COMPASSIONATE ALLOWANCE INFORMATION
Primary Angiosarcoma; Secondary Angiosarcoma
Angiosarcoma is a rare and aggressive soft tissue sarcoma that begins in the cells that line blood vessels or lymph vessels and can occur in any area of the body. Angiosarcoma most commonly present in the skin and breast, but may also occur in the liver, spleen and other deep tissues, and are frequently metastatic at the time of diagnosis. Lymphedema is a common, preceding association of angiosarcoma (for example, following breast cancer treatment). Other rare associations include prior radiation exposure or treatment, and carcinogen exposure such as vinyl chloride, arsenic and thorium dioxide.
DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING
Diagnostic testing: A biopsy is definitive. The diagnosis of angiosarcoma may involve a physical examination; imaging studies such as CT/MRI scans, PET scans and ultrasound.
Physical findings: Physical findings of angiosarcoma depend on the site of the tumor and degree of metastasis.
ICD 9: 171.9
ONSET AND PROGRESSION
Most angiosarcomas occur in adults, but may rarely affect some children. These tumors are high grade tumors that are fast growing and aggressive. Angiosarcomas are deep-seated and may go unnoticed until the late stages before they are diagnosed. Angiosarcomas metastasize freely because these cancerous cells are in the lining of blood vessels and lymph nodes. Even after surgical resection from the primary site, these tumors may recur in other locations in the body. The prognosis is poor if there is metastasis to other sites.
Treatment of angiosarcoma depends on the specific type of tumor, its size, and its location and the amount that it has spread (metastasized). The standard treatment of these tumors is a complete resection (surgical removal) of the primary tumor. If a complete resection is not feasible, radiation therapy is administered. Adjuvant chemotherapy is used in some patients with varying response to treatment.
SUGGESTED PROGRAMMATIC ASSESSMENT*
Suggested MER for evaluation:
Suggested Listings for Evaluation:
Listing level severity of soft tissue sarcomas in adults requires pathology diagnosis from biopsy tissue and either documentation of metastases, or recurrence or persistence following initial anti-neoplastic treatment. Listing level severity in children must be documented by confirmed diagnosis or recurrence. Evaluate cutaneous angiosarcoma in adults under listing 13.03A.
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* 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.