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Skin Radiation therapy > sarcoma > Flashcards

sarcoma Flashcards

1
Q

age distribution OS

A

PAEDS (teenagers) and >65 yo

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2
Q

causes of OS in different aged groups

A

spontaneous in paedswith rapid bone growth and associaed with Pagets disease in adults

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3
Q

causes of OS

A

previous XRT or alkylating Cx

pagets disease, Rb, werner syndrome, Li-fraumeni, bloom syndrome

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4
Q

most common site of osteosarcoma

A

the metaphysis near the growth plate

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5
Q

parts of the long bone

A

ephysis (knob like portions on the end of the bone both sup and inf), metaphysis,both sup and inf) (At the end of the diaphysis diaphysis (main shaft of bone) cartilage cap and periosteum

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6
Q

most common site of OS

A

the distal femus

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7
Q

most common area for mets OS

A

lung via hematogenous route

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8
Q

spread for OS

A

skip lesions are common and can occur in multile parts of the same bone, LN mets is rare unless located inthe trunk and blood mets for distant spread to lung

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9
Q

OS arise from _____ cells

A

msynchyme

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10
Q

mos common OS presentation

A

local swelling and pain

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11
Q

when is bone pain most common in OS

A

More common at night

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12
Q

how common is mets in oS

A

17% at Dx or 80% at 18 mos

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13
Q

what must be kept in mind regarding the incision after a BX in the Dx of OS

A

The incision should not be in the area that will be XRT

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14
Q

Bx offered in Dx of OS

A

open biopsy and core needle Bx

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15
Q

pattern of spread OS

A

symptoms that progress rapidly over a period of weeks to months is a sign of a malignancy whereas chronic symptoms suggest a benign lesion

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16
Q

What grade is OS typically dx at

A

3-4 grade in 85% cases

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17
Q

2 main types of OS

A

Surface and intramedulary OS

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18
Q

What subtype of OS s most common

A

intramedulary

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19
Q

what age group is intramedulary OS associated with? is it good or poor prognosis

A

intramedulary is associated with adolesence and is high grade/ dx late

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20
Q

main subtypes of intramedulary OS

A

Osteoblastic: Increased bone production
Chondroblastic: high degree of hyaline cartilage
Fibroblastic: contributes to the formation of connective tissue
Mixed Chondroblastic:

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21
Q

Subtypes of surfac OS

A

Parosteal- low grade, with low metastatic potential
Periosteal- intermediate grade, intermediate risk for developing mets (20% risk)
High grade surface

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22
Q

typical tx for OS

A

Typical treatment is neoadjuvant, multi-agent chemotherapy followed by surgery. (3 course CX, LSS, 6 courses Cx)

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23
Q

reconstruction methods for after Sx in os

A

Allografts: tissue graft from a donor
Endoprosthesis: artificial prostheses
Rotationplasty: knee, bottom of the femur and the upper tibia are removed, the leg is then rotated 180 degrees and reattached at the femur

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24
Q

in what case can sx be a sole tx in oS

A

low risk pts such as parosteal tumours (SURFACE OS )

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25
Q

Primary tx for oS

A

Sx

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26
Q

surgery of choice for OS

A

n bloc resection

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27
Q

what treatment should be last resort for pts with OS how oftn us this treatment used?

A

amputation

is still used today in 20% of cases

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28
Q

what agents are used for OS cx

A

Doxorubicin, vincristine, methotrexate and cyclophosphamide are best agents to use

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29
Q

is XRT typically used in the tx of OS ? why or why not?

