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

B6.016 - Soft Tissue Tumors Prework Part 1 Flashcards

(63 cards)

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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
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
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
desmoid tumor
31
desmoid tumor
32
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
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
leiomyoma
37
leiomyoma
38
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
leiomyosarcoma
41
leiomyosarcoma
42
leiomyosarcoma
43
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
rhabdomyosarcoma
46
rhabdomyosarcoma
47
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
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
sarcoma botryoides rhabdomyosarcoma
52
botryoid RMS
53
botryoid RMS
54
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
alveolar RMS
59
alvolar RMS
60
alvolar RMS
61
alveolar RMS
62
alveolar RMS
63
tx of RMS and ranking of prognosis best\>worst
surgery, radiation, chemo botryoid\>embryonal\>pleomorphic\>alveolar

MM1 Leesy (6 decks)

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