Case Presentation

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Soft tissue sarcoma of the extremities

Soft tissue sarcoma of the extremities

The soft tissues of the body include the muscles, tendons, fat, blood vessels,
lymph vessels, nerves, and tissues around joints.

Soft tissue sarcomas are a diverse group of neoplasms that arise in the connective
tissues throughout the body.

Adult soft tissue sarcomas can form almost anywhere in the body, but are most
common in the head&neck, arms, legs, trunk, and abdomen.

Soft tissue sarcoma of the extremities

They account for only 1% of adult malignancies and about 7-15% in pediatric. 50-60% of sarcoma appear in the extremities and although very rare, account for
more death than testicular cancer, Hodgkins disease and thyroid cancer combined.

Very notorious for recurring and metastasizing often with devastating results
despite complete resection.

More common in men with 1.2:1 ratio to women. More African american cases.

Different types..

Etiology..
Factors associated with Sarcomas Genetic predisposition RBI gene defect associated with some sarcomas Radiation exposure 7-25 yrs latency period Chemical carcinogens Immunologic and viral factors

!
These factors are associated with some sarcomas and not much is known for the majority of sarcomas.

Presentation..
Majority cases present with painless mass Aggressive tumors may present pain (33%) With Malignant peripheral nerve tumors, pain is the first presenting symptom
T end to progress very fast.

Assessment..
Things to assess in the physical examination include Location of the mass its shape size consistency relation to surrounding tissues State of the regional nodes

Biopsy..
FNA/CNB - used to get sample cells/tissues for further study Incisional biopsy for masses >5cm Excisional biopsy for masses <5cm
Surgeon must be very carful to prevent inadvertent dissemination of tumor cells

Staging..

Staging..
Stage I Low grade further divided into A & B Stage IA: <5cm & Stage IB: >5cm Stage II - Medium grade Further divided into A & B Stage IIA: <5cm & Stage IIB: >5cm Stage III - >5cm high grade tumor or Lymph node +ve Stage IV - distant mets.

T reatment options..
There are three standard treatments
Surgery Radiation Chemo

Surgery..

T umor is mapped and excised layer by layer; pathologically clear Each layer is examined until resection margins are

Radiation & Chemo..

Radiation and chemo are used in conjunction with surgery.

Other treatments currently being tried include the following regional therapies Regional Hyperthermia therapy
Tissue around the tumor is exposed to high temperature either to kill directly or to increase the effectiveness of chemo drugs

New T reatments..

Isolated limb perfusion

Administering chemotherapy agents directly into the affected site by temporarily limiting blood flow to and from that area. This sends high dose of chemo to the affected area while minimizing the systemic side effect.

T argeted therapies being tested in Clinical trials

Drugs that slow/inhibit cancer growth by blocking

molecules or blood vessel formation around the tumor imatinib being used for gastrointestinal stromal tumor sunitinib shown to slow the growth of many sarcomas bevacizumab when given with doxorubicin seemed to have anti-angiogenesis effect.

My opinion..
A good approach is one that uses the combination of all of the above treatment options.

Sources..
American Cancer Society. Sarcoma-Adult soft tissue cancer. http://www.cancer.org/cancer/sarcomaadultsofttissuecancer/detailedguide/sarcoma-adult-soft-tissue-cancer-new-research. Accessed 3 November 2013. Baylor University Medical Center Proceeding. Soft tissue sarcoma of the extremities. http://www.ncbi.nlm.nih.gov/pmc/ articles/PMC1200782/#B5. Accessed 3 November 2013. articles/PMC3637638/. Accessed 3 November 2013 BioMed Center-Clinical Sarcoma Research. Bone Metastasis in Soft tissue sarcoma. http://www.ncbi.nlm.nih.gov/pmc/ MDAnderson Center. Soft tissue sarcoma treatment. http://www.mdanderson.org/patient-and-cancer-information/ cancer-information/cancer-types/soft-tissue-sarcoma/treatment/index.html. Accessed 3 November 2013. soft-tissue-sarcoma/Patient/page4. Accessed 3 November 2013 PMC3353406/. accessed 3 November 2013. National Cancer Institute. Adult soft tissue sarcoma treatment. http://www.cancer.gov/cancertopics/pdq/treatment/adultNational Institute of Health. New therapeutic target in soft tissue sarcoma. http://www.ncbi.nlm.nih.gov/pmc/articles/