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Flashcards in Soft Tissue Tumors (Rao) Deck (40)
1

Approximately what percentage of all soft tissue tumors are sarcomas/

~1%

Benign tumors, mostly lipomas and hemangiomas, outnumber sarcomas by 100:1

2

What is the most common etiology of soft tissue tumors?

Unknown (idiopathic) or sporadic

3

Name (4) genetic syndromes associated with soft tissue tumors

  1. Neurofibromatosis type 1 (neurofibroma, malignant schwannoma)
  2. Gardner syndrome (fibromatosis)
  3. Li-Fraumeni syndrom (soft tissue sarcomas)
  4. Osler-Weber-Rendu syndrome (telangiectasia)

4

Rhabdomyosarcoma chiefly affects what patient population?

children

5

Synovial sarcoma tends to appear in what age group?

Young adults

6

Grading of a soft tissue tumor is primarily based on what criteria?

  1. Degree of differentiation/pleomorphism
  2. Average number of mitoses per high-power field
  3. Extent of necrosis

7

Tumors arising in which of the following locations is generally associated with a better prognosis?

  • Superficial locations
  • Deep lesions

Superficial locations

8

The presence of multiple lipomas is suggestive of what?

rare hereditary syndromes

9

What is the most common type of soft tumor found in adults?

Lipoma

10

True/False: complete excision of a lipoma is usually curative

True

11

Classify the following types of liposarcoma based on degree of aggressiveness

  1. Myxoid/round
  2. Well-differentiated liposarcoma (WD-LPS)
  3. Pleomorphic

  1. Myxoid/round - usually intermediate aggressiveness
  2. WD-LPS - Relatively indolent
  3. Pleomorphic - usually aggressive and may metastasize

12

At what anatomical locations are liposarcomas usually found?

Deep soft tissues of proximal extremities

Retroperitoneum

13

Identify:

  • a reactive non-neoplastic lesion that develops in response to trauma (or idiopathic). Develops suddenly and grows rapidly.

Pseudosarcomatous proliferation

**often mistaken for sarcoma

14

Identify: A pseudosarcomatous proliferation that occurs in the deep dermis, subcutis, or muscle. Several centimeters in diameter with poorly defined margins.

Nodular fasciitis

15

What is myositis ossificans?

  • Presence of metaplastic bone within muscle
  • Due to trauma in >50% of cases
  • Occurs in proximal extremities, usually young adults
  • Usually well-circumscribed (unlike osteosarcoma)

16

What is a desmoid tumor?

A deep-seated fibromatosis

Begavior of desmoid tumors lies somewhere between that of fibrous benign tumors and low-grade fibrosarcomas

Desmoid tumors frequently recur, even after complete excision

17

What is the most common age range for desmoid tumors?

teens to age 30

18

Desmoid tumors are occasionally associated with what genetic syndrome(s)?

Gardner syndrome

Also associated with mutations in APC or ß-Catenin genes

19

A superficial fibromatosis of occurring on the penis is called what?

Peyronie disease

20

A superificial fibromatosis of the palmar surface of the hand is called what?

Dupuytren contracture

21

Describe the general properties of a fibrosarcoma

  • Malignant tumor composes of fibroblasts
  • Occurs most often in adults
  • Generally found in the deep tissues of the thigh, knee, and retroperitoneum
  • Generally aggressive - recur in 50% of cases, with metastasis in 25% of cases

22

Name the disease associated with the following immunohistological marker(s):

  • (+) vimentin
  • all others negative

Fibrosarcoma

23

What is the most common neoplasm found in women?

Uterine leiomyoma

24

What type of sarcoma exhibits the following immunohistological markers?

  • (+)SMA
  • (+)Desmin

Leiomyosarcoma

25

Describe the properties of Leiomyosarcoma, including:

  • major patient population
  • common anatomic location(s)
  • prognosis
  • immunohistochemical staining

  • Adults, F>M
  • Location: skin and soft tissues of extremities and peritoneum
  • Prognosis: superficial associated with good prognosis. Retroperitoneal tumors display higher local invasion and metastasis, generally cannot be completely excised. Therfore, they tend to have a poorer prognosis.
  • (+)SMA, (+)Desmin

26

What is the most common type soft tissue sarcoma in children?

Rhabdomyosarcoma

27

In what anatomical locations can rhabdomyosarcoma generally be found?

  • Bladder
  • Head & neck
  • Genitourinary tract

Usually found at locations with little to no skeletal muscle otherwise present

28

What are the three (3) major subtypes of rhabdomyosarcoma?

  1. Embryonal
  2. Alveolar
  3. Pleomorphic

29

What is sarcoma botyroides?

A variant of embryonal rhabdomyosarcoma that has the gross appearance of a 'bunch of grapes'

30

What age group are embryonal rhabdomyosarcomas associated with? Alveolar rhabdomyosarcoma?

embryonal: < 10 years old

alveolar: 10-25 years old

31

What is the most common form of rhabdomyosarcoma?

Embryonal (49%)

Alveolar (31%)

32

What is(are) the gene(s) associated with alveolar rhabdomyosarcoma?

PAX3-FKHR (FOXO1)

PAX7-FKHR

33

What immunohistological findings are generally found with embryonal rhabdomyosarcoma?

(+)Desmin

(+)Myogenin, MyoD1

34

Where (anatomically) is alveolar RMS most frequently found?

Deep soft tissues of the extremities

Less common in: H&N, perineum, pelvis, retroperitoneum

35

Where (anatomically) is embryonal RMS generally found?

H&N, orbit and para-meninges

genitourinary tract

deep soft tissues of the extremities, pelvis, and retroperitoneum

36

In what patient population is synovial sarcoma most common?

young adults, primarily males

37

What is the most common anatomical location of synovial sarcoma?

Knee

Over 80% found in deep soft tissue of the extremities

38

What is the characteristic (90%) genetic association of synovial sarcoma?

t(X;18)(p11,q11)

39

Are the cells of synovial sarcoma derived from the synovium?

No

Its histogenesis is largely unknown

40

Synovial sarcoma

  1. Treatment
  2. common sites of metastasis
  3. Typical prognosis

  1. Aggressive limb-sparing excision (surgery)
  2. Common metastasis: lung, bone, regional lymph nodes
  3. Generally poor prognosis: 5yr 25-62%, 10yr 10-30%