ANGIOSARCOMA
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ALTERNATE NAMES
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Primary Angiosarcoma; Secondary Angiosarcoma
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DESCRIPTION
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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.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM CODING
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Diagnostic testing: A biopsy is definitive. The diagnosis of angiosarcoma may involve:
Physical findings: Physical findings of angiosarcoma depend on the site of the tumor and degree of metastasis.
ICD-9: 171.9
ICD-10: C22.3
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PROGRESSION
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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.
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TREATMENT
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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.
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SUGGESTED PROGRAMMATIC ASSESSMENT*
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Suggested MER for
Evaluation:
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•
Clinical history and examination that describes the diagnostic features of the impairment;
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-
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Suggested Listings for Evaluation:
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DETERMINATION
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LISTING
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REMARKS
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Meets
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13.03 A
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Evaluate in adults under 13.03 A depending upon site of origin, upon diagnosis confirmed
by objective medical evidence.
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13.04
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Evaluate in adults under 13.04 depending upon site of origin, upon diagnosis confirmed
by objective medical evidence.
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13.13 A 3
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Evaluate in adults under 13.13 A 3 depending upon site of origin, upon diagnosis confirmed
by objective medical evidence.
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113.03
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Evaluate in children under 113.03 depending on site of origin, upon diagnosis confirmed
by objective medical evidence.
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113.13 C
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Evaluate in children under 113.13 C, depending on site of origin, upon diagnosis confirmed
by objective medical evidence.
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Equals
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* Adjudicators may, at their discretion, use the Medical Evidence of Record or the
listings suggested to evaluate the claim. However, the decision to allow or deny the
claim rests with the adjudicator.
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