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Pathology > Sarcoma > Flashcards

Sarcoma Flashcards

1
Q

How common are adult sarcomas (percentage of malignancies)

A

1%
15% of childhood malignancies

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2
Q

What are the most common sarcomas in young adults

A

Synovial, Ewing, alveolar rhabdomyosarcoma

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3
Q

What are the most common sarcomas in older adults

A

Liposarcoma, leiomyosarcoma, undifferentiated pleomorphic sarcoma, fibrosarcoma

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4
Q

What is the median age for adult sarcomas

A

50-60

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5
Q

What sarcomas are associated with NF1?

A

Malignant peripheral nerve sheath tumours
Rhamdomyosarcoma

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6
Q

What sarcomas are associated with Li-Fraumeni syndrome?

A

Rhabdomyosarcoma

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7
Q

Which sarcomas are most commonly associated with previous radiation therapy

A

Angiosarcoma, undifferentiated pleomorphic, fibrosarcoma

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8
Q

What general type of tissue do sarcomas arise from

A

Mesenchymal

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9
Q

How is T stage defined for extremity soft tissue sarcomas

A

T1 = 5.0
T2 5.1-10.0
T3 10.1-15
T4 15.1+

N1= any node involvement

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10
Q

How is soft tissue extremity sarcoma staged

A

I: Tany N0M0 grade 1
II: T1 grade 2-3
III: T2-4 grade 2-3
IV: any N or M involvement

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11
Q

What are some examples of less aggressive and aggressive fibromatosis

A

Dupuytrens (benign)
Desmond tumours: frequently recur, but do not metastasize.

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12
Q

Which type of sarcoma arises from adipose tissue, what are the main subtypes

A

Liposarcoma
-well differentiated (amplification of region of chromosome 12q encoding MDM2 and CDK4)
-myxoid (t12;16 translocation)
-pleomorphic (complex karyotype/genetic instability)

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13
Q

What type of sarcoma is associated with skeletal muscle. What are the most common subtypes

A

Rhabdomyosarcoma
-note they arise in areas without skeletal muscle as they arise from stem cells rather than differentiated myocytes
-Alveolar (most common in childhood, specific common translocation pax:FOX01)
-embryonal (childhood); includes spindle cell/sclerosing. Negative for pax:fox01
-pleomorphic/undifferentiated

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14
Q

What type of sarcoma is associated with smooth muscle and where are they most commonly found

A

Leiomyosarcoma
Found in extremities and retroperitoneum.

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15
Q

What type of sarcoma is associated with cartilage

A

Chondrosarcoma

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16
Q

What type of sarcoma is associated with blood vessels. What is the benign condition

A

Angiosarcoma
Benign:haemangioma

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17
Q

What are the microscopic features of undifferentiated pleomorphic sarcoma

A

Pleomorphic, bizarre cells

MANFICS
Mitosis
Atypia
Necrosis
Foamy cytoplasm
Inflamed collagenous cytoplasm

18
Q

Which sarcomas are more likely to metastasis to lymph nodes

A

CARE
Clear cell sarcoma
Angiosarcoma
Rhabdomyosarcoma
Epithelioid sarcoma

19
Q

What is the most radiosensitive type of adult soft tissue sarcoma

A

Myxoid liposarcoma

20
Q

What IHC markers are general for all muscle types

A

Desmin
MSA (muscle specific actin)

21
Q

What is one IHC specific to smooth muscle, and one specific to skeletal muscle

A

SMA (smooth muscle actin): smooth muscle
MyoD: skeletal/striated muscle. This is a nuclear marker

22
Q

What are the microscopic features of myxoid liposarcoma

A

Uniform round to oval shaped primitive non-lipogenic cells
Chicken wire vasculature
Abundant myxoid matrix
Multivacuolar and univacuolar lipoblasts (sometimes with appearance of small signet ring cells)

23
Q

What are key genetic/molecular abnormalities associated with well differentiated liposarcoma

A

12q amplification, which results in amplification of CDK4 (cell cycle progression) and MDM2 (p53 breakdown)
FISH for MDM2 amplification
p16 positivity is a highly sensitive, but non-specific surrogate for CDK4 amplification (due to negative feedback loop)

24
Q

What sarcomas are most associated with distant metastasis

A

Leiomyosarcoma
Dedifferentiated liposarcoma and myxoid liposarcoma

25
Q

What are the two histological patterns of synovial sarcoma

A

Monophonic: spindle cells only
Biphasic: spindle cells and epithelial cells

26
Q

What are the components of grading of soft tissue sarcomas

A

1- differentiation
2- mitotic rate
3- necrosis

27
Q

What is the relevance of IHC for myxoid liposarcoma

A

Not usually required.
Should be S100 positive
t(12:16) and t(12:22)

28
Q

How is leiomyosarcoma defined

A

Smooth muscle tumour with:
-atypia
AND
-mitotic activity OR necrosis OR size >10cm

Otherwise would be a leiomyoma

29
Q

What are the epidemiological patterns of leiomyosarcoma

A

Median age 60-69
Usually female
A/w EBV in immune compromised patients
Third most common retroperitoneum sarcoma

30
Q

What is the microscopic appearance of leiomyosarcoma

A

Spindle cells in intersecting fascicular arrangements
Elongated cigarette shaped nuclei

31
Q

What is the typical IHC profile of leiomyosarcoma

A

Positive: SMA, vimentin, desmin, CD34, S100
Negative: CD117

32
Q

What is the characteristic architecture of fibrosarcoma

A

Herringbone

33
Q

What is the main value of grading soft tissue sarcomas, and what are the three components of grading

A

Prognosticates for OS and development of metastasis. Does not predict local recurrence risk

Composed of:
-differentiation
-mitotic count
-degree of necrosis

34
Q

Which sarcomas are high grade by definition

A

Ewings
Rhabdomyosarcoma
Epithelioid sarcoma
Clear cell sarcoma
Alveolar soft part sarcoma

35
Q

How is myxoid liposarcoma graded

A

Based on the percentage round cell component
>5% = high grade

36
Q

How are bone sarcomas graded

A

Based on the histological subtypes

37
Q

What constitutes a good response to neoadjuvant chemotherapy in oesteosarcoma/ Ewings sarcoma

A

<10% viable tumour

38
Q

How are margins defined in sarcoma

A

Radical: margin through normal tissue, extracompartmental
Wide: margin through normal tissue, intracompartmental
Marginal: margin within reactive zone, but extracapsular
Intralesional: within lesion

The aim is to achieve a “wide margin”

Numerically aim for margin of 1-2cm

39
Q

What is the role of radiation therapy for bone sarcomas

A

Ewings (but first line is chemo and surgery)
Inoperable osteosarcoma and chondrosarcoma

40
Q

What are the IHC and microscopic differences between kaposi sarcoma and angiosarcoma

A

Both: Infiltrative vascular channels. CD34, CD31 positive
Kaposi: HHV8+. Mitosis, but minimal nuclear atypia or pleomorphism. Spindled cells. Extravasation of erythrocytes, hemosiderin
Angiosarcoma: HHV8 negative. Marked nuclear atypia. Usually in sun damaged or radiation exposed skin

Pathology (16 decks)

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