A

not typically used as OS is radioresistant

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30
Q

most important prognostic indicator OS

A

mets at dx

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31
Q

pst op disease dose XRT

A

55-60Gy (close but negative margins)
60-68Gy (microscopically positive margins)
>68Gy (gross disease postop)

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32
Q

Neo disease for XRT

A

50Gy

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33
Q

this pat of the bone contains the growth plate

A

metapysis

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34
Q

why is it important to spare a strip of skin in the XRT of OS

A

prevent edema and constrictive fibrosis

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35
Q

What age and gender has worse prognosis in the tx of OS

A

AGE <10 yo is worse

male has worse prognosis

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36
Q

what type of OS has worse prognosis

A

radiation induced OS is worse

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37
Q

what isx the typical size of a retroperitoeal STS at dx

A

10cm

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38
Q

what subtype of OS has the best prognosis

A

parosteal OS a subtype of surface OS

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39
Q

What dose can cause height abnormalities in children

A

> 35Gy

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40
Q

in children bones are ______. and in adults bones are _____.

A

children they are radiosensitive

adults are radioresistant

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41
Q

what age is STS most commonin

A

50-60+

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42
Q

what causes STS

A 
prev xrt, DISEASES: NF1, gardner syndrome, werner syndrome, tuberous sclerosis, gorlin syndrome, Vinyl chloride
Dioxin
Arsenical pesticides
Phenoxyherbicides
HIV and herpes simplex 8
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43
Q

smooth muscle controls _____.

striated muscl controls _____.

A

smooth muscle is NOT controlled by me

skeletal/ striated muscle IS controlled by me

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44
Q

sarcoma of smooth muscle

A

Leiomyosarcoma

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45
Q

sarcoma of striated muscle

A

Rhabdomyosarcoma

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46
Q

sarcoma of fat

A

liposarcoma

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47
Q

sarcoma of joint

A

synovial sarcoma

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48
Q

sarcoma of cartilage

A

chondrosarcoma

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49
Q

sarcoma of nerves

A

schwanoma

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50
Q

angiosarcoma is most common in wha area

A

H&N

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51
Q

How does STS typically grow

A

along the muscle longitudinally without traversing or violating the major fascial planes or bone path of least resistance in the longitudinal axis of the primary site compartment
Primary site compartment is the natural boundary around the STS primary
As the tumour grows it pushes away from the basement membrane and forms a pseudocapsule made of healthy and fibrotic tissue

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52
Q

Desmoid tumours are most common in which area

A

the abdomen

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53
Q

what STS is most common with gardner syndrome

A

desmoid tumour

54
Q

epithiliod tumours are most common in what part of the body

A

hand and forearm

55
Q

what types of STS are most common to have LN mets

A

Epithelioid carcinoma 20-30%
Clear cell carcinoma 10-18%
Rhabdomyosarcoma 20-25%
Cutaneous angiosarcoma 10-15%

56
Q

distant mets in STS

A

most common hematogenously to the lung

57
Q

what STS areas are more commonly high grade

A

head and neck and trunk

lots of lymphatics in these areas

58
Q

most common S&S of STS

A

Presents as a painless lump, gradually growing most common

59
Q

are STS usually dx early or late

A

Patients usually seek treatment late because the tumours are usually asymptomatic until they are large

60
Q

what type of tumours can cause hypoglycemia (STS)

A

Retroperitoneal liposarcoma or lipoma

61
Q

Most common area for STS

A

40-50% are in the lower extremities (thigh) next most common is in the trunk

62
Q

avg time from onset of s&s to dx for STS

A

4-6 months on avg

63
Q

biopsies for small vs large STS

A

Large Lesion= core needle biopsy (most widely used)

Small Lesion= excisional biopsy (<5cm)

64
Q

what STS areas are more commonly dx early? late?

A

H&N and distal extremities are more likely to be diagnosed early as opposed to tumours of the thigh and retroperitoneum

65
Q

diagnostic method of choice for sts

A

MRI ( soft tissue delineation)

66
Q

when is CT the imaging method of choice for STS

A

for tumours of the retroperitoneum

67
Q

4 main categories of STS (severity)

A

Benign
Intermediate, locally aggressive
Intermediate, rarely metastasizing
Malignant

