COMPASSIONATE ALLOWANCE INFORMATION

SOFT TISSUE SARCOMA

ALTERNATE NAMES

Malignant Soft Tissue Sarcoma; Malignant Soft Tissue Tumor; Malignant Mesenchymal Tumor ; Mesenchymal Sarcoma

DESCRIPTION

Soft Tissue Sarcoma is cancer that develops in the connective tissues (e.g. skeletal muscles, tendons, fat, fibrous tissues, deep skin tissues, and blood vessels) and the peripheral nervous system. Adult soft tissue sarcomas can form almost anywhere in the body, but are more common in the head, neck, arms, legs, trunk, and abdomen. In children, the tumors form most often in the arms, legs, or trunk (chest and abdomen). The exact cause of soft tissue sarcoma is unknown. Individuals with environmental exposure to high levels of some chemicals, radiation, or have certain genetic conditions may be at risk for developing soft tissue sarcoma. Soft Tissue Sarcoma with distant metastases or recurrent is considered an aggressive tumor because it often quickly spreads from the original site to other parts of the body such as lungs and lymph nodes.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING

Diagnostic testing: The definitive diagnosis of soft tissue sarcoma is based on histologic findings; incisional biopsy; core biopsy; excisional biopsy; immunohistochemistry, cytogenic and molecular studies; frozen section and intraoperative cytology; fluorescence in situ hybridization (FISH); and flow cytometry. Biopsy reports are definitive.

Physical findings: The physical findings are dependent upon the location of the tumor mass and may include localized pain; swelling and warmth at the site of the tumor; fever; and unexplained weight loss.

ICD-9 code: 171.X and other codes, depending upon the site

ONSET AND PROGRESSION

The prognosis of soft tissue sarcoma depends on the type of soft tissue sarcoma; the size of the tumor at first diagnosis; location of the tumor and spread (metastasis) to other organs; whether all of the tumor is removed by surgery; age of the individual; and recurrence. The rate at which these tumors grow is variable. Soft tissue sarcomas are generally large at the time of first diagnosis because they tend not cause any symptoms, until the tumor starts to push aside normal tissue, creating lumps, swelling and other symptoms such as pain or soreness. When soft tissue sarcomas spread to other sites (metastasize) they can have a poor prognosis.

TREATMENT

The treatment of soft tissue sarcomas are based on clinical presentation of local vs. metastatic disease. The main treatment of soft tissue sarcoma is surgery, chemotherapy, and radiation, virtually always used in combination.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for evaluation:

• Clinical history and examination that describes the diagnostic features of the impairment.

• Imaging tests.

• Surgical procedures.

• Pathology reports of biopsy.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets Listing

13.04 A or B

113.03

Listing level severity must be documented. To meet listing 13.04, the cancer must have regional or distant metastases (13.04A), or be recurrent following initial antineoplastic therapy (13.04B).

Equals Listing

* Adjudicators may, at their discretion, use the Medical Evidence of Record or Listings suggested to evaluate the claim. However, the decision to allow or deny the claim rests with the adjudicator.