sarcoma Flashcards
age distribution OS
PAEDS (teenagers) and >65 yo
causes of OS in different aged groups
spontaneous in paedswith rapid bone growth and associaed with Pagets disease in adults
causes of OS
previous XRT or alkylating Cx
pagets disease, Rb, werner syndrome, Li-fraumeni, bloom syndrome
most common site of osteosarcoma
the metaphysis near the growth plate
parts of the long bone
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
most common site of OS
the distal femus
most common area for mets OS
lung via hematogenous route
spread for OS
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
OS arise from _____ cells
msynchyme
mos common OS presentation
local swelling and pain
when is bone pain most common in OS
More common at night
how common is mets in oS
17% at Dx or 80% at 18 mos
what must be kept in mind regarding the incision after a BX in the Dx of OS
The incision should not be in the area that will be XRT
Bx offered in Dx of OS
open biopsy and core needle Bx
pattern of spread OS
symptoms that progress rapidly over a period of weeks to months is a sign of a malignancy whereas chronic symptoms suggest a benign lesion
What grade is OS typically dx at
3-4 grade in 85% cases
2 main types of OS
Surface and intramedulary OS
What subtype of OS s most common
intramedulary
what age group is intramedulary OS associated with? is it good or poor prognosis
intramedulary is associated with adolesence and is high grade/ dx late
main subtypes of intramedulary OS
Osteoblastic: Increased bone production
Chondroblastic: high degree of hyaline cartilage
Fibroblastic: contributes to the formation of connective tissue
Mixed Chondroblastic:
Subtypes of surfac OS
Parosteal- low grade, with low metastatic potential
Periosteal- intermediate grade, intermediate risk for developing mets (20% risk)
High grade surface
typical tx for OS
Typical treatment is neoadjuvant, multi-agent chemotherapy followed by surgery. (3 course CX, LSS, 6 courses Cx)
reconstruction methods for after Sx in os
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
in what case can sx be a sole tx in oS
low risk pts such as parosteal tumours (SURFACE OS )
Primary tx for oS
Sx
surgery of choice for OS
n bloc resection
what treatment should be last resort for pts with OS how oftn us this treatment used?
amputation
is still used today in 20% of cases
what agents are used for OS cx
Doxorubicin, vincristine, methotrexate and cyclophosphamide are best agents to use
is XRT typically used in the tx of OS ? why or why not?
not typically used as OS is radioresistant
most important prognostic indicator OS
mets at dx
pst op disease dose XRT
55-60Gy (close but negative margins)
60-68Gy (microscopically positive margins)
>68Gy (gross disease postop)
Neo disease for XRT
50Gy
this pat of the bone contains the growth plate
metapysis
why is it important to spare a strip of skin in the XRT of OS
prevent edema and constrictive fibrosis
What age and gender has worse prognosis in the tx of OS
AGE <10 yo is worse
male has worse prognosis
what type of OS has worse prognosis
radiation induced OS is worse
what isx the typical size of a retroperitoeal STS at dx
10cm
what subtype of OS has the best prognosis
parosteal OS a subtype of surface OS
What dose can cause height abnormalities in children
> 35Gy
in children bones are ______. and in adults bones are _____.
children they are radiosensitive
adults are radioresistant
what age is STS most commonin
50-60+
what causes STS
prev xrt, DISEASES: NF1, gardner syndrome, werner syndrome, tuberous sclerosis, gorlin syndrome, Vinyl chloride Dioxin Arsenical pesticides Phenoxyherbicides HIV and herpes simplex 8
smooth muscle controls _____.
striated muscl controls _____.
smooth muscle is NOT controlled by me
skeletal/ striated muscle IS controlled by me
sarcoma of smooth muscle
Leiomyosarcoma
sarcoma of striated muscle
Rhabdomyosarcoma
sarcoma of fat
liposarcoma
sarcoma of joint
synovial sarcoma
sarcoma of cartilage
chondrosarcoma
sarcoma of nerves
schwanoma
angiosarcoma is most common in wha area
H&N
How does STS typically grow
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
Desmoid tumours are most common in which area
the abdomen
what STS is most common with gardner syndrome
desmoid tumour
epithiliod tumours are most common in what part of the body
hand and forearm
what types of STS are most common to have LN mets
Epithelioid carcinoma 20-30%
Clear cell carcinoma 10-18%
Rhabdomyosarcoma 20-25%
Cutaneous angiosarcoma 10-15%
distant mets in STS
most common hematogenously to the lung
what STS areas are more commonly high grade
head and neck and trunk
lots of lymphatics in these areas
most common S&S of STS
Presents as a painless lump, gradually growing most common
are STS usually dx early or late
Patients usually seek treatment late because the tumours are usually asymptomatic until they are large
what type of tumours can cause hypoglycemia (STS)
Retroperitoneal liposarcoma or lipoma
Most common area for STS
40-50% are in the lower extremities (thigh) next most common is in the trunk
avg time from onset of s&s to dx for STS
4-6 months on avg
biopsies for small vs large STS
Large Lesion= core needle biopsy (most widely used)
Small Lesion= excisional biopsy (<5cm)
what STS areas are more commonly dx early? late?
