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FPP_MSS > Soft Tissue Tumors (Rao) > Flashcards

Soft Tissue Tumors (Rao) Flashcards

1
Q

Approximately what percentage of all soft tissue tumors are sarcomas/

A

~1%

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

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2
Q

What is the most common etiology of soft tissue tumors?

A

Unknown (idiopathic) or sporadic

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3
Q

Name (4) genetic syndromes associated with soft tissue tumors

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

Rhabdomyosarcoma chiefly affects what patient population?

A

children

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5
Q

Synovial sarcoma tends to appear in what age group?

A

Young adults

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6
Q

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

A
  1. Degree of differentiation/pleomorphism
  2. Average number of mitoses per high-power field
  3. Extent of necrosis
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7
Q

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

  • Superficial locations
  • Deep lesions
A

Superficial locations

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8
Q

The presence of multiple lipomas is suggestive of what?

A

rare hereditary syndromes

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9
Q

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

A

Lipoma

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10
Q

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

A

True

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11
Q

Classify the following types of liposarcoma based on degree of aggressiveness

  1. Myxoid/round
  2. Well-differentiated liposarcoma (WD-LPS)
  3. Pleomorphic
A
  1. Myxoid/round - usually intermediate aggressiveness
  2. WD-LPS - Relatively indolent
  3. Pleomorphic - usually aggressive and may metastasize
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12
Q

At what anatomical locations are liposarcomas usually found?

A

Deep soft tissues of proximal extremities

Retroperitoneum

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13
Q

Identify:

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

Pseudosarcomatous proliferation

**often mistaken for sarcoma

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14
Q

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

A

Nodular fasciitis

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15
Q

What is myositis ossificans?

A
  • 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)
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16
Q

What is a desmoid tumor?

A

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

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17
Q

What is the most common age range for desmoid tumors?

A

teens to age 30

18
Q

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

A

Gardner syndrome

Also associated with mutations in APC or ß-Catenin genes

19
Q

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

A

Peyronie disease

20
Q

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

A

Dupuytren contracture

21
Q

Describe the general properties of a fibrosarcoma

A
  • 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
Q

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

  • (+) vimentin
  • all others negative
A

Fibrosarcoma

23
Q

What is the most common neoplasm found in women?

A

Uterine leiomyoma

24
Q

What type of sarcoma exhibits the following immunohistological markers?

  • (+)SMA
  • (+)Desmin
A

Leiomyosarcoma

25
Q

Describe the properties of Leiomyosarcoma, including:

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

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

A

Rhabdomyosarcoma

27
Q

In what anatomical locations can rhabdomyosarcoma generally be found?

A
  • Bladder
  • Head & neck
  • Genitourinary tract

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

28
Q

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

A
  1. Embryonal
  2. Alveolar
  3. Pleomorphic
29
Q

What is sarcoma botyroides?

A

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

30
Q

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

A

embryonal: < 10 years old
alveolar: 10-25 years old

31
Q

What is the most common form of rhabdomyosarcoma?

A

Embryonal (49%)

Alveolar (31%)

32
Q

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

A

PAX3-FKHR (FOXO1)

PAX7-FKHR

33
Q

What immunohistological findings are generally found with embryonal rhabdomyosarcoma?

A

(+)Desmin

(+)Myogenin, MyoD1

34
Q

Where (anatomically) is alveolar RMS most frequently found?

A

Deep soft tissues of the extremities

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

35
Q

Where (anatomically) is embryonal RMS generally found?

A

H&N, orbit and para-meninges

genitourinary tract

deep soft tissues of the extremities, pelvis, and retroperitoneum

36
Q

In what patient population is synovial sarcoma most common?

A

young adults, primarily males

37
Q

What is the most common anatomical location of synovial sarcoma?

A

Knee

Over 80% found in deep soft tissue of the extremities

38
Q

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

A

t(X;18)(p11,q11)

39
Q

Are the cells of synovial sarcoma derived from the synovium?

A

No

Its histogenesis is largely unknown

40
Q

Synovial sarcoma

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

FPP_MSS (30 decks)

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