68
Q

most common subtype of STS

A

MALIGNANT FIBROUS HISTOCYTOMA

69
Q

When is XRT typically given in STS

A

Neoadjuvantly as there is less SE

70
Q

Advantages of treating ST adjuvantly

A

improved wound healing

71
Q

cons of treating STS adjuvantly

A

High dose- tumour isn’t getting oxygen b/c blood vessels have been disturbed during surgery making the area hypoxic
Greater volume treated- tumour is not present to use as a guide
Greater risk of fibrosis and joint problems

72
Q

when is XRT done adjuvantly in ST s

A

FOR + MARGINS AFTER SURGERY ESPECIALLY IN THED EXTREMITIES

73
Q

Dose neoadjuvant for STS

A

50/25

74
Q

Pros of neoadjuvant XRT in STS

A

Better functional outcome
Tx volume is well defined b/c the tumour is present
Debulks
Lower dose- better blood supply before surgery
Smaller tx volume
Reduces the risk of seeding during surgery

75
Q

Cons of neoadjuvant XRT in STS

A
  • WOUnd healing after surgery
76
Q

what magins should be given (general) for sts XRT

A

the entire compartment

77
Q

adjuvant margins high vs low grade for STS

A

High Grade: 10cm on surgical scar

Low Grade: 5cm on surgical scar w/ cone down on 2cm around tumour bed

78
Q

Neoadjuvant margins high vs low grade STS

A

High Grade: 8-10cm on tumour

Low Grade: 5cm on tumour

79
Q

What STS tumour types have LN included in tumour voulme

A

not included in these techniques except for: Rhabdomyosarcomas, synovial sarcomas and epitheliod sarcomas

80
Q

when is chemo given in the tx of STS

A

Advanced
High risk
Metastatic
Recurrent disease

81
Q

What chemo agent is used most commonly in the tx of STS

A

Doxorubicin

82
Q

when would STS be used adjuvantly (not due to + margins)

A

Patients with subcutaneous myxofibrosarcoma where it is difficult to define the target volumes without having prior surgery
Obese, or diabetic patients who may have more wound healing problems

83
Q

doses adjuvant XRT

A

60-66 -margins

66-68 + margins

84
Q

V40 for bone

A

<64%

85
Q

mean dose to bone

A

<37Gy

86
Q

max dose to bone

A

<59Gy

87
Q

what histology has the worst prognosis for STS

A

fibrosarcoma & malignant peripheral-nerve tumours are worse

88
Q

STS sequencing in general

A

Most commonly neoadjuvant XRT 50/25
Surgery
Adjuvant Cx for high risk patients and XRT given for high risk patients also

89
Q

tx retroperitoneal STS

A

Usually XRT given neoadjuvantly 45Gy then 4 weeks before surgery
AP/PA / MLO is best, >10MV, 3-5mm margin around GTV
Surgery
Post op boost/ adjuvant XRT if indicated if surgical margins are close or + boost is given: EBRT, IORT:15Gy or interstitial implants 15Gy

90
Q

typical treatment for STS of the extremities and trunk

A

neo XRT 50/25 -> dox + ifosfamide -> surgery -> XRT boost if + margins (16-20Gy)

91
Q

most important STS prognostic factor

A

tumour stage

92
Q

what area of STS most commonly has recurrence

A

retroperitomeum (50^%) has recurrence and 20% in the extremities has recurrence

93
Q

RMS age

A

bimodal age distribution

2-6 and then adolescence

94
Q

what diseases cause RMS

A 
Li-Fraumeni Syndrome
Beckwith-Wiedemann Syndrome
Neurofibromatosis
Costello Syndrome
Gorlin basal cell nevus syndrome
Congenital pulmonary cysts 
Neurofibromatosis
95
Q

What environmental factors cause RMS

A

Paternal cigarette smoking
Prenatal x-ray exposure
Maternal recreational drug use

96
Q

RHABDOMYOBLASTS ARE____

A

Primitive skeletal muscle cells

97
Q

what locations of the body with RMS commonly have RMS? dont commonly have RMS?typically embryonal or botryoid in histology, occurs primarily in infants

A

LN spread is rare in orbital tumours
15% H&N cancer has ln spread (most common in npc)
praatesticular, trunk and extremity has 25% risk of LN spread

