soft tissue Flashcards
Lesions you can treat with photodynamic therapy?
Aktinic Keratosis
Superficial BCCs
SCC in situ
Sarcoma T staging
T1: <5cm
T2: 5-10 cm
T3: 10-15 cm
T4: >15 cm
Major contraindication to limb sparring surgery for STS?
If nerve function is already gone then no point in limb sparring
STS involvement of aorta and IVC
Sideclamp IVC, fix hole with a bovine pericardial patch
resect segment of aorta, do an interposition graft
Chemical risk factor for SCC?
Arsenic
Topical interferon response rate?
50-80% regression in non-melanoma skin cancer.
Preop RT for extremity sarcoma?
yes for all G2-G3 [nccn catagory 1]
Other flaps for closure of melanoma defect
Keystone flap - limbs
hatchet flap - large areas with a lot of tension
vemurafenib/trametinib during pregnancy?
class D
penile radiation?
outcomes are generally non-functional. Would not reccomend
How do you do an ILP of the lower extremity?
cannulation of the external iliac vessels via a hockey stick incision for thigh sarcoma
connect cannula to the inflow and outflow lines of an extracorporeal bypass circuit. (prevents hypoxia, allows longer run time)
A tourniquet is applied around the root of the extremity
SLNB for MCC?
guides RT
Rate of SLN positivity for T1b melanoma?
8%
Differences between ILI and ILP
ILI circulates blood in the isolated extremity at a much slower rate than ILP and for only 30 minutes.
During ILI, the extremity is hypoxic, which leads to marked acidosis.
ILI uses standard chemotherapy drugs and not TNF.
S100 and Sox10 in a retroperitoneal mass
Malignant PEripheral Nerve Sheath Tumor
NCI 1998 EBRT RCT for extremity sarcoma?
High grade had 0 v 22% LR at 10 yrs (p = 0.003)
Low grade had 4% v 33% LR at 10 yrs (p = 0.016)
Supplement to reduce risk of BCC/SCC?
Vitamin A
adjuvant therapies for desmoplastic melanoma?
Generally a lower threshold for RT but no prospective data.
66% improved local control with RT; have a higher local recurrence rate via retrospective data.
IS there a specific biomarker for olaratumab?
no
When do you do SLNB for desmoplastic melanoma?
Same for regular melanoma, no randomized data, but no evidence of greater LN mets.
Melanoma on plantar surface of foot?
let granulate from below
do a STSG from other part of the plantar surface of the foot. (glabrous skin)
High risk subtypes of BCC?
Infiltrative
Morpheoform
Radiation margin for EBRT for soft tissue sarcoma
5cm
High risk BCC with nerve involvement?
spare nerve and add RT
Neoadjuvant chemo for extremity STS?
would heavily emphasize tumor board and clinical trial enrollment.
Risk factors to add RT for desmoplastic melanoma?
Logical to recommend for most, but especially:
T3 or higher
perineural invasion
Head and Neck
Radiation margin for MCC
5cm
additional test for sarcomatous transformation of a DFSP?
send FISH for a translocational fusion gene
collagen to PDGFbeta
Treatment for cutaneous horn?
excise to subdermal fat to remove the base.
Agents used in isolated limb infusion for STS
TNF alpha - only available in Europe, or a trial
melphan
interferon
two radiosensitive STS
synovial cell sarcoma
myxoid sarcoma
Actinic Keratosis cancer risk?
10% become SCC
Radiation dosing for MCC primary
R0: 50-56 Gy
R1: 56-60 Gy
R2/definitive: 60-66 Gy
Sarcoma near the popliteal fossa
find the nerves proximal and trace them inferiorly
Merkel Cell Polyomavirus
DNA virus
almost all humans get at some point
mild respiratory symptoms in adolescence/late childhood
only causes cancer in very small % of those infected
virus is permanently integrated into the cancer cells, so not infectious.
What margin is most important for BCC?
deep
Olaratumab and OS of sarcoma
1 year improvement
Metastatic MCC
No big NCCN reccomendation, would present at tumor board, try aggressive surgery and radiation for a fit patient.
Any increased toxicity by adding Doxorubicin or Ifosfamide to RT for STS?
