M Khin - St Tumors & Tumor-Like Lesions

Front 1

Where are soft tissue tumors/tumor-like lesions seen?

Back 1

tumors of mesenchymal non-epithelial tissues (exc viscera)

including: mm, fibrous CT, fat, BV & nerves

Front 2

Benign: malignant

Back 2

100:1

Front 3

Cause of tumors?

Associations?

Back 3

Idiopathic

Taco Bell HouR (TBHR)

Trauma
Burns
Herbicides
Radiation

Front 4

What are the associated genetic syndromes?

Back 4

associated syndromes are LONG

Li-Fraumeni
Osler-Weber-Rendu syndromes
Neurofibromatosis I
Gardner

Front 5

What are the 4 MC malignancies in children?

Where do ST tumors falls?

Back 5

1. Brain
2. Hemopoietic
3. Wilms tumor
4. ST TUMORS

Front 6

In what locations do these tumors usually arise?

Back 6

Anywhere

Front 7

WHat are the features that influence prognosis?

Back 7

Histologic type
Grade & stage of tumor
Site of Tumor

Front 8

What is the most frequent ST tumor in subQ tissue?

Back 8

Lipoma

Front 9

What is the histo presentation of a lipoma?

How does it feel to touch?

Back 9

Encapsulated, composed of mature adipose tissue

Soft, moveable, painless

Front 10

What tumor is the 2nd MC sarcoma in adults?

Is it common in children?

Describe tumor; where is it usually seen?

Back 10

Liposarcoma

No

Bulky; thigh & retroperitoneum

Front 11

What is the microscopic appearance of a liposarcoma?

Back 11

lipoblasts in different grades of differentiation

ring chromosome cells w/ **abundant clear multi-cavuolated cytoplasm & eccentric darkly staining vacuole-compressed nucleus**

Front 12

Are liposarcomas differentiated?

Back 12

Poorly differentiated
Aggressive (85-90% metastasize)

Front 13

Describe benign fibrous histiocytomas.

What do the cells resemble?

Back 13

mobile, well-defined nodules in subQ tissues (limbs)

resemble fibroblasts, myofibroblasts, histiocytes

Front 14

Where do benign fibrous histiocytomas usually occur?

Back 14

skin as DERMATOFIBROMAS

Front 15

Malignant fibrous histiocytomas (MFH) are aka?

Significance of this tumor?

Back 15

Pleomorphic spindle cell sarcoma

***MC malignant soft tissue tumor***

The 2nd MC tumor was LIPOsarcoma

Front 16

1st and 2nd MC ST tumor?

Back 16

1. malignant fibrous histiocytoma
2. liposarcoma

Front 17

Where is MFH usually seen?

What does it look like?

Back 17

1. extremities
2. lobulated, fleshy infiltrating tumors w/ areas of hemorrhage & necrosis

Front 18

WHat is the microscopic appearance of MFH?

Back 18

pleomorphic anaplastic spindle cells (storiform pattern - cartwheel, spindle cells w/ elongated nuclei radiating from center) with giant tumor cells and abnL mitosis

Front 19

What does a fibroma look like?

Back 19

well-defined gray masses & composed of mature fibrous tissue

Front 20

Where do fibromas usually occur?

Back 20

in ST as well as **around teeth & in ovaries**

Front 21

Where do fibrosarcomas usually occur?

MCly in?

Back 21

deep soft tissue

thigh

Front 22

What do fibrosarcomas present as?

Back 22

gray soft fish-flesh infiltrating large masses, showing spindle cell growth w/ anaplasia

Front 23

Fibrosarcomas:
Rate of growth?

***microscopic appearance?***

Back 23

slow, painless

**herringbone pattern- spindle shaped cells w/ fascicular growth**

Front 24

Rhabdomyomas are benign tumors of?

WHere do they arise?

What are they a/w?

Back 24

benign tumors of skeletal muscles

arise in tongue & heart

a/w tuberous sclerosis

Front 25

Tuberous sclerosis:
1. genetic pattern
2. genetic dysfunction
3. type/location of tumors
4. in brain?
5. in heart?

Back 25

1. autosomal dominant
2. ch 9, 16
3. benign/brain,heart,kidney,retina,skin
4. cortical tubers (abnL glial & neural cells --> seizures) & subependymal
5. rhabdomyomas (large cells w/ spider-like nuclei)

Front 26

Important characteristic of rhabdomyosarcoma?

Back 26

MC ST sarcoma in children & young adults (uncommon in older adults)

Front 27

What is embryonal R seen? what does it consist of?

Back 27

1. heat and neck regions
2. consists of sheets of small round cells

Front 28

Where are botryoid R seen?

What do they look like***?

Histo?

Back 28

1. GU, biliary, URT in young children

2. **polypoid, soft, grape-like**

3. undifferentiated rhabdomyoblasts in myxoid stroma

Front 29

What ages are alveolar R seen?

Where do they occur?

Histo?

Back 29

1. 10-25 yoa
2. extremities
3. undifferentiated rhabdomyoblasts in alveolar or pseudoglandular arrangements

Front 30

What ages are pleomorphic R seen**?

Where do they occur?

Histo?

Malignancy?

Back 30

1. **adults >45 yoa**
2. lower limbs
3. large, atypical tumor cells resembling pleomorphic spindle cells
4. highly malignant tumors (respond to chemo)

Front 31

Pseudosarcomatous proliferations are often mistaken....

Back 31

as tumors; are NOT ca

Front 32

What is the etiology of pseudosarc prolif? a/w?

In what types of pts is there an inc incidence of fibromatosis?

Back 32

idiopathic; a/w local trauma & irritation

diabetes, alcoholics, epileptics

Front 33

What is the rate of progression of pseudosarc prolif?

Back 33

develop suddenly & grow rapidly

hypercellularity w/ high mitotic activity

Front 34

What are the 3 types of pseudosarc prolif and (their ddx)?

Back 34

1. nodular fasciitis (fibrosarcoma)
2. myositis ossificans (osteosarcoma)
3. Fibromatosis

Front 35

Nodular fasciitis:

Back 35

reactive cell prolif (2* to trauma) in adults

fibroblasts in loose myxoid backgroud

small masses in ext (rapid growth)

Front 36

Traumatic myositis ossificans

Back 36

circumstribed non-encap mass w/in mm extremities

prolif fibroblasts in myxoid stroma a/w cartilage & bone formation

hemorrhages

Front 37

fibromatosis (locally invasive - no metastasis)

Back 37

abd: affects mothers in perinatal period

extra-abd: m=f

Front 38

intra-abd:

a/w?

other stuff?

Back 38

Gardner syndrome (FAP, multiple osteomas, desmoid tumors)

nonencap exuberent fibroprolif lesion --> invades surround tissues (young women)

white sacr-like tumor w/ bnads of fibrocytes & collagenous tissue

Front 39

Palmar fibromatosis:

aka

other crap?

Back 39

dupuytren contracture on palm of hand

fixed flexion contracture of hand where fingers bend towards palm & cannot be fully extended

Front 40

Penile fibromatosis:

aka

other crap?

Back 40

peyronie ds

fibrous envelop surrounding penile corpora cavernosa causing abnL curvature of penis

Front 41

Deep-seated fibroma:

aka?

other imp stuff**?

Back 41

desmoid tumor

**local invasion, no metastasis, abd walls of young fem is a common site

Front 42

What are the 3 superficial fibromatoses?

What is the deep seated fibromatosis?

Back 42

1. palmar fibro... --> Dupuytren contracture

2. plantar fibro...

3. penile fibro... --> peyronie ds