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Path II > ST Neoplasm > Flashcards

ST Neoplasm Flashcards

1
Q

What do Soft tissue tumors recapitulate?

A

Mesenchymal tissues

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2
Q

What is the ratio of benign ST tumors to malignant?

A

100:1

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3
Q

How do sarcomas metastasize?

A

Hematogeneously

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4
Q

How often to benign ST tumors transform into malignant?

A

Rarely

Exception: Neurofibroma may transform to malignant in peripheral nerve sheath in NF Type 1

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5
Q

How is more susceptible to Kaposi Sarcom?

A

HIV patients, immunocompromised

HHV8

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6
Q

Who is predisposed to angiosarcoma?

A

Chronic lymphedema s/p mastectomy

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7
Q

What type of soft tissues end up in children and which end up in adults?

A

Oncogenic translocations in children

Genomic instability in Adults

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8
Q

What is critical for sacoma diagnosis?

A

Immunohistochemistry for lineage-specific etiopathogenetic markers

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9
Q

What are some ancillary techniques to find sarcomas?

A

RT-PCR (FuSION transcripts)

FISH for chromosomal rearrangements

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10
Q

How do you stage a sarcoma?

A

Based on depth

Superficial=suprafascial

Deep: Subfascial

Size: 5cm

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11
Q

What are the four common benign soft tissue tumors?

A

Lipoma

Fibrous/fibrohistiocytic tumors (noduclar fascitis, ealstofibroma)

Hemangioma/lymphangioma

Benign nerve sheath tumors (schwannoma, neurofibroma)

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12
Q

What are the 6 most common sarcoma types?

A

Undifferentiate/unclassifiable

Liposarcoma

Leiomyosarcoma

Myxofibrosarcoma

Synovial sarcoma

Malignant peripheral nerve sheath tumor

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13
Q

What are the the soft tissue tumor biologies?

A

Benign

Locally aggressive/intermediate

Rarely metastasizing/intermediate

Malignant

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14
Q

What are the the soft tissue tumor biologies?

A

Benign

Locally aggressive/intermediate

Rarely metastasizing/intermediate

Malignant

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15
Q

How are sarcomas graded?

A

Tumor differentiation

MItotic count

Tumor necrosis

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16
Q

How are liposarcomas divided?

A

3 categories

Atypical lipomatous tumor(ALT)/well-diff liposaroma/de deiff liposarcoma

Myxoid round cell liposarcoma

Pleomorphic liposarcoma

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17
Q

What is teh characteristic of ALT/WDLPS

A

Locally aggressive, non mets

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18
Q

What are the immune markers of ALT/WDLPS?

A

MDM2, CDK4 amplification

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19
Q

How do you detect ALT/ADLPS?

A

Immunohisto or FISH

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20
Q

What are the molecular characteristics of DDLPS?

A

Holds on to MDM2/CDK4 amplification but with more genomic instability

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21
Q

What are the characteristics of myxoid liposarcoma?

A

Continumum with round cell liposarcoma

%round cell component is correlated with mets risk

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22
Q

What is the translocation seen in myxoid liposarcoma?

A

t(12:16)

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23
Q

What is a pleomorophic liposarcoma?

A

High grade, genetrically unstable neoplasm

Scattered lipoblasts

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24
Q

What are rhabdomyosarcomas divided into?

A

Embryonal

Alveolar

Pleomorphic

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25
Q

What are rhabdomyosarcomas divided into?

A

Embryonal

Alveolar

Pleomorphic

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26
Q

What is a Botyroid rhabdomyosarcoma?

A

Presentation of embryomal RMS

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27
Q

What is alveolar RMS associated with which translocation?

A

t(2:13) or t(1:13)

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28
Q

What is the most aggressive sarcoma type?

A

Pleomorphic RMS

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29
Q

What is pyogenic granuloma?

A

Lobular capillary hemangioma

Neither pyogenic nor granuloma

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30
Q

What are angiosarcomas?

A

Highly malignant vascular tumors

Exposure to arsenic containing compounds high risk
Other exposures:
Thorotrast, Polyvinyl chloride

Chronic lymphedema is a risk factor also

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31
Q

What is a synovial sarcoma?

A

Spindle cell carcinoma of soft tissue

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32
Q

What is the translocation associated with synovial sarcoma?

A

t(X;18) with expression of TLE1 oncoprotein

Causes expression of EMA and cytokeratins

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33
Q

What is the translocation associated with synovial sarcoma?