98
Q

what areas most commonly spread hematogenously in RMS

A

TRUNK AND EXTREMITIES MOST COMMOLNY

99
Q

Areas of distant mets in RMS

A

LUNGS BONE MARROW AND BONE

100
Q

BLADDER /VAGINAL RMS subtype

A

typically embryonal or botryoid in histology, occurs primarily in infants

101
Q

age and subtype of RMS in trunk and rxtremity

A

occur more commonly in adolescents and are aveolar or undifferentiated in type

102
Q

age and subtype of H&N RMS

A

: typically embryonal and occur throughout childhood

103
Q

What areas are embryonal subtype inRMS

A

H&N Bladder/ vaginal tumours

104
Q

What areas are alveolar and undiferentiated RMS

A

TRUNK AND EXTREMITY

105
Q

what areas are botyroid RMS

A

GYNE

106
Q

MOST COMMON PRESENTATION OF RMS

A

Asymptomatic mass

107
Q

s&s of orbital RMS

A

Proptosis and opthomalegia

108
Q

s&s of GU rms

A

Urinary difficulties
Hematuria
Constipation

109
Q

most common s&s of gyne RMS

A

FLESHY MASS EXTRUDING FROM THE VAGINA

110
Q

Most s&s of parameningeal RMS

A

Nasal, aural, sinus obstruction
Cranial nerve palsy
Headache

111
Q

MOST COMMON AREAS OF RMS

A

GU, parameningeal, extremities and orbit (most to less common)

112
Q

most common pathology of RMS

A

Embryonal 60-70%

113
Q

age most common in enbryonal RMS

A

2-6 YO

114
Q

What pathologies are good prognosis of rms

A

botyroid, and spindle cell

115
Q

intermediate prognosis of RMS

A

embryonal RMS

116
Q

poor prognosi subtype of RMS

A

ALVEOLAR AND UNDIFFERENTIATED

117
Q

Alveolar cancer is most common in what age

A

adolescents

118
Q

what subsites is Sx not done for with RMS

A

ORBIT AND GU as its difficult to resect without removing th entire organ which causes functional dysfunction

119
Q

chemo for RMS (sequencing)

A

usually neo and the followed by SX + XRT

USED IN ALL CASES

120
Q

CHEMO agents used in RMS

A

VAC (vincristine, adryamycin and cyclophosphamide)

or VAC + doxorubicin

121
Q

When is XRT the primary treatment for RMS

A

for certain subsites that are not amenable to surgery (ex: orbit, NPC

122
Q

WHEN IS xrt DONE IN SEQUENCING IN rms TREATMENT

A

Cx first-> Sx-> adjuvant xrt is the most common sequencing

123
Q

doses RMS

A

Adjuvantly
Microscopic margins 40-41.5/22
gross margins: 50.4-55.8/

124
Q

tx orbital RMS

A

not typically treated with Sx -> last resot
Chemorads is done instread
-VAC and rads started after 3-12 wks of chemo and is tx with 50HGy

125
Q

tx parameningeal RMS

A

chmorads- not typically amenable to surgery 50Gy XRT + VAC

126
Q

Tx nonparameningeal RMS

A

Neo VAC -> SX ( mor amenable to sx than parameningeal) -> XRT

127
Q

Bladder and prostate RMS

A

doxorubicin + cisplatinum ->parital cystectomy (some bladder dometumour) -> adjuvant XRT

128
Q

Tx paratesticular RMS

A

Surgery (inguinal orichedectomy) if it is completely excised this is the sole treatment- otherwise XRT will be done for pA an IL LN XRT is done

129
Q

TX RMS gyne

A

VAC follwoed by Sx gyne is chemosensitive so it usually doesnt require XRT unless there is residual disease postop

130
Q

tx extremites RMS

A

LSS -> XRT + Multiagent cx (vac)

131
Q

what RMS subsite has good progonsis

A

orbital

Skin Radiation therapy (40 decks)

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