No
Medical treatment for desmoid after delivery?
tamoxifen and NSAIDs
hibernoma
benign retroperitoneal tumor
Radiation dosing for MCC nodal basin
high risk but SLN not done: still do 46-50 Gy
SLN- : no RT
SLN+: 50-56 Gy
cystic lymphangioma
benign retroperitoneal tumor
High risk pathology for BCC
subtypes other than nodular or superficial
perineural involvement
Do margins ever impact OS in any of the margin trials for melanoma?
No
What to add for epitheliod sarcoma and clear cell sarcoma?
Consider adding PET or other modality to look for lymph node metastasis.
Imatinib dosing
400mg for exon 11
400mg bid for exon 9
When to do neoadjuvant RT for RP sarcoma?
when an R2 resection is otherwise expected.
Always better to do RT upfront
Non-surgical considerations for patient with Li Fraumeni syndrome?
radiation causes secondary tumors consider avoiding radiation therapy;
get a surveillance MRI instead of surveillance CT scan
Original RCT showing equivalent OS between amputation and limb sparring surgery with adjuvant RT.
Steven Rosenberg at NCI, published in 1982
Treatment for Actinic Keratosis?
topical 5-fu
Things to add at the end of an inguinal lymph node dissection?
preserve the saphenous
do a sartorius transposition flap
Most common retroperitoneal sarcomas?
liposarcoma
leiomyosarcoma
Incidence of metastatic disease with a Marjolin’s Ulcer?
20-30%; Would get systemic staging for all.
What is “image guided therapy” RT for sarcomas?
re-image during therapy to reduce dosage as tumor shrinks.
Response rate to ILI
85-90%
Surgical approach to retrohepatic sarcoma
hockey stick Makuuchi incision
Mobilize the liver
dissect out the hepatic veins and control
Be prepared to take right kidney.
Can take left renal vein and let left kidney drain via the gonadal. (test clamp)
Preop workup for DFSP?
consider MRI if big.
Sox 10
immunostain for desmoplastic melanoma
OB/GYN asks you to resect a desmoid at the time of cesarian section?
No; tumor will regress (see above)
Is estimated tumor necrosis standardized by pathologists in STS?
no (but it is in osteosarcoma and Ewings…)
ILI response rate
about 40%
MDM2 amplification
Liposarcoma
Two STS that can go to lymph nodes?
Epitheliod sarcoma
Clear Cell sarcoma
smooth muscle actin and desmin
Leiomyosarcoma
isolated limb infusion
vessels accessed by VIR, Tourniquet is applied
No ECMO, so is acidotic/hypoxic
Sarcoma grading
2-8 point scale based on tumor differentiation (1-3) mitotic count (1-3) tumor necrosis (0-2)
5 year survival extremity sarcoma all comers?
50-60%
High risk tumor factors for BCC/SCC
any H area of central face, genitals, hands or feet
>1cm HEENT
>2cm Trunk/limbs
Which is more cosmetically acceptable for NMSC treatment, RT or surgery?
surgery!
Risk of SCC after organ transplant?
50% at 20 years.
Theoretical arguments for neoadjuvant RT for STS?
RT works better due to less hypoxia smaller field (Rad/Onc can aim) no delays due to surgical complications
Most common location of desmoplastic melanoma?
Head and neck
Prognosis of desmoplastic v mixed desmoplastic melanoma?
pure desmoplastic has better prognosis than mixed desmoplastic.
Radiation dosing for SCC
can go up to 70 Gy for macroscopic disease.
Basal Cell Nevus Syndrome
multiple BCC
Skeletal abnormalities
1st BCC in 20s
average life expectancy with strict sun avoidance
Merkel Cell management?
do the SLNB
can choose to irradiate the draining bed instead of CLND
adrenal angiomyelolipoma
benign retroperitoneal tumor
EORTC 62961
better local control for regional hyperthermia plus chemotherapy than for chemotherapy alone for those patients with the highest risk of local recurrence
Desmoid first step?
repeat CT scan in 3 months unless exceedingly symptomatic
Isolated Limb Infusion
surgical isolation of the vascular inflow and outflow of an extremity to separate the circulation of the affected limb from that of the remainder of the body
Sarcoma staging
Stage I - any G1
Stage II - T1 G2-3
Stage III T2+ G2-3
Stage IV any nodes or distant mets
Recurrent BCC
refer for Mohs surgery
How do you do an ILP of the upper extremity?
