MALIGNANT RENAL RHABDOID TUMOR
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ALTERNATE NAMES
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Malignant Rhabdoid Tumor of the Kidney; Malignant RT
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DESCRIPTION
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Malignant Renal Rhabdoid Tumor (MRRT) is a rare malignant tumor that can develop in many types of organs and tissues, but
occurs primarily in the kidney or brain. It is a highly aggressive cancer mostly affecting
children. MRRTs are caused by genetic mutations in chromosome 22 and mutations in
the SMARCB1/INI1 gene.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND
ICD-9-CM/ICD-10-CM
CODING
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Diagnostic testing: The diagnosis of MRRT is made by:
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Biopsy with histology reports;
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Molecular genetic testing--usually chromosome microarray analysis or fluorescent in
situ hybridization (FISH) test analysis documenting the 22q11.1 deletion; and
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Imaging studies such as, chest CT, abdominal ultrasound.
MRI, CT, or PET scan imagining of the brain and bone scan are warranted when evidence
of metastases .
Physical findings: People with MRRTs may present with signs and symptoms related to an intra-renal or
large intra-abdominal mass.
Children with this tumor may present with:
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Synchronous or metachronous CNS lesions; and
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Metastases and secondary primary cancers.
ICD-9:
189.0
ICD-10:
C79.0
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PROGRESSION
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MRRTs are very aggressive tumors that have a poor prognosis with mortality generally
within 12 months of diagnosis. These tumors tend to occur in children under the age
of two years.
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TREATMENT
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Depending on the size and location of the tumor, treatment of MRRT generally consists
of surgical tumor removal, and chemotherapy. Treatment of children with malignant
rhabdoid tumors will require treatment planning by a multidisciplinary team consisting
of pediatric oncologists, pediatric surgeon or urologist, radiation oncologist, pediatric
geneticist, social worker, or nutritionist.
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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 the diagnostic features of the impairment;
and
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Laboratory findings are needed to confirm the diagnosis.
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Suggested Listings for Evaluation:
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DETERMINATION
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LISTING
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REMARKS
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Meets
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13.21
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Inoperable, unresectable, metastatic, or recurrent disease in adults meets the criteria
in listing 13.21.
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113.03
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Confirmed diagnosis of the original tumor or recurrence in children meets the criteria
in listing 113.03.
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Equals
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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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