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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
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.
Lower Extremities:
Thigh: Femur
Knee: Patella
Leg: Tibia and Fibula
Foot: Tarsals, metatarsals, and phalanges
Superficial:
Epitrochlear 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
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
RADIATION THERAPY
The location of the scar should be oriented strategically in surgery when
RADIATION THERAPY
The location of the scar should be oriented strategically in surgery when
RADIATION THERAPY
The location of the scar should be oriented strategically in surgery when
SIDE EFFECTS
Surgery:
Deprive skin of elasticity
Wound healing complications
Subcutaneous tissue damage
Radiation therapy:
Fatigue
Skin reaction
Delayed wound healing
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
VIRUS TREATMENT
Tumor necrosis factor-alpha (TNF-a) makes the chemotherapy drugs go
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
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
SIDE EFFECTS
There were no adverse side effects with the virus experienced by the rats, which shows
Infection
Bruising/bleeding
Fatigue
Diarrhea
Sore mouth
Mouthwash
Hair loss
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
Doxorubicin (50mg/m2 day-1) and ifosphamide (2000mg/m2 day-1 to day-5 with mesna
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%
BEST TREATMENT?
BEST TREATMENT?
VIRUS
BEST TREATMENT?
VIRUS
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
REFERENCES
Hackworth, R. Soft Tissue Sarcoma. [PowerPoint]. The Ohio State University Radiation Therapy Program; 2013.
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].