Cannulate axillary artery and vein
connect cannula to the inflow and outflow lines of an extracorporeal bypass circuit.
A tourniquet is applied around the root of the extremity
Non-surgical option for problematic desmoid during pregnancy?
low dose doxorubicin (established as safe from breast cancer series)
myxoid liposarcoma staging
needs full body CT scan
Stage IV recurrence of BCC
clinical trial or Vismodegib (hedgehog inhibitor)
immunotherapy during pregnancy?
not recommended on basis of mechanism; no data yet class D
Rate of compartment syndrome after ILP?
3% -
bad histologies for SCC
acanthotic
adenosquamous
desmoplastic
metaplastic
Mechanism of Imiquod
topical modulator of immune response
SMAC Metanalysis of RCTs for adjuvant chemo after resection of STS?
doxorubicin ifosfamide had improved OS with p = 0.01
Melanoma during pregnancy?
Be aggressive
transplacental metastasis of melanoma to baby has been reported.
RT for gorlins syndrome?
contraindicated
preventative drugs for SCC
oral retinoids (Vit A) - teratogenic nicotinamide (Vit D3)
Survival after metastasectomy for stage IV sarcoma?
40-50% 3 year survival
Unresectable RP sarcoma
Bilateral Renal artery involvement
Portal Vein/SMA encasement
Aortic encasement without reconstruction option
IVC encasement without reconstruction option
larotrectinib?
FDA approved TRK Inhibitor, for Patients with Advanced Solid Tumors Harboring an NTRK Gene fusion
Response rate for desmoids for most systemic therapies?
20%
Stage IV/unresectable Merkel Cell Carcinoma
Pembolizumab
first line systemic agent for disseminated MCC?
clinical trial
avelumab/pembrolizumab (combined PD-1 PDL-1 blockade) (better PFS than chemo in early trials)
Recurrent abdominal liposarcoma in 3 locations, what next?
re-resect
[TARPS study, Cancer 2017]
Time frame to regression of desmoid after delivery?
1-2 years
Is there a definitive RCT for melanomas between 1 and 2 mm for margin?
No
Where is RT contraindicated for NMSC?
Hands, feet and genitals
high risk pathology for SCC
poorly differentiated
bad histologies
>6mm deep
perineural invasion
SMI 2008 ILI series for extremity sarcoma?
ILI with melphan/actinomycin D
similar outcomes to ILP in europe
What percent of melanoma patients get in transit disease?
3-10%
score for a G2 sarcoma
4-5
4 most common extremity sarcomas?
undifferentiated pleomorphic
liposarcoma
synovial sarcoma
fibrosarcoma
retroperitoneal sarcoma with hepatic vein involvement?
Be prepared to go on ECMO
FDA approved targeted therapies for metastatic sarcomas?
trabectedin - intercalating agent
eribulin - microtubule inhibitor
olaratumab - PDGFR blocker
pazopanib - dirty TKI
pattern of metastasis for myxoid liposarcoma?
liver instead of lung
Leiomyosarcoma recurrence pattern?
highest risk of metastatic over local recurrence.
use to support systemic chemo in adjuvant setting.
SCC when to do lymphnode dissection
selective dissection if solitary node <3 cm
comprehensive nodal dissection if >3 cm or multiple
metastatic pattern of pleomorphic sarcoma?
can met outside the lung, get full body CT
Mitotic count sarcoma grade score:
1 - <10 mitosis/10hpf
2 - 10-20 mitosis/10hpf
3 - > 20 mitosis/10hpf
Gorlin syndrome synonym
Basal Cell Nevus Syndrome
Skin biopsy - spindle cells with storiform or fascicular pattern. Bland with minimal cytologic atypia.
dermatofibrosarcoma protuberans
AIM chemotherapy
doxorubicin, ifosfamide, and mesna
biopsy of extremity sarcoma
plan with incision
Time to recurrence after ILI
about 2 years
staging for a well differentiated liposarcoma
CXR
Local control rate/cure for definitive radiation for BCC/SCC?
