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Hereditary Papillary Renal Cell Carcinoma via the MET Gene

Summary and Pricing

Test Method

Sequencing and CNV Detection via NextGen Sequencing using PG-Select Capture Probes
Test Code Test Copy GenesTest CPT Code Gene CPT Codes Copy CPT Codes Base Price
3207 MET 81479 81479,81479 $640 Order Options and Pricing
Test Code Test Copy Genes Test CPT Code Gene CPT Codes Copy CPT Code Base Price
3207MET81479 81479(x2) $640 Order Options and Pricing

Pricing Comments

This test is also offered via our exome backbone with CNV detection (click here). The exome-based test may be higher priced, but permits reflex to the entire exome or to any other set of clinically relevant genes.

An additional 25% charge will be applied to STAT orders. STAT orders are prioritized throughout the testing process.

Turnaround Time

18 days on average for standard orders or 13 days on average for STAT orders.

Please note: Once the testing process begins, an Estimated Report Date (ERD) range will be displayed in the portal. This is the most accurate prediction of when your report will be complete and may differ from the average TAT published on our website. About 85% of our tests will be reported within or before the ERD range. We will notify you of significant delays or holds which will impact the ERD. Learn more about turnaround times here.

Targeted Testing

For ordering sequencing of targeted known variants, go to our Targeted Variants page.

EMAIL CONTACTS

Genetic Counselors

Geneticist

  • Hannah Cox, PhD

Clinical Features and Genetics

Clinical Features

Renal cell carcinoma (RCC) is the most common kidney cancer representing approximately 95% of all renal tumors. RCC includes three different subtypes: clear cell (ccRCC, 75% of RCC), papillary (pRCC, 10%), and chromophobe (chRCC, 5%) (Hagenkord et al. Cancer Genetics 204:285-297, 2011). Most kidney cancers occur sporadically, but approximately 2-4% of renal tumors show familial inheritance (Axwijk et al. Eur J Clin Invest 40:433-439, 2010). Hereditary Papillary Renal Cell Carcinoma (HPRCC) is the familial inheritance of papillary renal tumors, which are often multifocal and bilateral, and have a typical age of onset of 50 to 70 years (Zbar et al. J Urol 151:561– 6, 1994). Papillary renal carcinomas have been morphologically divided into small (type 1) and large (type 2) cell tumors. HPRCC leads to type 1 renal cell carcinomas. Individuals with type 1 pRCC have been shown to have significantly better survival than individuals with type 2 pRCCs. The kidney is the only organ affected in HPRCC, and although the tumors are usually well differentiated, they are malignant and can metastasize with poor prognosis. HPRCC is caused by mutations in the proto-oncogene MET, and targeting the oncogenic form with administration of tyrosine kinase inhibitors combined with conventional chemo- and radio-therapies is currently being investigated (Salvi et al. International Journal of Oncology 33: 271-276, 2008).

Genetics

HPRCC shows an autosomal dominant pattern of inheritance and is highly penetrant. Mutations in the MET oncogene lead to ligand-independent activation of the tyrosine kinase domain of the protein, which leads to constitutive activation of the hepatocyte growth factor (HGF)/c- Met pathway (Coleman and Russo. Current Opinion in Urology 19:478–485, 2009). Gain of function mutations lead to increases in cellular proliferation, neovascularization, and cell motility. Mutations in MET are also observed in sporadic papillary renal carcinomas (Hagenkord et al. Cancer Genetics 204:285-297, 2011). Reported germline MET mutations that correlate to HPRCC are missense, and are mostly located within the tyrosine kinase domain (Salvi et al. International Journal of Oncology 33: 271-276, 2008).

Clinical Sensitivity - Sequencing with CNV PG-Select

Analytical sensitivity should be high because the mutations reported are readily detectable by gene sequencing. Clinical sensitivity of MET mutations in HPRCC is unknown at this time.

Testing Strategy

This test provides full coverage of all coding exons of the MET gene, plus ~10 bases of flanking noncoding DNA. We define full coverage as >20X NGS reads or Sanger sequencing.

Indications for Test

Individuals who have isolated papillary renal cell carcinomas that are multifocal and bilateral. Individuals who have a family history of HPRCC. This test is specifically designed for heritable germline mutations and is not appropriate for the detection of somatic mutations in tumor tissue.

Gene

Official Gene Symbol OMIM ID
MET 164860
Inheritance Abbreviation
Autosomal Dominant AD
Autosomal Recessive AR
X-Linked XL
Mitochondrial MT

Disease

Name Inheritance OMIM ID
Renal Cell Carcinoma, Papillary, 1 AD 605074

Related Test

Name
Renal Cancer Panel

Citations

  • Axwijk et al. (2010). "Hereditary causes of kidney tumours." Eur J Clin Invest 40:433-439. PubMed ID: 20534065
  • Coleman and Russo. 2009. Hereditary and familial kidney cancer. Current Opinion in Urology 19:478–485. PubMed ID: 19584731
  • Hagenkord et al. (2011). "Clinical genomics of renal epithelial tumors." Cancer Genetics 204:285-297. PubMed ID: 21763625
  • Salvi et al. (2008). "Germline and somatic c-met mutations in multifocal/bilateral and sporadic papillary renal carcinomas of selected patients." International Journal of Oncology 33: 271-276. PubMed ID: 18636147
  • Zbar et al. (1994). "Hereditary papillary renal cell carcinoma." J Urol 151:561– 6. PubMed ID: 8308957

Ordering/Specimens

Ordering Options

We offer several options when ordering sequencing tests. For more information on these options, see our Ordering Instructions page. To view available options, click on the Order Options button within the test description.

myPrevent - Online Ordering

  • The test can be added to your online orders in the Summary and Pricing section.
  • Once the test has been added log in to myPrevent to fill out an online requisition form.
  • PGnome sequencing panels can be ordered via the myPrevent portal only at this time.

Requisition Form

  • A completed requisition form must accompany all specimens.
  • Billing information along with specimen and shipping instructions are within the requisition form.
  • All testing must be ordered by a qualified healthcare provider.

For Requisition Forms, visit our Forms page


Specimen Types

Specimen Requirements and Shipping Details

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ORDER OPTIONS

View Ordering Instructions

1) Select Test Method (Backbone)


1) Select Test Type


2) Select Additional Test Options

STAT and Prenatal Test Options are not available with Patient Plus.

No Additional Test Options are available for this test.

Note: acceptable specimen types are whole blood and DNA from whole blood only.
Total Price: $
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