Program Operations Manual System (POMS)
TN 37 (09-20)
DI 23022.146 Desmoplastic Small Round Cell Tumors
COMPASSIONATE ALLOWANCES INFORMATION
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DESMOPLASTIC SMALL ROUND CELL TUMORS
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ALTERNATE NAMES |
DSRCT; Desmoplastic small round-cell tumor
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DESCRIPTION
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Desmoplastic small round cell tumor (DSRCT) is a rare, highly aggressive type of soft tissue cancer (sarcoma) that usually begins
in the abdomen. It primarily affects children and young adults and is more common
in males. It is formed by small, round cancer cells surrounded by scar-like tissue
and is often found in the tissue (peritoneum) that lines the inside of the abdomen
and pelvis.
DSRCT often is not diagnosed until the cancer is advanced, tumors grow large and spread
through the lymph system or blood stream to other parts of the body. They can spread
to many areas of the body, including the lymph nodes, lungs, bone, and liver.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND
ICD-9-CM/ICD-10-CM
CODING
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Diagnostic testing: A diagnosis of DSRCT is usually made by a combination of the histologic appearance
and immunohistochemical staining results from a biopsy specimen.
Other testing may include:
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Fluorescence in situ hybridization (FISH).
Physical findings:
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Abdominal pain and a feeling of fullness;
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Hepatomegaly (enlarged liver);
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Ileus (obstruction of ileum or other part of the intestine); and
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Symptoms of gastrointestinal obstruction, such as constipation.
ICD-9:
158
ICD-10:
C48.2
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PROGRESSION
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DSRCT typically arises in serous lined surfaces of the abdominal or pelvic peritoneum,
and spreads to the omentum, lymph nodes, and hematogenously (carried by blood) disseminates
especially to the liver.
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TREATMENT
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Treatment of DSRCT may include chemotherapy, hyperthermic intraperitoneal chemotherapy
(HIPEC), radiation therapy, surgery, and stem cell transplantation. DSRCT is often
resistant to treatment and frequently recurs. Despite aggressive therapy, 3-year overall
survival has been estimated at 44% and the 5-year survival rate remains around 15%.
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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 diagnostic features of the impairment;
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Results of cytogenic analysis or FISH testing; and
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Results of immunohistochemical staining.
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Suggested Listings for
Evaluation: |
DETERMINATION
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LISTING
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REMARKS
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Meets
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113.03 A or B
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Equals
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13.04 A or B
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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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