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EXTREMITY

Soft Tissue Sarcoma


Kylee McConnell

ETIOLOGY
Median Age: 40-60 years
Sporadically occurs
Cause is undefined

Possible causes:
Genetics
Environmental exposure
Epstein Barr virus (HIV/herpes)
Previous radiation

EPIDEMIOLOGY
Mesenchymal tumors that are rare
Originate from connective tissue
Around 50 different subtypes
Approximately 100 benign tumors for each malignant tumor of soft tissue
Commonly found in bones and joints (connective tissue)
Children: 15%
Adults: <1%

PRESENTATION
Lower extremities: 45%
Trunk: 30%
Upper extremities: 15%
Head and Neck: 8%

HISTOLOGY
Malignant Fibrous Histiocytoma: 20-30%
Most common in adults

Liposarcoma (fat): 10-20%


Leiomyosarcoma (smooth muscle): 5-10%
Fibrosarcoma: 5-10%
Synovial cell sarcoma: 5-10%
Rhabdomyosarcoma: 5-10%
Most common in peds

Schwannoma (Schwann cells/nerves): 5-10%

SYMPTOMS
Do patients present with symptoms right away for a diagnosis to be

made immediately?

SYMPTOMS
Very few early symptoms are recognized.

SYMPTOMS
Very few early symptoms are recognized.
Most patients will have this disease 4-6 weeks before symptoms or diagnosis.
Diagnosis and treatment is often delayed.

Painless mass that gradually increases


Occasionally, it will present with pain secondary to pressure effects or

direct invasion of neural structures by the tumor.

Are extremities part of the appendicular or axial skeleton?

ANATOMY & PHYSIOLOGY


Appendicular skeleton

ANATOMY & PHYSIOLOGY


Appendicular skeleton
Upper extremities:
Arm: Humerus
Forearm: Radius and ulna
Hand: Carpals, metacarpals, and phalanges

Lower Extremities:
Thigh: Femur
Knee: Patella
Leg: Tibia and Fibula
Foot: Tarsals, metatarsals, and phalanges

UPPER EXTREMITY LYMPH DRAINAGE


Divided into deep and superficial
Deep:
Axilla: drain the upper extremity, breast, and lower part of neck
About 20-30 nodes in the axilla

Superficial:
Epitrochlear nodes

LOWER EXTREMITY LYMPH DRAINAGE


Popliteal:
Drains to the deep inguinal nodes

Superficial nodes:
Lie above the termination of the great saphenous vein
Drain the skin from all areas lying below the umbilicus

Subinguinal nodes:
Superficial and deep nodes

STS
Grows by local extension
Infiltrates adjacent tissues and extends along tissue planes
At diagnosis, 90% of patients with STS will have localized disease.

METASTASIS
Eventual tumor spread to other sites is the most common form of

failure, especially for large, high-grade tumors.


The most common metastatic site from STS of the extremity is the lung

through the bloodstream.


Other sites may include:
Liver
Nodes

TREATMENT
Stage 1: Surgery alone (unless close or positive margins, then post op

RT)
Stage II-III: Surgery and post-op radiation therapy (60-66 Gy) OR pre-op

RT (50 Gy) then surgery


Stage IV: Possible surgical resection or chemo

RADIATION THERAPY
The location of the scar should be oriented strategically in surgery when

adjuvant radiation therapy is going to be used.


Should be vertical along the limb and lateral if possible.

Immobilize to minimize set up error

RADIATION THERAPY
The location of the scar should be oriented strategically in surgery when

adjuvant radiation therapy is going to be used.


Should be vertical along the limb and lateral if possible.

Immobilize to minimize set up error

Why should it be lateral?

RADIATION THERAPY
The location of the scar should be oriented strategically in surgery when

adjuvant radiation therapy is going to be used.


Should be vertical along the limb and lateral if possible.

Immobilize to minimize set up error

Why should it be lateral?


Lymph drainage medially is generally richer.

RADIATION THERAPY (POST OP)


Field includes tumor bed, scar, drainage sites, and 5-7 cm longitudinal

and 2-3 cm laterally.


IMRT
Approximately 28 fxs using 180-200 cGy/fx
Treat to 50 Gy
Then reduce field to surgical bed with 2 cm margin up to 60-66 Gy
If gross disease remains, then up to 72-76 Gy

Always spare strip of skin to allow lymph drainage to the extremity


Spare one joint

SIDE EFFECTS
Surgery:
Deprive skin of elasticity
Wound healing complications
Subcutaneous tissue damage

Radiation therapy:
Fatigue
Skin reaction
Delayed wound healing

SURVIVAL & PROGNOSIS


In extremities only:

Once disease has spread from the arms and legs, survival decreases.

VIRUS TREATMENT
To avoid amputation of an extremity, studies show that viruses that find

and kill cancer cells can enhance the chemotherapy effect


Used for smallpox vaccinations

Administered intravenously through the affected limb only, using

multiple cycles spanning over a few months.


Used in conjunction with isolated limb perfusion chemotherapy

(melphalan) and TNF-a


Done by using a lung or heart bypass machine connected to the extremity so it is

not exposing the rest of the body.


Allows large doses of the drug to be given
Used as a last resort for patients with advanced cancer

VIRUS TREATMENT
Tumor necrosis factor-alpha (TNF-a) makes the chemotherapy drugs go

through the veins to the tumor more easily.


