Program Operations Manual System (POMS)
TN 73 (08-24)
DI 23022.879 Renal Medullary Carcinoma
COMPASSIONATE ALLOWANCES INFORMATION
|
RENAL MEDULLARY CARCINOMA
|
ALTERNATE NAMES
|
Kidney Medullary Carcinoma; Medullary Carcinoma of the Kidney; Medullary Renal Cell
Carcinoma; RCCU-MP; Renal Cell Carcinoma, Unclassified, with Medullary Phenotype;
RMC
|
DESCRIPTION
|
Renal medullary carcinoma (RMC) is a rare but aggressive type of kidney cancer. It’s a very rare subclass of renal
cancer that is largely restricted to patients who carry the sickle cell trait, sickle
cell disease, or other sickle hemoglobinopathies that can cause sickling of the red
blood cells. Generally, about 80 percent of cases have metastatic disease present
at diagnosis. Patients without initial metastatic disease almost universally develop
metastatic spread. RMC cells lack a protein called INI1 or SMARCB1. This protein is
a tumor suppressor and normally helps prevent cells from becoming cancerous.
RMC develops in the innermost part of the kidney (medulla). Its main function is to
maintain the blood’s water and salt balance.
|
DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM
CODING
|
Diagnostic testing: Diagnostic testing for RMC may include:
-
•
Physical exam and history;
-
•
Computed tomography (CT) scan;
-
•
Magnetic resonance imaging (MRI); and
Physical findings: Symptoms of RMC include:
-
•
Low red blood cell count; and
-
•
Swelling in the lower body.
ICD-9: 189.0
ICD-10: C64.9; C7A.093
|
PROGRESSION
|
The prognosis for people with RMC is dismal with a current median overall survival
of about 13 months. Generally, by the time most people are diagnosed, the cancer has
spread to the lymph nodes or other organs. The survival rate is within 2 years regardless
of treatment and therapy.
|
TREATMENT
|
Due to the rarity of the disease, there is no standard of care for RMC. Many of the
therapies that are used for other kidney cancers do not work against RMC. Initial
chemotherapy is most commonly employed with subsequent consideration for surgery or
additional chemotherapy. Other specific therapeutic procedures may include radiation
therapy or other therapies.
|
SUGGESTED PROGRAMMATIC ASSESSMENT*
|
Suggested MER for Evaluation:
-
•
Clinical history and physical examination;
-
•
Results of imaging (CT scan, MRI, ultrasound); and
-
•
Biopsy to determine if the SMARCB1 gene is present or nonexistent.
|
Suggested Listings for Evaluation:
|
DETERMINATION
|
LISTINGS
|
REMARKS
|
Meets
|
13.21 A or B
|
Metastatic disease is typically present at diagnosis and should be evaluated under
listing 13.21 B.
|
Equals
|
|
|
*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.
|