A

t(X;18) with expression of TLE1 oncoprotein

Causes expression of EMA and cytokeratins

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34
Q

What are the common locations of ST neoplasms?

A

60% extremeities
25% Retroperiotoneum
10% HEENT
5% combined

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35
Q

What are the most common types of tumors in children?

A

Rhabdomyosarcoma

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36
Q

What is the most common type of tumor in adult?

A

Lipoma

Dermatofibroma

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37
Q

What causes death from ST neoplasms?

A

Mets to Lung (hematogenous spread)

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38
Q

What are some etiologic factors of ST neoplasms?

A

Radiation

Chemical exposure

Heat burns

Genetic syndromes

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39
Q

What are some genetic syndromes associated with St neoplasms?

A

Neurofibromatosis I
Gardner Syndrome (APC mutation): Polyposis/desmoid
Li-Fraumeni (TP53)
Osler-Weber-Rendu (ENG, ACVRL1, SMAD4): vascular

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40
Q

What are patients with chronic lymphedema at risk for?

A

Angiosarcoma

41
Q

What are patients with HIV at risk for?

A

Kaposis HHV8

42
Q

What are patients with a history of trauma at risk for?

A

Nodular fascicitis

Myositis ossificans

43
Q

What is seen on histo for nodular fascitis?

A

Cellular spindle tumor

No specific atypia

Extravasated RbCs

44
Q

What is the dual molecular pathogenesis of soft tissue tumors?

A

Specific gene alterations (reciprocal translocations) and simple karyotypes:

  • Similar to leukemia
  • Children/young adults

Complex unbalanced karyotypes due to p53 inactivation

  • Similar to carcinoma
  • Adults
45
Q

What is the dual molecular pathogenesis of soft tissue tumors?

A

Specific gene alterations (reciprocal translocations) and simple karyotypes:

  • Similar to leukemia
  • Children/young adults

Complex unbalanced karyotypes due to p53 inactivation

  • Similar to carcinoma
  • Adults
46
Q

How do you diagnose a ST neoplasm?

A

HIstory/PE/Imaging

Biopsy required for Dx
->3cm, FNA or core
-

47
Q

What procedures aid in Dx?

A

Histochemical stains

Electron microscopy

Immunochemistry (antibody directed against Tissue specific antigen) Most USED now

48
Q

What is S-100 a marker of for immunohistochemistry?

A

Marker of nerve sheath

Melanocytic, lipomatous, cartilaginous differentation

49
Q

What stain do you use for a well-diff liposarcoma?

A

CDK4

50
Q

What stain do you use for kaposis sarcoma?

A

HHV8

51
Q

What is the FISH method used for de-diff liposarcoma?

A

MDM2, shows high amplification

52
Q

What is the FISH method used for de-diff liposarcoma?

A

MDM2, shows high amplification

53
Q

What is the stage of a T1a tumor?

A

Superficial tumor

54
Q

What is the stage for T1b tumor?

A

Deep tumor

55
Q

What makes it a stage 2 tumor?

A

> 5cm

56
Q

Where are sarcomas most likely found and what is their size?

A

75% extremities

2/3 are deep and >9cm

(1/3 superficial and 5cm)

57
Q

How do you know if the tumor is deep on gross image?

A

It is below the SubQ fat

58
Q

What points towards a malignant lesion?

A

Hemorrhage/necrosis

59
Q

What points towards a benign lesion?

A

Well circumsribed with good behavior

60
Q

What are types of lipomas?

A

Lipoma, angiolipoma, spindle cell lipoma

61
Q

What are types of fibrous/fibrohistiocytic tumors?

A

Nodular fasciitis, elastofibroma

62
Q

What are types of vascular tumors?

A

Hemangioma, lymphangioma

63
Q

What are types of nerve sheath tumors?

A

Schwannoma, neurofibroma

64
Q

What is an example of a intermediate locally aggressive ST tumor?

A

Desmoid fibromatosis (gardner syndrome)

65
Q

What is an example of a intermediate rarely mets lesion?

A

Plexiform fibrohistiocytic tumor

Angiomatoid fibrous histiocytoma

66
Q

What factors do you use in grading soft tissue tumors?

A

Tumor diferentiation

  • Score One: Well differentiated
  • Score 2: Histo is certain but sarcoma looking
  • Score 3: Can’t tell where it came from

Mitotic count:
Score1: 0-9
Score 2: 10-19
Score 3: >19

Tumor Necrosis:

  • Score 0: no necrosis
  • Score 1: 50%

Grade is based on sum:

  • grade 1: 2,3
  • Grade 2: 4,5
  • Grade 3: 6,7,8
67
Q

Where do most lipomas arise?

