of elongated fibroblasts and collagen fibers, IHC: B-catenin

locally aggressive, recur if incompletely excised. Dont metastasize

may respond to tamoxifen, chemo or irradiation

30

Q image thumb

desmoid tumor

31

Q image thumb

desmoid tumor

32

Q image thumb

desmoid tumor

33

fibrosarcoma features, histo

malignant fibrous tumor

rare sarcoma, occurs mostly in retroperitoneum, thigh, knee and distal extremities

histo: typical composed of malignant spindle cells arranged in herringbone pattern with frequent mitoses and areas of necrosis

aggressive with high recurrence rate, may metastasize

34

Q image thumb

fibrosarcoma

35

leiomyoma fetures and histo

benign tumor of smooth muscle

very common in uterus

histo: intersecting bundles (fascicles) of spindle cells with blunt ended cigar shaped nuclei, minimal atypica and few mitoses

treated by simple surgical removl

36

Q image thumb

leiomyoma

37

Q image thumb

leiomyoma

38

Q image thumb

leiomyoma

39

features, histo of leiomyosarcoma

more often in women than men

most develop in skin, deep soft tissues of limbs and retroperitoneum

histo: malignant spindle cells with cigar shaped nuclei, frequent mitoses and necrosis

IHC: smooth muscle actin (SMA), H-caldesmon

 

40

Q image thumb

leiomyosarcoma

41

Q image thumb

leiomyosarcoma

42

Q image thumb

leiomyosarcoma

43

Q image thumb

leiomyosarcoma

44

rhabdomyosarcoma features, histo, IHC

most common soft tissue sarcoma of children and adolescents (<20)

most occur in head and neck or GU

histo: malignant cell is rhabdomyoblast (aka strap cell)

exccentric nucleus iwht abundnat granular eosinophilic cytoplasm with cross striations 

IHC: Desmin, myoglobin, MyoD1

45

Q image thumb

rhabdomyosarcoma

46

Q image thumb

rhabdomyosarcoma

47

Q image thumb

rhabdomyosarcoma

48

3 types fo rhabdomyosarcoma

1. embryonal

2. alveolar

3. pleomorphic

49

compare embryonla, alveolar, pleomorphic rhapbdomyosarcoma based on site, epidemiology, and clinical appearance

A image thumb
50

embryonal rhabdomyosarcoma features

most common (70%)

develops in wall of hollow mucosal lined organs, like nasopharynx, bile duct, vagina and bladder

children <5 yo

sarcoma botryoides is most common variant "bunch of grapes"

no characteristic cytogenic abnormality

51

Q image thumb

sarcoma botryoides

rhabdomyosarcoma

52

Q image thumb

botryoid RMS

53

Q image thumb

botryoid RMS

54

Q image thumb

sarcoma botryoides 

RMS

55

alveolar RMS features

aggressive, 30%

deep muscles of extremities, head and neck, 10-25 yo

t(2;13) (q35;q14)PAX-FOXO1 translocation

FISH detects FOXO1 breakpoint

PCR detects FOXO1-PAX fusion transcripts

 

56

what does PAX3-FOXO1 protein do

feature of alveolar RMS

chimeric protein leads to dysregulation of skeletal muscle differentiation

57

histo of alveolar RMS

high grade small round cell tumor, solid alveolar growth pattern, central loss of cohesion

rhabdomyoblasts may be seen

58

Q image thumb

alveolar RMS

59

Q image thumb

alvolar RMS

60

Q image thumb

alvolar RMS

61

Q image thumb

alveolar RMS

62

Q image thumb

alveolar RMS

63

tx of RMS and ranking of prognosis best>worst

surgery, radiation, chemo

botryoid>embryonal>pleomorphic>alveolar