> 90%
When to biopsy RP mass
after you ruled out pheochromocytoma
before you plan non-surgical therapy
response to HILP for melanoma?
50% will have a complete response
50% of the responses will eventually recur
When to consider observation for a GIST?
if <2cm and no suspicious features on EUS or other imaging
Small leiomyosarcoma encircling the left renal artery, what next?
Neoadjuvant radiation since high risk of positive margins.
chemo agent for ILI
only melphan used in any country
Stat6
Solitary Fibrous Tumor
Immunostains for MCC
positive for CK20
negative for TTF1
Merkel cell polyomavirus T antigen
Chemo used in ILI
doxorubicin
RP Sarcoma staging
CT chest (add belly for some)
No PET
do Renal Scan if near kidney
Get multidisciplinary review
Adjuvant RT for MCC after completion LND?
only for multiple involved nodes and or the presence of extra-capsular extension.
Send sarcoma to look for MSI?
yes, FDA approval includes sarcomas; would probably also consider clinical trials and only do when in disseminated StageIV
When to get nodal RT for SCC
could give to all, but consider observation for single node < 3cm with no ECE
Size cutoff for biopsy of lung nodules for STS?
1 cm
Cutaneous horn cancer risk?
10% have an SCC at the base
schwannoma
benign retroperitoneal tumor
Risk of metastasis with G2-3 extremity sarcoma?
40-50% of all patients will eventually develop
Utility of imaging for staging imaging during pregnancy?
12% of MRIs done during pregnancy have incidental findings
5 prognostic variables for STS
Size >5 cm Deep to fascia High grade tumor location (trunk higher risk) local invasion
Suspect retroperitoneal lymphoma, what to check for?
additional lymphadenopathy
First lab test when working up a desmoid tumor
pregnancy test
SLN+ on an MCC
look for a clinical trial and consult tumor board
can do RT, completion LND or both.
Mechanism of topical interferon?
Stimulates a T-cell response.
Isolated Limb Perfusion agent
melphan in US, reached 20x systemic doses with minimal systemic toxicity
How do you do a Stage I sarcoma operation?
include biopsy site with incision
2cm margins if within compartment and not taking neurovascular tissue
Place clips
place drain near incision in case needs RT
Ink margins with pathologist
ILI v ILP mnemonic
isoloated limb INfusion is minimally INvasive
IMRT v conventional radiaiton for sarcomas?
can reduce dose of radiation
Do SLNB for BCC or SCC?
Just don’t do it on oral boards, no evidence to establish it as helpful.
typical pigmentation of desmoplastic melanoma?
amelanotic - possibly explains why presents at later stages
RT to primary site for MCC
do for all except tumor <1cm with no high risk features (LVI, immunosuppression)
PET avid liposarcoma?
More likely to be high grade and therefore chemo/radiosensitive.
Indications for CLND?
> 3 positive SLN
ECE
immunosuppressed patient
poor compliance/access to ultrasound surveilance
metastatic pattern of epithelial sarcoma?
can metastasize to nodes
T1a SCC of the penis
Tumor invades subepithelial connective tissue without lymph vascular invasion and is not poorly differentiated (ie, NOT grade 3-4)
ILP technique
cutdown on inflow and outflow to limb
hook up to ECMO machine
perfuse 1-1.5 hrs at 39 C
Surgery for Stage II/II (G2/G3) extremity sarcoma?
wide excision with limb sparing en bloc R0 resection
Fibrosarcomatous transformation of a DFSP?
cytologic atypia
mitotic activity >5/HPF
negative CD34 staining
How do you do an ILI of the lower extremity?
cannulation of the external iliac vessels via a hockey stick incision for thigh sarcoma
connect cannula to the inflow and outflow lines of an extracorporeal bypass circuit.
A tourniquet is applied around the root of the extremity
SLNB for SCC of penis?
Yes for T1b or higher. do the completion LND
High risk patient factors for BCC/SCC
immunosuppresion
previous RT
Radiation margin for brachytherapy for soft tissue sarcoma
2 cm
Recurrence rates afte Mohs
BCC
SCC
BCC 1%
SCC 3%
which limb chemotherapy technique can be repeated?
