EWING SARCOMA

ALTERNATE NAMES

Ewing Tumor; Ewing Sarcoma of Bone, Ewing Sarcoma of Soft Tissue; Primitive Neuroectodermal Tumor; PNET; Askin Tumor; Diffuse Bone Endothelioma; Endothelial Myeloma; Bone Endothelioma; Endothelial Sarcoma of Bone; Extraosseous Ewing Sarcoma

DESCRIPTION

Ewing Sarcoma is a malignant, vascular, solid, round-cell tumor in which cancer cells are found in the bone or in soft tissue. The most common areas in which it occurs are the pelvis, the femur, the humerus, and the ribs. It is an aggressive cancer and multimodal (therapy that combines more than one method of treatment) therapy is virtually always required.

Ewing sarcoma is part of a group of four different types of cancer, known collectively as the Ewing Family of Tumors (EFT): Ewing bone sarcoma, which accounts for about 85% of all cases, is usually found in the long bones in the arm or leg, although it sometimes occurs in the pelvis or ribcage. Extraosseous sarcomas are tumors that occur outside the bone. Primitive Neuroectodermal Tumor (PNET), also known as peripheral neuroepithelioma, can occur in bone and/or soft tissue. Askin tumor is a PNET that occurs in the marrow cavities of the chest wall. General symptoms may include local pain, local swelling, local tenderness, fever, warm skin over tumor, or visible blood vessels on skin over tumor.

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

Diagnostic testing: Diagnostic testing for Ewing sarcoma includes x-rays, biopsy, CT scan, radionuclide bone scan, MRI, and PET scan. The definitive diagnosis is based on histologic findings, immunohistochemistry, and cytogenetic and molecular studies.

Physical findings: Physical findings may include localized pain, swelling and warmth at site of tumor, fever, weight loss, and pathological fracture.

ICD-9: 170.9

ONSET AND PROGRESSION

Onset for Ewing sarcoma can occur any time during childhood, with peak incidence in the teen years. The prognosis depends on the location of the tumor, and whether or not the cancer has spread. Approximately 30% are metastatic at presentation. Diagnostic staging procedures attempt to distinguish individuals with localized disease from those with metastatic disease. Most commonly, metastases occur in the chest, bone and/or bone marrow. Less common sites include the central nervous system and lymph nodes.

TREATMENT

All types of the Ewing family of sarcomas are treated similarly based on clinical presentation of local vs. metastatic disease. The main treatments for Ewing sarcoma are chemotherapy, surgery, and radiation, virtually always used in combination.

In the past, surgery on tumors in the arm or leg bones usually required amputation. However, new techniques such as limb-sparing surgery, bone grafts, and artificial bones, can help certain patients avoid losing an arm or leg and maintain some degree of function.

Side effects associated with radiation may include skin damage, muscle scarring and loss of joint flexibility, damage to nearby organs, loss of bone growth in growing children, secondary cancers caused by radiation, chronic swelling of an extremity, and slow wound healing.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for evaluation: The adjudicator needs medical evidence from treating sources and hospitals. It should include clinical history and examination that describe the diagnostic features of the impairment, imaging tests, biopsies, surgical procedures, pathology reports, pertinent treatment records, and up-to-date progress notes.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets Listing

13.04

13.11

113.03

Ewing sarcoma in adults treated with multimodal therapy meets 13.11D without regard to effectiveness of treatment. Pediatric Ewing sarcoma meets 113.03.

Medical Equals