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

B6.016 - Soft Tissue Tumors Prework Part 1 Flashcards

1
Q

what are soft tissue tumors

A

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

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2
Q

describe where soft tissue tumors occur in the body

A

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

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3
Q

3 categories of soft tissue tumors

A
  1. benign 2. intermediate (borderline), frequently recur and rarely metastasize 3. malignant (sarcoma)
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4
Q

describe treatment of sarcomas

A

depends on:

Type/site

grade

stage

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5
Q

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

A
  1. tumors of adipose tissue
  • Lipoma
  • Atypical lipomatous tumor
  • Liposarcoma
  1. Tumors and tumor like lesions of fibrous tissue
  • fibroma, nodular fasciitis
  • fibromatoses
  • fibrosarcoma
  1. Tumors of smooth muscle
  • leiomyom
  • leiomyosarcoma
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6
Q

what are the tumors of skeletal muscle

A

Rhabdomyoma

rhabdomyosarcoma

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7
Q

what are the vascular tumors

A

hemangioma

kaposi sarcoma

angiosarcoma

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8
Q

what are peripheral nerve sheath tumors

A

neurofibroma, schwannoma

malignant peripheral nerve sheath tumor

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9
Q

what are the tumors of uncertain histogenesis

A

synovial sarcoma

undifferentiated pleomorphic sarcoma

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10
Q

describe typical ages for rhabdomyosarcoma, synovial sarcoma, liposarcoma

A

rhabdomyosarcoma - children

synovial sarcoma - young adults

liposarcoma - mid to late adult life

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11
Q

important prognostic information for tumor

A

size, mitotic activity, degree of necrosis

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12
Q

lipoma features, tx, histo

A

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

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13
Q
A

lipoma

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14
Q

liposarcoma features, where it arises

A

one of the most common sarcomas in adults

arise in deep soft tissues of proximal extremities and retroperitoneium

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15
Q

subtypes of liposarcomas

A
  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)
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16
Q

liposarcoma histology

A

lipoblasts are almost always present

resemble fetal fat cells

contain cytoplasmic vacuoles of lipid that scallop the nucleus

wide excision necessary

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17
Q
A

Well differentiated liposarcoma

has lipoblasts and MDM2 amplification

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18
Q
A

well differentiated liposarcoma

has lipoblasts and MDM2 amplification

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19
Q
A

dedifferentiated liposarcoma

has MDM2 amplification, arises from WD liposarcoma, higher grade

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20
Q
A

Myxoid liposarcoma

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

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21
Q
A

arrows pointing to lipoblasts

myxoid liposarcoma

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

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22
Q

nodular fasciitis features

A

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

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23
Q

histo of nodular fasciitis

A

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

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24
Q
A

nodular fasciitis

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25
Q

types of superficial fibromatosis and histo appearance

A

palmar (dupuytrens contracture)

plantar (ledderhosens disease)

penile (peyronies disease)

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

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26
Q
A

superficial fibromatosis

27
Q

describe deep seated fibromatosis

A

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
Q

intra abdominal desmoids often occur in patients having

A

gardner syndrome, variant of familial adenomatous polyposis

29
Q

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

A

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
A

desmoid tumor

31
Q
A

desmoid tumor

32
Q
A

desmoid tumor

33
Q

fibrosarcoma features, histo

A

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
A

fibrosarcoma

35
Q

leiomyoma fetures and histo

A

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
A

leiomyoma

37
Q
A

leiomyoma

38
Q
A

leiomyoma

39
Q

features, histo of leiomyosarcoma

A

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
A

leiomyosarcoma

41
Q
A

leiomyosarcoma

42
Q
A

leiomyosarcoma

43
Q
A

leiomyosarcoma

44
Q

rhabdomyosarcoma features, histo, IHC

A

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
A

rhabdomyosarcoma

46
Q
A

rhabdomyosarcoma

47
Q
A

rhabdomyosarcoma

48
Q

3 types fo rhabdomyosarcoma

A
  1. embryonal
  2. alveolar
  3. pleomorphic
49
Q

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

A
50
Q

embryonal rhabdomyosarcoma features

A

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
A

sarcoma botryoides

rhabdomyosarcoma

52
Q
A

botryoid RMS

53
Q
A

botryoid RMS

54
Q
A

sarcoma botryoides

RMS

55
Q

alveolar RMS features

A

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
Q

what does PAX3-FOXO1 protein do

A

feature of alveolar RMS

chimeric protein leads to dysregulation of skeletal muscle differentiation

57
Q

histo of alveolar RMS

A

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

rhabdomyoblasts may be seen

58
Q
A

alveolar RMS

59
Q
A

alvolar RMS

60
Q
A

alvolar RMS

61
Q
A

alveolar RMS

62
Q
A

alveolar RMS

63
Q

tx of RMS and ranking of prognosis best>worst

A

surgery, radiation, chemo

botryoid>embryonal>pleomorphic>alveolar

MM1 Leesy (6 decks)

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