EWING SARCOMA

ALTERNATE NAMES

Askin Tumor; Askin's Tumor; Bone Endothelioma; Diffuse Bone Endothelioma; Endothelial Myeloma; Endothelial Sarcoma of Bone; Ewing Sarcoma of Bone; Ewing Sarcoma of Soft Tissue; Ewing Tumor; Primitive Neuroectodermal Tumor; Extraosseous Ewing Sarcoma; Extraskeletal 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 sarcoma of bone, 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 Ewing sarcomas are tumors that occur in soft tissue;

• •

  Primitive neuroectodermal tumor (PNET), also known as peripheral neuroepithelioma, can occur in bone and/or soft tissue; and

• •

  Askin tumor, a PNETthat occurs in the marrow cavities of the chest wall.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM CODING

Diagnostic testing: Diagnostic testing for Ewing Sarcoma includes:

• •

  X-rays;

• •

  Biopsy;

• •

  Computed tomography (CT) scan;

• •

  Radionuclide bone scan;

• •

  Magnetic resonance imaging (MRI); and

• •

  Positron emission tomography (PET) scan.

The definitive diagnosis is based on histologic findings, immunohistochemistry, and cytogenetic and molecular studies.

Physical findings: Physical findings for individuals with Ewing sarcoma may include:

• •

  Localized pain;

• •

  Swelling and warmth at site of the tumor;

• •

  Fever;

• •

  Weight loss;

• •

  Pathological fracture;

• •

  Local tenderness; and

• •

  Visible blood vessels on skin over the tumor.

ICD-9: 170.9

ICD-10: C41.9

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 of metastases include the central nervous system and lymph nodes.

TREATMENT

All types of the EFTs are treated similarly based on clinical presentation of local versus metastatic disease. The main treatments for Ewing sarcoma are chemotherapy, surgery, and radiation, almost 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 treatment 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:

• •

  Report that specifies the type, extent, and site of the primary, recurrent, or metastatic lesion;

• •

  Biopsy or needle aspiration information found in operative notes, pathology reports, summaries of hospitalization or other medical reports that include details of the surgical and pathological findings;

• •

  Clinical history and examination that describes the diagnostic features of the impairment;

• •

  Imaging tests; and

• •

  Treatment records including surgical procedures, and up-to-date progress notes.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets

13.04

Extraosseous Ewing sarcoma and other Ewing sarcoma tumors that occur outside of the bone in adults should be evaluated under listing 13.04.

13.11

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

113.03

Pediatric Ewing sarcoma meets listing 113.03.

Equals