TN 8 (11-12)
DI 23022.927 Alveolar Soft Part Sarcoma
COMPASSIONATE ALLOWANCE INFORMATION
ALVEOLAR SOFT PART SARCOMA
ASP Sarcoma; ASPS
Alveolar Soft Part Sarcoma (ASP) is a rare type of soft tissue sarcoma occurring primarily in children and adolescents. These tumors are usually located in the head and neck, arms or legs (usually the deep thigh area), chest, abdomen, genital organs, or anal area. These tumors usually arise in the soft tissues containing many blood vessels, but can also grow inside the bones. The exact cause of ASP is unknown, but some scientific research suggests that genetic mutations in the ASPL and TFE3 genes are contributory to the cause of ASP. Signs and symptoms of ASP include:
a painless swelling or lump
pain or soreness caused by compressed nerves or muscles
limping or other difficulty using the legs and feet
DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING
Diagnostic testing: X-ray, CT scan, MRI scan, Bone scan, ultrasound exam, bone marrow aspiration and biopsy, lumbar puncture, immunohistochemistry study, and cytogenetic analysis.
Physical findings: Physical findings of ASP depend on the site of the tumor and degree of metastases.
ONSET AND PROGRESSION
ASP occurs mainly in children between the ages of infancy to approximately 19 years of age. The prognosis is poor if there are metastases to other sites. Persons with ASP tumors may relapse several years after a prolonged period of apparent remission.
Treatment of ASP tumors depends on the specific type of tumor, its size, its location, and the amount that it has spread (metastasis). The standard treatment of these tumors is a complete resection of the primary tumor. If complete excision 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 must be documented by metastases, or by recurrence or persistence following initial anti-neoplastic treatment. Listing level severity in children must be documented by confirmed diagnosis or recurrence.
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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.