LEIOMYOSARCOMA

ALTERNATE NAMES

Leiomyosarcoma of the Uterus; Leiomyosarcoma of Vascular Origin; Leiomyosarcoma of the Bone; Leiomyosarcoma of the Retroperitoneum; Leiomyosarcoma Stage IV; Leiomyosarcoma Stage III; High Grade Leiomyosarcoma

DESCRIPTION

Leiomyosarcoma (LMS) is an aggressive soft tissue sarcoma derived from smooth muscle cells of uterine, gastrointestinal, or soft tissue origin. LMS are most often found in the uterus or abdomen, but can spread to other areas through the bloodstream and can affect the lungs, liver, internal organs, blood vessels or other soft tissue in the body. There are 5 types of LMS:

• Soft tissue LMS

• Cutaneous LMS

• Vascular LMS

• LMS of the Immunocompromised

• Bone LMS

LMS is one of the more common types of sarcoma to occur in adults. The exact cause of LMS is unknown. Very rarely, soft tissue sarcomas may occur in an area that has previously been treated with radiotherapy for another type of cancer. Exposure to some types of chemicals may increase the risk of developing some sarcomas. People with early LMS often do not have any symptoms, until the cancer has developed to advanced stages. When the retroperitoneum (area in back of the abdominal cavity) is involved, symptoms may be hidden for a longer period of time because of the large volume of the abdomen, and therefore at presentation the tumor is generally larger than the typical extremity tumor. Symptoms may include: a lump or swelling, abdominal discomfort or bloating, swelling or pain in any area of the body, or unusual bleeding.

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

Diagnostic testing: Diagnostic testing for LMS includes: Physical examination; imaging tests such as X-ray, ultrasound, CT scan, MRI scan; endoscopy for tumors with gastrointestinal involvement; and laboratory analysis. A biopsy confirms the diagnosis.

Physical findings: Physical findings of LMS vary, depending on the location and size of the tumor.

ICD-9: 171.5; 171.6; 171.8

ONSET AND PROGRESSION

LMS generally occur in adults, but may affect children. Prognosis depends on the type of LMS, although it is generally poor. Deep soft tissue LMS are usually detected before they reach the large size of many retroperitoneal tumors. About half of these patients die of metastatic disease. True intradermal (Cutaneous) LMS is curative with surgery and does not metastasize. LMS of vascular origin has a poor prognosis--metastatic disease to the liver and lungs occurs in 54% of cases. LMS in the Immunocompromised Host behaves aggressively. In Bone LMS, recurrences and metastases occurs in 25% of cases.

TREATMENT

The usual treatment for a LMS is surgery to remove the tumor. This may be followed by radiotherapy to reduce the chance of the cancer coming back. Chemotherapy is also used for some LMS to reduce the chances of the recurrence or to treat LMS that has spread.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for evaluation:

• Clinical history and examination that describes the diagnostic features of the impairment and laboratory studies are needed to confirm the diagnosis, including biopsy and imaging.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets Listing

13.04

13.23 A

Leiomyosarcoma of the uterine corpus which invades adjoining organs or is persistent, recurrent or metastatic meets listing 13.23A.

Medical Equals