Targets TAV (tumor-associated vasculature)
Destructs the tumors vascular lining
Causes hyperpermeability

Works synergistically with the chemo and virus to enhance the effect
Causes chemo to accumulate only near the tumor
High doses of TNF-a will cause toxicity of endothelial cells
This helps with the hyperpermeability

Will not effect or kill healthy vessels


Causes rapid responses from the chemo

VIRUS TREATMENT
This was tested on rat sarcoma
Found that the combination controlled and decreased tumor growth
Increased survival by half compared to the melphalan and TNF-a alone.

Since the appendages are separate from the body, it makes it more

difficult for the immune system to rid of these viruses.


Although viruses show to be very effective, they prove not to be so

effective when used alone.


The virus, along with isolated limb perfusion chemotherapy, was very

successful used in conjunction with each other rather than being


administered separately.

SIDE EFFECTS
There were no adverse side effects with the virus experienced by the rats, which shows

how safe it is.


Melphalan produces side effects like:

Infection

Bruising/bleeding

Anemia (low RBCs)

Fatigue

Diarrhea

Sore mouth

Gently clean teeth every morning/night and after every meal,

Soft bristle toothbrush

Mouthwash

Drink plenty of fluids

Hair loss

Skin changes (creams or medications)

PROGNOSIS/SURVIVAL
Do you think the prognosis and survival will be good or bad?

PROGNOSIS/SURVIVAL
Do you think the prognosis and survival will be good or bad?
Good

PROGNOSIS/SURVIVAL
Do you think the prognosis and survival will be good or bad?
Good
Doubles survival

PERI-OPERATIVE BRACHYTHERAPY
Interstitial brachytherapy implantation delivers a high dose right around the tumor

bed.
A wide excision is used to implant brachy catheters.
Implants are then irradiated 3-5 days after surgery BID using HDR at a dose of 3 Gy.
EBRT is then used giving a dose of 40-50 Gy.

Surgical clips that are radio-opaque cover a 4 cm margin around the tumor.
The CTV is the entire tumor with 2 cm margins.
Used in pts with surgical margins that are narrow, compartmentalized, surrounded by

vasculature and with high grade and recurrence rates.


Chemo needs to be used in conjunction.

Doxorubicin (50mg/m2 day-1) and ifosphamide (2000mg/m2 day-1 to day-5 with mesna

uroprotection) for 6-cycles


The regimen is repeated every 4 weeks.

PERI-OPERATIVE BRACHY

SIDE EFFECTS
Subcutaneous fibrosis
Wound rupturing and discoloration

PERI-OPERATIVE BRACHY
2 year local control rate: 88%
Reduces recurrence from18% to 3%

2 year survival rate: 50%


Survival rate is still low because of pulmonary mets from larger tumors.

SIDE EFFECTS COMPARISON


Far less worse for virus treatment than with peri-operative brachy
No side effects experienced with the virus
Because the virus is isolated in the limbs

Minimal side effects with melphalan chemo


Fatigue, hair loss, skin changes, diarrhea, bruising, etc.

Fibrosis and wound dehiscence with brachy

BEST TREATMENT?

BEST TREATMENT?

VIRUS

BEST TREATMENT?

VIRUS

Only used on affected limb alone


Makes more difficult for body to attack virus

Will not effect or kill healthy vessels


Rapid responses
Controlled and decreased tumor growth
No side effects from virus
Increased survival by half
Non-invasive

DRAWBACKS OF OTHERS
Therapy & Surgery:
More dose
Wound healing complications
More side effects

Brachy:
Did not use correct fractionation to discern true effect
Should be more prolonged than 3-5 days

Worse side effects


Longer
Surgery, then brachy, then radiation

Decreased survival rate

REFERENCES

Hackworth, R. Soft Tissue Sarcoma. [PowerPoint]. The Ohio State University Radiation Therapy Program; 2013.

Community College of Rhode Island. The Upper Extremity. [PowerPoint].


faculty.ccri.edu/egoffe/Upper%20Extremity.ppt. Accessed [October 26, 2014].

Cast Online. Bones of the Lower Extremity. [PowerPoint].

Hackworth, R. Lymph Nodes per Site. [PowerPoint]. The Ohio State Radiation Therapy Program; 2013.

Chao C and Goldberg M. Surgical Treatment of Metastatic Pulmonary Soft-Tissue Sarcoma. Oncology. 2000; 14(6):835-841.
http://www.cancernetwork.com/sarcoma/surgical-treatment-metastatic-pulmonary-soft-tissue-sarcoma-1. Accessed [October 27,
2014].

Survival by Stage of Soft Tissue Sarcoma. American Cancer Society. 2013. http://www.cancer.org/cancer/sarcomaadultsofttissuecancer/detailedguide/sarcoma-adult-soft-tissue-cancer-survival-rates. Accessed [November 5, 2014].

Pencavel T, Wilkinson M, et al. Viral Therapy Could Boost Limb-saving Cancer Treatment. Institute of Cancer Research. 2014.
http://www.sciencedaily.com/releases/2014/07/140722091559.htm. Accessed [October 26, 2014].

Van Horssen R, Hagen T, et al. TNF-a in Cancer Treatment: Molecular Insights, Antitumor Effects, and Clinical Utility. The
Oncologist. 2006; 11:397-408. http://theoncologist.alphamedpress.org/content/11/4/397.full.pdf+html. Accessed [October
26, 2014].

Biswal B, Wan Z, et al. Peri-Operative Brachytherapy in Soft Tissue Sarcomas. The Internet Jounal of Oncology. 2009;7(2).
http://ispub.com/IJO/7/2/4178. Accessed [October 26, 2014].

http://www.castonline.ilstu.edu/mccaw/KNR181/Lower%20Extremity%20Skeleton/Bones%20of%20Lower%20Extremity%20I.pdf.
Accessed [October 26, 2014].