H&N and distal extremities are more likely to be diagnosed early as opposed to tumours of the thigh and retroperitoneum
diagnostic method of choice for sts
MRI ( soft tissue delineation)
when is CT the imaging method of choice for STS
for tumours of the retroperitoneum
4 main categories of STS (severity)
Benign
Intermediate, locally aggressive
Intermediate, rarely metastasizing
Malignant
most common subtype of STS
MALIGNANT FIBROUS HISTOCYTOMA
When is XRT typically given in STS
Neoadjuvantly as there is less SE
Advantages of treating ST adjuvantly
improved wound healing
cons of treating STS adjuvantly
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
when is XRT done adjuvantly in ST s
FOR + MARGINS AFTER SURGERY ESPECIALLY IN THED EXTREMITIES
Dose neoadjuvant for STS
50/25
Pros of neoadjuvant XRT in STS
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
Cons of neoadjuvant XRT in STS
- WOUnd healing after surgery
what magins should be given (general) for sts XRT
the entire compartment
adjuvant margins high vs low grade for STS
High Grade: 10cm on surgical scar
Low Grade: 5cm on surgical scar w/ cone down on 2cm around tumour bed
Neoadjuvant margins high vs low grade STS
High Grade: 8-10cm on tumour
Low Grade: 5cm on tumour
What STS tumour types have LN included in tumour voulme
not included in these techniques except for: Rhabdomyosarcomas, synovial sarcomas and epitheliod sarcomas
when is chemo given in the tx of STS
Advanced
High risk
Metastatic
Recurrent disease
What chemo agent is used most commonly in the tx of STS
Doxorubicin
when would STS be used adjuvantly (not due to + margins)
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
doses adjuvant XRT
60-66 -margins
66-68 + margins
V40 for bone
<64%
mean dose to bone
<37Gy
max dose to bone
<59Gy
what histology has the worst prognosis for STS
fibrosarcoma & malignant peripheral-nerve tumours are worse
STS sequencing in general
Most commonly neoadjuvant XRT 50/25
Surgery
Adjuvant Cx for high risk patients and XRT given for high risk patients also
tx retroperitoneal STS
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
typical treatment for STS of the extremities and trunk
neo XRT 50/25 -> dox + ifosfamide -> surgery -> XRT boost if + margins (16-20Gy)
most important STS prognostic factor
tumour stage
what area of STS most commonly has recurrence
retroperitomeum (50^%) has recurrence and 20% in the extremities has recurrence
RMS age
bimodal age distribution
2-6 and then adolescence
what diseases cause RMS
Li-Fraumeni Syndrome Beckwith-Wiedemann Syndrome Neurofibromatosis Costello Syndrome Gorlin basal cell nevus syndrome Congenital pulmonary cysts Neurofibromatosis
What environmental factors cause RMS
Paternal cigarette smoking
Prenatal x-ray exposure
Maternal recreational drug use
RHABDOMYOBLASTS ARE____
Primitive skeletal muscle cells
what locations of the body with RMS commonly have RMS? dont commonly have RMS?typically embryonal or botryoid in histology, occurs primarily in infants
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
what areas most commonly spread hematogenously in RMS
TRUNK AND EXTREMITIES MOST COMMOLNY
Areas of distant mets in RMS
LUNGS BONE MARROW AND BONE
BLADDER /VAGINAL RMS subtype
typically embryonal or botryoid in histology, occurs primarily in infants
age and subtype of RMS in trunk and rxtremity
occur more commonly in adolescents and are aveolar or undifferentiated in type
age and subtype of H&N RMS
: typically embryonal and occur throughout childhood
What areas are embryonal subtype inRMS
H&N Bladder/ vaginal tumours
What areas are alveolar and undiferentiated RMS
TRUNK AND EXTREMITY
what areas are botyroid RMS
GYNE
MOST COMMON PRESENTATION OF RMS
Asymptomatic mass
s&s of orbital RMS
Proptosis and opthomalegia
s&s of GU rms
Urinary difficulties
Hematuria
Constipation
most common s&s of gyne RMS
FLESHY MASS EXTRUDING FROM THE VAGINA
Most s&s of parameningeal RMS
Nasal, aural, sinus obstruction
Cranial nerve palsy
Headache
MOST COMMON AREAS OF RMS
GU, parameningeal, extremities and orbit (most to less common)
most common pathology of RMS
Embryonal 60-70%
age most common in enbryonal RMS
2-6 YO
What pathologies are good prognosis of rms
botyroid, and spindle cell
intermediate prognosis of RMS
embryonal RMS
poor prognosi subtype of RMS
ALVEOLAR AND UNDIFFERENTIATED
Alveolar cancer is most common in what age
adolescents
what subsites is Sx not done for with RMS
ORBIT AND GU as its difficult to resect without removing th entire organ which causes functional dysfunction
chemo for RMS (sequencing)
usually neo and the followed by SX + XRT
USED IN ALL CASES
CHEMO agents used in RMS
VAC (vincristine, adryamycin and cyclophosphamide)
or VAC + doxorubicin
When is XRT the primary treatment for RMS
for certain subsites that are not amenable to surgery (ex: orbit, NPC
WHEN IS xrt DONE IN SEQUENCING IN rms TREATMENT
Cx first-> Sx-> adjuvant xrt is the most common sequencing
doses RMS
Adjuvantly
Microscopic margins 40-41.5/22
gross margins: 50.4-55.8/
tx orbital RMS
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
tx parameningeal RMS
chmorads- not typically amenable to surgery 50Gy XRT + VAC
Tx nonparameningeal RMS
Neo VAC -> SX ( mor amenable to sx than parameningeal) -> XRT
Bladder and prostate RMS
doxorubicin + cisplatinum ->parital cystectomy (some bladder dometumour) -> adjuvant XRT
Tx paratesticular RMS
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
TX RMS gyne
VAC follwoed by Sx gyne is chemosensitive so it usually doesnt require XRT unless there is residual disease postop
tx extremites RMS
LSS -> XRT + Multiagent cx (vac)
what RMS subsite has good progonsis
orbital