A

Most common soft tissue neoplasm

SubQ adiopose tissue

68
Q

What are the histiological different types of lipomas?

A

Intramuscular
Angiolipoma
Fibrolipoma
Myelolipoma

69
Q

What is the treatment for a lipoma?

A

Local excision

70
Q

What can happen in a aniolipoma?

A

It can be mistaken for a vascular neoplasm

71
Q

What is the commonality and composision of liposarcomas?

A

20% of all sarcomas

3 Genetically distinct types
- Atypical lipomatous tumor (somatic ST)/well-differentiated liposarcoma (spermatic cord,mediatstinum, retroperit)

Can transform into de-differentiated liposarcoma

72
Q

What characterizes ALT/WDLPS/DDLPS?

A

MDM2/CDK4 amplification

ALT/WDLPS: No mets risk

DDLPS: 15-20% mets risk

73
Q

What is a feature of WDLPS on histo?

A

Lipoblasts with a CENTRAL NUCLEUS

74
Q

What is seen in a WDLPS slide sometimes?

A

It can abruptly transform in a DDLPS

75
Q

What are characterisitcs of myxoid/round cell liposarcomas?

A

Younger adults

90% in THIGH

Risk of Mets determined by percent of Round cell component

76
Q

What is the translocation of a myxoid/round tumor cell liposarcoma?

A

t(12:16)
t(12:22)]

Involves teh DDIT3 locus

77
Q

How does a myxoid tumor appear grossly?

A

Wet appearing

78
Q

What is the histo of a myxoid tumor?

A

Chicken wire vascular appearance

Scattered lipoblasts

79
Q

What are characteristics of pleomorphic liposarcoma?

A

Genetcially unstable

High grade

40-50% risk of mets

80
Q

What are characteristics of pleomorphic liposarcoma?

A

Genetcially unstable

High grade

40-50% risk of mets

81
Q

What is the histo appearance of pleomorphic sarcoma?

A

Lipoblasts in a background

82
Q

What are some tumors of skeletal muscle?

A

Rhabdomyosarcoma

Rhabdomyoma

83
Q

What is associated with cardiac rhabdomyoma?

A

Associated with Tuberous Sclerosis

Extracardiac found in HEENT

84
Q

What are the characterisitcs of a rhabdomyosarcoma?

A

Most common childhood sarcoma
Found in: HEENT, GU, Retro, extremities
Locally agressive
Mets to lung

3 subtypes

  • Embryonal
  • Alveolar
  • Pleomorphic
85
Q

What are the characterisitcs of a embryonal RMS?

A

Botyroid (bunch of grapes)

Non-uniform genetics

86
Q

What characterizes a alveolar RMS?

A

Dyscohesive

t(2:13)
t(1:13)

More aggressive than embryonal

87
Q

What are the characteristics of pleomorphic RMS?

A

Genetically unstable

High grade

ADULTs

80-90% mets risk at 5 years

88
Q

What do all subtypes of RMS express?

A

Desmin, Myogenin, MyoD1

89
Q

How do you distinguish its a pleomorphic RMS?

A

Desmin stain

90
Q

What are characteristis of hemangiomas?

A 
Multiple subtypes
Juvenile(port wine), regresses on its own
Capillary
Cavernous
Venous
Lymphangioma
91
Q

What is a pyogenic granuloma?

A

Lobular capillary hemangioma

Superficial, polypoid, can see in the gums, looks like a bud/adenoma polyp

92
Q

What are characterics of angiosarcomas?

A

Rare

Adults

93
Q

What are characterics of angiosarcomas?

A

Rare
Adults
Associated with exposures

Irregular vascular channels lined by atypical cells

Very poor prognosis

94
Q

What markers are used to identify a angiosarcoma?

A

CD31
CD34
vWF
ERG

95
Q

What is a synovial sarcoma?

A

Young adults 20-40

Monophasis (spindle cell

Biphasic (spindle cell and epithelial component)

96
Q

What is the translocation seen in synovial sarcoma?

A

t(X:18) detected by FISH/RT-PCR

97
Q

What does a synovial sarcoma overexpress?

A

TLE1, use in immunohistochemistry

98
Q

What do synovial sarcomas typically express?

A

Cytokeratin and EMA

5 year survival: 60%
10 yeaer: 40%

Path II (11 decks)

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