ILI
Imaging for Merkel Cell Carcinoma?
only for clinical suspicion or SLNB+ (then get PET)
T staging for MCC
same as SCC!
T1 < 2cm
T2 2-5 cm
T3 >5 cm no invasion
T4 invades other structure
Preop chemo/chemoradiation for sarcoma
consider for all stage III (G2-3 T2+)
genetic syndrome associated with leiomyosarcoma?
hereditary retinoblastoma
CD99, EWSR-1
Ewing Sarcoma
R2 resection of RP sarcoma?
try to re-resect
Radiation
AIM is most common chemo
Recurrent retroperitoneal liposarcoma?
Consider aggressive compartmental surgery skelotonizing the ureter and vessels down to IVC/Aorta.
Only evidence for this is high risk of additional recurrence and getting back into the retroperitoneum is going to be hard.
metastatic/unresectable DFSP?
basically nothing in NCCN guidelines. Keep cutting, consider imatinib.
60% of non-melanoma skin cancers occur where?
H&N
Where do you inject blue dye?
subdermally
Melanoma incidence is?
increasing
sarcoma tumor differentiation score:
1 - normal mesenchymal tissue
2 - myxoid indeterminate structure
3 - embryonal undifferentiated
Treatment of SCC of the penis
T1a - local skin excision or Mohs and grafting
T1b or worse - partial or complete amputation
2011 Dutch series ILP for extremity sarcoma?
ILP with TNFalpha and Melphan
71% response
81 % limb salvage
40% overall survival
SCC with rapid growth or neurologic symptoms?
automatic high risk. make sure it goes to resection (Mohs v 1 cm margins)
Modifiable risk factor for melanoma?
ask about tanning bed use.
Desmoid growing during pregnancy
still watch, this is typical
How do you do an ILI of the upper extremity?
Cannulate axillary artery and vein
connect cannula to the inflow and outflow lines of an extracorporeal bypass circuit.
A tourniquet is applied around the root of the extremity
How do you do photo-dynamic therapy?
Apply photosensitizer (Photofrin -Porfimer sodium) Shine UV light
Isolated Limb Perfusion
surgical isolation of the vascular inflow and outflow of an extremity to separate the circulation of the affected limb from that of the remainder of the body
Surveillance after BCC?
Complete skin exam q6 months.
medicine for risk reduction of BCC in gorlins syndrome
Nicotinamide
Nx for technically unsuccessful SLNB for MCC?
would radiate primary draining basin anyway
Agent with highest response rate for desmoid tumors?
sorafenib
renal angiomyolipoma
benign retroperitoneal tumor
retroperitoenal mass and scrotal mass
lost likely metastatic testicular cancer
Clinically positive node for MCC
do IR biopsy, then manage as per SLN+
look for a clinical trial and consult tumor board
can do RT, completion LND or both.
Best lesions to treat with topical 5FU
multiple superficial Basal cell carcinomas
multiple aktinic keratosis
curretage for BCC?
low risk only, never on hair bearing area
if adipose is reached then just do surgical excision with primary closure.
What to do for an infiltrative or morpheoform BCC?
Consider wider margin (1 cm) if cosmetically acceptable
Treatment for recurrent STS
If resectable -> resect
If no prior RT -> radiation (before or after)
sarcoma v renal mass, tests to get?
plasma and urine catacholamines
steroid hormones
DHEA
sarcoma tumor necrosis score:
0 - no necrosis
1 - <50% necrosis
2 - >50% necrosis
Surgical options for DFSP?
Mohs v WLE with 2-cm margins.
delayed closure
do STSG if margin is close/equivocal.
Surgery for G1 (Stage I) sarcoma
Limb sparing function preserving en bloc R0 resection
subungual melanoma?
distal phalanx amputation
Margin for a DFSP?
NCCN rx - 2-4 cm
some retrospective evidence that >3 is better than <4cm
pink/violet slowly growing lesion?
dermatofibrosarcoma protuberans
unresectable DFSP?
consider gleevec, has a characteristic PDGFR gene fusion
Anal mass S-100, HMB-45 and Melan-A positive?
anal melanoma
BRAF/MEK inhibition?
dabrafenib and trametinib
heterogenously enhancing retroperitoneal nodules
dedifferentiated liposarcoma
